American Thoracic Society International Conference · P1393 9:15 am-4:15 pm; Poster Viewing 11:15...

May 18-May 23, 2018San Diego, CA

conference.thoracic.org

Rare Lung Disease GuideWhere today’s science meets tomorrow’s careTM

American Thoracic Society International Conference

1

May 2018

Dear Colleagues:

The scope of the American Thoracic Society is amazingly broad – covering pulmonary, critical care, and sleep medicine. The challenge that we face each year as we put the International Conference program together is that some clinical topics that may be of central importance to our members, conference attendees, and patients may not be prominently featured in the program. This is especially the case with some rare lung diseases.

With each challenge though, comes opportunity. Because of the wealth of scientific and clinical information presented at ATS 2018, these diseases, though uncommon, will be the focus of many presentations over the next several days. The purpose of this Rare Lung Disease Guide is to help you more easily find these presentations.

In his chapter on rare lung diseases in “Breathing in America: Diseases, Progress, and Hope” (2010) Francis X. McCormack, MD noted that research on uncommon respiratory diseases has produced some of the most exciting discoveries in pulmonary medicine, stating that “Insights gained from uncommon lung diseases often shed light on the more common ones.”

The definition of a rare disease by its prevalence varies by country. A disease or disorder is defined as rare in the United States when it affects fewer than 200,000 Americans at any given time. This guide was compiled by a small group of ATS Members who studied the ATS 2018 abstracts – authors requested to be considered for inclusion. Many thanks to Dr. Frank McCormack, Dr. Lisa Young, Dr. Jonathan Kropski, Dr. Nishant Gupta, and Mr. Jack Kelly for their assistance with this resource.

There are a number of patient advocacy groups that are exhibiting at ATS 2018. In addition to attending the presentations noted within, I encourage you to visit with them to learn more about these rare conditions. Groups listed in bold are members of the ATS Public Advisory Roundtable and can all be found in PAR Path, near the ATS Center in the Exhibit Hall. Learn more about this group and its many contributions to the Society and its work at: http://www.thoracic.org/patients/par/.

Sincerely,

Jess Mandel, MD Chair, ATS International Conference Committee

Jess Mandel, MDChair, ATS International Conference Committee

Alpha-1 Foundation, Booth 536ARDS Foundation, Booth 439Asthma and Allergy Network, Booth 641Children’s Interstitial Lung Disease Foundation, Inc. (CHILD), Booth 740Cystic Fibrosis Research, Inc., Booth 638Foundation for Sarcoidosis Research, Booth 639Hermansky-Pudlak Syndrome Network, Inc., Booth 637The LAM Foundation/Lymphangioleiomyomatosis, Booth 538Lung Transplant Foundation, Booth 736

LUNGevity Foundation, Booth 541Lymphangiomatosis and Gorham’s Disease Alliance (LGD), Booth 537NTM Info & Research, Inc., Booth 539Primary Ciliary Dyskinesia Foundation (PCD), Booth 636Pulmonary Fibrosis Foundation, Booth 722Pulmonary Hypertension Association, Booth 1542Scleroderma Foundation, Booth 640Tuberous Sclerosis Alliance, Booth 738

List Current as of 3/29/18

DISCLAIMER

The purpose of this guide is to help the American Thoracic Society International Conference attendees navigate the viewing of posters for disorders that typically get less attention at the conference because of their rarity. During abstract submission, authors were instructed to check the box for consideration in the Rare Lung Disease Guide; the abstracts on that list were then further narrowed down. Conditions represented by less than 50 abstracts were considered for inclusion. We regret that not all rare diseases could be represented, and welcome suggestions for additional categories for listing in future editions. Please email us with any suggestions: [email protected].

3

Rare Disease Term

Date/Day Board Number/Time Abstract Title Session ID and Title Location

ACD-MPV (ALVEOLAR CAPILLARY DYSPLASIA WITH MISALIGNMENT OF THE PULMONARY VEINS)

Tuesday, May 22, 2018

P1046 9:15 am-4:15 pm;Poster Viewing 11:15 am-1:00 pm

A Novel Pathogenic Variant in FOXF1 Gene in an Infant with Severe Pulmonary Hypertension

C65: PEDIATRIC CASE REPORTS: NEONATAL LUNG DISEASE

Area J (Hall A-B2, Ground Level)San Diego Convention Center


Neonatal Severe Refractory Persistent Pulmonary Hypertension of the Newborn (PPHN), Rare and Perplexing Diagnosis

C66: PEDIATRIC CASE REPORTS: CARDIOVASCULAR AND CRITICAL CARE DISEASE


ALPHA-1 ANTITRYPSIN DEFICIENCY

Sunday, May 20, 2018


Alpha-1 Antitrypsin Deficiency Is Not a Risk Factor for Cancer: A Cross-Sectional Study

A46: TOPICS IN ALPHA-1-ANTITRYPSIN DEFICIENCY Area L (Hall A-B2, Ground Level)San Diego Convention Center

P1396 9:15 am-4:15 pm; Poster Viewing 11:15 am-1:00 pm

Alpha-1 Antitrypsin Therapy: A Satisfaction Survey of Individuals Self-Administering



Alpha-1 Antitrypsin Deficiency Screening in Adults with Bronchiectasis Using Dry Blood Spot



Results from a Large-Scale Screening Program for Alpha-1 Antitrypsin Deficiency: A History of Bronchiectasis Is Associated with the ZZ Genotype



Smoking and SZ Alpha-1 Antitrypsin Deficiency (AATD): Exploring the Relationship Between Smoke Exposure and Airflow Obstruction



Analysis of Factors Associated with FEV1 Decline in Alpha-1 Antitrypsin Deficient Patients: A Comparison of Statistical Approaches



Demographics and Patient Characteristics of the PI*MZ Allele Subgroup Identified in the US National Detection Program for Alpha-1-Antitrypsin Deficiency



Characteristics of Patients with Rare PI*F and PI*I Alleles from the US National Detection Program for Alpha-1-Antitrypsin Deficiency



Modern Epidemiology of Alpha-1-Antitrypsin Deficiency (AATD) in the UK

A55: ENVIRONMENTAL AND OCCUPATIONAL GENETICS, GENOMICS, AND EPIGENETICS

Area A (Hall A-B2, Ground Level)San Diego Convention Center


The Irish National Alpha-1 Antitrypsin Deficiency Targeted Detection Programme



4

Rare Disease Term




Effects Of Recombinant Alpha1-Antitrypsin IgG1 Fc-fusion Protein (AAT-Fc) In Experimental Emphysema

A75: COPD: TARGETS, MODELS, AND CLINICAL STUDIES Area E (Hall A-B2, Ground Level)San Diego Convention Center


Effect Of N-glycosylation Of Recombinant Alpha-1 Antitrypsin Therapeutic Protein Structure And Function: A Biophysical And Molecular Dynamic Simulation Study

A75: COPD: TARGETS, MODELS, AND CLINICAL STUDIES Area E (Hall A-B2, Ground Level)San Diego Convention Center

Monday, May 21, 2018


Cardiovascular status and risk in Alpha 1 anti-trypsin Deficiency B54: CHRONIC DISORDERS: EVALUATION AND INTERVENTION

Area B (Hall A-B2, Ground Level)San Diego Convention Center


Ventilation and Gas Transfer Distribution in Alpha-1 Antitrypsin Deficiency After Replacement Therapy

B64: COPD: LUNG FUNCTION, IMAGING AND PATHOPHYSIOLOGY

Area L (Hall A-B2, Ground Level)San Diego Convention Center


Proposal and Validation of a Minimal Clinically Important Difference (MCID) for Annual Pulmonary CT Density Decline




Imaging Biomarkers of Emphysema in COPD and Alpha-1 Antitrypsin Deficiency Patients using Accelerated 129Xe MRI




Ventilation And Gas Transfer Measured By 129Xe MRI In Alpha-1 Antitrypsin Heterozygotes: A Pilot Study



Oral Presentation 2:15 pm-4:15 pm Comprehensive Gene Resequencing of SERPINA1 in SPIROMICS Re-veals Novel Rare Loci for 1-antitrypsin Deficiency and Emphysema

B96: GENETICS, EPIGENETICS, AND SMOKING Room 15 A-B (Mezzanine Level)San Diego Convention Center


622 9:15 am-11:15 am;Poster Viewing 9:15 am-9:45 amPoster Discussion 9:45 am-11:15 am

Evaluation of the Safety and Effectiveness of the Spiration Valve System for Single Lobe Treatment of Severe Emphysema in Patients with Alpha-1 Antitrypsin Deficiency

C24: NEW TECHNOLOGIES FOR MANAGING COPD Room 30 C-E (Upper Level)San Diego Convention Center


Measuring Endoplasmic Reticulum Viscosity in Cell Models of 1-Antitrypsin Deficiency.

C28: ROLE OF CELLULAR CROSS TALK IN LUNG HOMEOSTASIS AND AGING

Pacific Ballroom 15-17 (North Tower, First Floor)Marriott Marquis San Diego Marina


A Systematic Analysis of Terminal Bronchiole Remodeling in COPD Patients with and without Alpha-1 Antitrypsin Deficiency

C29: LUNG STRUCTURE AND FUNCTION: NEW INSIGHTS FROM MEASUREMENT AND MODELING



Aα-Val360 and Aα-Val541in Early Alpha-1 Antitrypsin Deficiency C34: COPD POTPOURRI Area L (Hall A-B2, Ground Level)San Diego Convention Center


Proteomic Profiling of Protein-Protein Interactions of Alpha-1 Antitrypsin in Plasma

C34: COPD POTPOURRI Area L (Hall A-B2, Ground Level)San Diego Convention Center

5

Rare Disease Term




Increased Neutrophil-Platelet Interactions in Alpha-1 Antitrypsin Deficient Individuals is Corrected Post Augmentation Therapy

C34: COPD POTPOURRI Area L (Hall A-B2, Ground Level)San Diego Convention Center


Predictors of Alpha-1 Antitrypsin Deficiency Among Medicare Beneficiaries with Chronic Obstructive Pulmonary Disease in the United States

C42: CONTEMPORARY TOPICS IN COPD Area L (Hall A-B2, Ground Level)San Diego Convention Center


Predictors of Alpha-1 Antitrypsin Deficiency Among Commercially Insured Chronic Obstructive Pulmonary Disease Patients in the United States

C42: CONTEMPORARY TOPICS IN COPD Area L (Hall A-B2, Ground Level)San Diego Convention Center


Pulmonary Hypertension Characteristics in Alpha-1 Antitrypsin Deficiency Patients Undergoing Lung Transplant Evaluation

C70: WHO'S NEXT? NEW INSIGHTS INTO ATYPICAL WHO GROUP 1 AND GROUP 2-5 PH

Area C (Hall A-B2, Ground Level)San Diego Convention Center


Comparison of Pulmonary CT Density analysis between two leading software programs

C75: LUNG MEASUREMENT: NOVEL METHODS AND TECHNIQUES

Area D (Hall A-B2, Ground Level)San Diego Convention Center

Wednesday, May 23, 2018


Recurrent Acute Pancreatitis Secondary to Alpha-1-Antitrypsin Deficiency

D40: COPD: CASE REPORTS Area L (Hall A-B2, Ground Level)San Diego Convention Center


A Comprehensive Repository Of Aatd Patient-specific Induced Pluripotent Stem Cells With Detailed Clinical Phenotyping For Translational Research

D53: OFF THE BEATEN PATH: NEW APPROACHES TO COPD PATHOGENESIS



Alpha-1 Antitrypsin Antisense Oligonucleotide Modulates Protease-Antiprotease Imbalance without Further Aggravating Smoke-induced Lung Injury




Lectin Complement Pathway Activation In Copd And Alpha-1 Antitrypsin (A1AT) Deficiency




The Altered Abundance of Hydrogen Voltage-Gated Channel 1 (HVCN1) in Neutrophils of Alpha-One Antitrypsin Deficient Patients




Role for Cela1 in Postnatal Lung Remodeling and AAT-Deficient Emphysema



606 1:30 pm-3:30 pm;Poster Viewing 1:30 pm-2:00 pmPoster Discussion 2:00 pm-3:30 pm

Neutrophil Elastase and Proteinase 3 Activity in PiSZ Alpha-1 Antitrypsin Deficiency

D101: MECHANISTIC AND TRANSLATIONAL STUDIES IN COPD

Room 30 C-E (Upper Level)San Diego Convention Center

6

Rare Disease Term



612 1:30 pm-3:30 pm; Poster Viewing 1:30 pm-2:00 pmPoster Discussion 2:00 pm-3:30 pm

Gene Co-expression Networks Analysis Reveals Novel Molecular Endotypes in Alpha-1 Antitrypsin Deficiency




The Circulating Neutrophil Proteome is Altered in Alpha-1 Antitrypsin Deficiency and is Modified By Intravenous Augmentation Therapy



AMYLOIDOSIS


206 2:15 pm-4:15 pm;Poster Viewing 2:15 pm-2:45 pPoster Discussion 2:45 pm-4:15 pm

Presentation, Survival and Factors of Survival in Respiratory Tract Amyloidosis: A French Consecutive Case Series of 72 Patients

A103: ILD: OUTCOMES Room 6 D (Upper Level)San Diego Convention Center



Everything that Wheezes Is Not Asthma: Rare Case of Primary Endobronchial Amyloidoma

B37: INTERVENTIONAL PULMONARY: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


Endobronchial Obstruction by a Solitary Extramedullary Plasmacytoma with Light Chain Amyloidosis

B69: CASE REPORTS IN THORACIC ONCOLOGY I Area D (Hall A-B2, Ground Level)San Diego Convention Center



Pulmonary Amyloidosis — A Deadly Disease D32: ILD: CASE REPORTS II Area H (Hall A-B2, Ground Level)San Diego Convention Center


A Unique Case of Systemic Amyloidosis Presenting with Recurrent Pleural Effusion

D36: PLEURAL DISEASE: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

ANTI-GLOMERULAR BASEMENT MEMBRANE DISEASE



Alveolar Hemorrhage in Anti-Basement Membrane Antibody Disease B32: CLINICAL STUDIES IN AUTOIMMUNE LUNG DISEASE Area G (Hall A-B2, Ground Level)San Diego Convention Center


A Co-Occurrence of Good Pastures Disease and Graves Disease B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level) San Diego Convention Center



Relapsing Remitting Isolated Pulmonary Anti Glomerular Basement Membrane Disease

D32: ILD: CASE REPORTS II Area H (Hall A-B2, Ground Level)San Diego Convention Center

ARTERIOVENOUS MALFORMATION



Bronchial Dieulafoy’s Disease and Mediastinal Arteriovenous Malformation


7

Rare Disease Term




Paradoxical Embolization and a Large Pulmonary Arteriovenous Malformation

B58: WHO'S BAD: CASE REPORTS IN PULMONARY VASCULAR MEDICINE I



An Uncommon Cause of Hypoxia; Pulmonary Arteriovenous Malformations Successfully Treated with Embolization





Successful Catheter Embolization of a Pulmonary Arteriovenous Malformation in a Patient with Hereditary Pulmonary Arterial Hypertension Due to BMPR2 Mutation

D49: SURFING SAFARI: CASE REPORTS IN PULMONARY VASCULAR MEDICINE II

Area E (Hall A-B2, Ground Level)San Diego Convention Center


A Case of a Massive Pulmonary Artery Aneurysm D50: CLOT ON THE ROCKS: CASE REPORTS OF PE AND CTEPH

Area E (Hall A-B2, Ground Level) San Diego Convention Center

BENIGN ASBESTOS PLEURAL EFFUSION



BAPE, A Rare Cause of Exudate Effusion B37: INTERVENTIONAL PULMONARY: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center

BENIGN METASTASIZING LEIOMYOMA



Benign Metastasizing Leiomyoma Presenting with Lymphangitic Carcinomatosis

A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center



Benign Metastasizing Leiomyomas of the Lung in a Woman 12 Years After Hysterectomy for Uterine Fibroids

B71: CASE REPORTS IN THORACIC ONCOLOGY III Area D (Hall A-B2, Ground Level)San Diego Convention Center



Not All Wheezes Are the Same: A Rare Presentation of Pulmonary Benign Metastasizing Leiomyoma

D31: INTERVENTIONAL PULMONARY: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

BERYLLIUM ASSOCIATED LUNG DISEASE



A Case of Fatal Community Acquired Chronic Beryllium Disease Surrounding a Beryllium Facility



DNA Methylation Changes in Bronchoalveolar Lavage Associated with Chronic Beryllium Disease



BIRT-HOGG-DUBE SYNDROME



Characterization of CT Scan in Birt-Hogg-Dube Syndrome (BHDs) Compared with Non-BHDs Diffuse Cysts Lung Diseases in Chinese Patients

A39: SARCOIDOSIS AND RARE LUNG DISEASE SCIENTIFIC ABSTRACTS


8

Rare Disease Term




Strange Coincidences: A Case of Concurrent Sarcoidosis and Birt-Hogg-Dubé



Secondary Pneumothorax in a Patient with Birt-Hogg-Dube Syndrome

A48: DYSPNEA: CASE REPORTS I Area L (Hall A-B2, Ground Level)San Diego Convention Center



Birt Hogg Dube Syndrome — A Rare Cause of Recurrent Pneumothorax

B39: PLEURAL DISEASE: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center



Rethinking the Epidemiology of Birt-Hogg-Dubé: A Genetics-Based Approach

D103: TRANSPLANT AND ILD POTPOURRI Room 6 D (Upper Level)San Diego Convention Center

BLUE RUBBER BLEB NEVUS SYNDROME



Blue Rubber Bleb Nevus Syndrome: A Rare Cause of Obstructive Sleep Apnea

D59: CASE REPORTS IN SLEEP DISORDERS Area F (Hall A-B2, Ground Level)San Diego Convention Center

BRONCHIOLITIS OBLITERANS



Study of the Etiology and Outcomes in Non-Transplant Related Small Airway Disease

A23: ILD: DIAGNOSIS Room 5 A-B (Upper Level)San Diego Convention Center



Comparative Histological and Morphometrical Analysis of Small Conducting Airways in Constrictive Bronchiolitis and Chronic Obstructive Pulmonary Disease




221 9:15 am-11:15 amPoster Viewing 9:15 am-9:45 amPoster Discussion 9:15 am-11:15 am

Feasibility And Validity Of A Home-based Spirometry Program After Hematopoietic Stem Cell Transplantation For The Early Detection Of Pulmonary Impairment

C23: LUNG TRANSPLANT AND ACUTE ILD Room 6 D (Upper Level)San Diego Convention Center


Quantitative CT and Parametric Response Mapping Analysis for the Assessment and Monitoring of Bronchiolitis Obliterans Syndrome

C32: LUNG TRANSPLANTATION: CLINICAL STUDIES Area H (Hall A-B2, Ground Level)San Diego Convention Center


Un-Even Steven: An Unusual Complication of Stevens-Johnson Syndrome

C67: PEDIATRIC CASE REPORTS: INTERSTITIAL LUNG DISEASE


912 Poster Viewing 2:15 pm-2:45 pm;Poster Discussion 2:45 pm-4:15 pm

Flavoring-Related Lung Disease in a Worker at a Coffee Roasting and Packaging Facility

C106: OCCUPATIONAL LUNG EPIDEMIOLOGY Pacific Ballroom 15-17 (North Tower, First Floor)Marriott Marquis San Diego Marina

9

Rare Disease Term




Successful Treatment of Deployment-Related Constrictive Bronchiolitis with Azithromycin




Pulmonary Screening of Pediatric Hematopoietic Stem Cell Transplant Patients

D25: ADVANCES IN RARE LUNG AND PULMONARY VASCULAR DISEASE IN CHILDREN

Room 6 D (Upper Level)San Diego Convention Center


Obliterative Bronchiolitis: A Rare Complication of Aspiration Pneumonitis



Every Dog Has Its Day: FIBOS Caused by Diacetyl Exposure from an Unusual Source

D34: LUNG TRANSPLANT AND DRUG INDUCED LUNG DISEASE: CASE REPORTS



Obliterative Bronchitis and Bronchiolitis After Toxic Epidermal Necrolysis

D37: CF AND NON-CF BRONCHIECTASIS AND IMMUNE DEFICIENCIES: CASE REPORTS

Area G (Hall A-B2, Ground Level)San Diego Convention Center


Angry Airways: A Tale of Bronchocentric Granulomatosis D39: DYSPNEA: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

P785 11:15 am-1:00 pm Inexplicable Dyspnea in a Soldier: Constrictive Bronchiolitis D39: DYSPNEA: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

BRONCHOLITHIASIS



Bronchoscopic Removal of Broncholiths D31: INTERVENTIONAL PULMONARY: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

CASTLEMAN DISEASE



Bronchiolitis Obliterans from Paraneoplastic Pemphigus Associated with Unicentric Castleman Disease

B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center

CONGENITAL CENTRAL HYPOVENTILATION SYNDROME



Mild Phenotype in Nonpolyalanine Repeat Mutations of PHOX2B in Congenital Central Hypoventilation Syndrome

A59: PEDIATRIC SLEEP Area F (Hall A-B2, Ground Level)San Diego Convention Center


Congenital Central Hypoventilation Syndrome in Israel — a New Nationwide Center



NIH Toolbox Cognition Battery Study: A Multi-Site Evaluation of Neurocognition in Congenital Central Hypoventilation Syndrome (CCHS)


10

Rare Disease Term


CONNECTIVE TISSUE DISEASE ASSOCIATED ILD



The Common Gain-of-function MUC5B Promoter Variant rs35705950 Contributes to the Risk of Developing Rheumatoid Arthritis Interstitial Lung Disease

A71: THE EPIGENOME, GENOME AND NON-CODING RNAs IN LUNG DISEASE



The Incidence of Interstitial Pneumonia with Autoimmune Features in a Tertiary Referral Center Interstitial Lung Disease Clinic

A43: ILD SCIENTIFIC ABSTRACTS: GENERAL Area K (Hall A-B2, Ground Level)San Diego Convention Center


Survival Distinction in Interstitial Pneumonia with Autoimmune Features (IPAF) Patients: Results from a 5-Year Historical Cohort in an Interstitial Lung Diseases (ILD) Population




Pulmonary 18F-FDG Up-Take Refines Current Risk Stratification in Non Specific Interstitial Pneumonitis (NSIP)

B32: CLINICAL STUDIES IN AUTOIMMUNE LUNG DISEASE Area G (Hall A-B2, Ground Level)San Diego Convention Center


Autoantibodies associated with Connective Tissue Disease in Interstitial Lung Disease — A Prospective Study

B35: LUNG TRANSPLANT AND ILD SCIENTIFIC ABSTRACTS: DRUG INDUCED LUNG DISEASE, AUTOIMMUNE LUNG DISEASE


CYSTIC AND BULLOUS LUNG DISEASE — OTHER



Occam’s Razor: An Extremely Rare Presentation of a Very Rare Pulmonary Manifestation of Lupus




Idiopathic Giant Bullous Emphysema in a Female Teenager C64: PEDIATRIC CASE REPORTS: AIRWAY DISEASES AND BRONCHOSCOPY




The Cocktail Effect of Cigarettes and Marijuana in Giant Bullous Emphysema

D40: COPD: CASE REPORTS Area L (Hall A-B2, Ground Level)San Diego Convention Center

CYSTIC FIBROSIS



The ENaC Regulatory Peptide SPX-101 Is Resistant to Proteolytic Degradation in Diseased Sputum

A28: ADVANCES IN COPD AND ASTHMA Room 28 C-E (Upper Level)San Diego Convention Center


Attain Health - Integrating Telehealth and Integrative Health Coaching in Cystic Fibrosis Care

A67: PULMONARY REHABILITATION IN NON-COPD AND LUNG TRANSPLANTATION



Distal Airway Oxygen Tension Is Low in Cystic Fibrosis: Noninvasive Measurement Using a Novel, Fast-Response Oxygen Sensor

A72: WHAT'S NEW IN CYSTIC FIBROSIS, BPD AND OTHER CONGENITAL PEDIATRIC LUNG DISEASES


11

Rare Disease Term




Lung Transplantation In Cystic Fibrosis Patients In Italy: A Single Center Experience Of 20 Years Follow-Up




Baseline Cystic Fibrosis Transmembrane Conductance Regulator Protein Expression Predicts Subject Response to Lumacaftor-Ivacaftor Treatment





Multi-Omics Profiling of Lumacaftor/Ivacaftor Response in Cystic Fibrosis

B25: CHRONIC SUPPERATIVE LUNG DISEASE IN CHILDREN

Room 6 D (Upper Level) San Diego Convention Center


Biochemical and Biophysical Analysis of Direct Bronchial Mucus from Preschool Aged Patients with CF

B25: CHRONIC SUPPURATIVE LUNG DISEASE IN CHILDREN



Increased Airway Mucus Rheology in Cystic Fibrosis Coincides with Positive Microbial Culture in Bronchioalveolar Lavage Fluid

B56: CYSTIC FIBROSIS Area F (Hall A-B2, Ground Level)San Diego Convention Center


Characterization of Cystic Fibrosis Plasma Induced Innate Immune Response and Its Clinical Implication

B56: CYSTIC FIBROSIS Area F (Hall A-B2, Ground Level)San Diego Convention Center


Proteostatic Network Gene Expression Is Decreased in Fdel508 Homozygote Cystic Fibrosis Patients

B63: INFLAMMATION AND CELLULAR RESPONSES IN CYSTIC FIBROSIS

Area H (Hall A-B2, Ground Level)San Diego Convention Center


High-throughput Yeast Phenomics Identifies Genetic Modifiers That Partially Rescue Human W1282X-CFTR




CFTR-deficient Pigs Have Altered Bone Microarchitecture And Elevated Closed Cortical Porosity At Birth




Targeting ENaC With An Epithelial RNAi Trigger Delivery Platform For The Treatment Of Cystic Fibrosis




Pharmacology ofα-7 nicotinic acetylcholine receptors (α-7 nAChR) agonists in Cystic Fibrosis




Evaluation of Apremilast as a novel Therapeutic Agent in Cystic Fibrosis




Neutrophil Extracellular Traps Increase Proinflammatory Cytokine Expression by Human Airway Epithelial Cells In Vitro



12

Rare Disease Term




Applying Epix™ Technology to Enable Ex Vivo Theratyping Using Air-way Epithelial Cells Derived from Patients with Pulmonary Diseases





Social Complexity: A Strong Influence on Outcomes in Adolescents with Cystic Fibrosis

C39: IDENTIFYING AND TREATING ANXIETY AND DEPRESSION IN PULMONARY DISEASES



Differences in the Transition Towards Lung Transplantation for Cystic Fibrosis Between the United States and United Kingdom

C41: HEALTH SERVICES RESEARCH IN PULMONARY DISEASE



Non Concordance between Sputum and Pulmonary Explant Fragments Culture in Patients with Cystic Fibrosis after Lung Transplantation




Synergistic Activity of Colistin in Combination with N-Acetylcysteine Against Colistin-Resistant Acinetobacter Baumannii Grown in Biofilms

C60: TRANSLATIONAL AND BASIC INVESTIGATIONS IN PULMONARY INFECTION

Area K (Hall A-B2, Ground Level)San Diego Convention Center


Gotta Catch ‘Em All: Delayed Diagnosis of Cystic Fibrosis (CF) in an Infant with a Novel CF Transmembrane Conductance Regulator (CFTR) Mutation

C64: PEDIATRIC CASE REPORTS: AIRWAY DISEASES AND BRONCHOSCOPY



Fatal Outcome in a Patient with Cystic Fibrosis and Candia Blankii Infection: A Case Report





Routine Evaluation for Hypoxia, Hypercarbia, and Pulmonary Hypertension is Infrequent for Patients with Advanced Cystic Fibrosis Prior to Lung Transplant Referral

D23: ADULT CF AND NON-CF BRONCHIECTASIS Room 30 C-E (Upper Level)San Diego Convention Center


Regrets Over a Missed Opportunity to Have a Biological Child — One Couple's Journey After Lung Transplantation for Cystic Fibrosis




Cystic Fibrosis and Twin Pregnancy, the Challenge of Multidisciplinary Care: A Case Report




hCFTR (human Cystic Fibrosis Transmembrane Conductance Regulator) mRNA Delivered Using a Novel Biodegradable Nanovector to CFTR Knock-out Rats Improves CFTR Expression and Chloride Transport Across the Nasal Epithelium in vivo

D110: EPITHELIAL FUNCTION IN HEALTH AND DISEASE Pacific Ballroom 24-26 (North Tower, First Floor)Marriott Marquis San Diego Marina

DERMATOMYOSITIS/ANTISYNTHETASE SYNDROME



Long Term Pulmonary Function in a Cohort of Interstitial Lung Disease Patients Positive to Antisynthetase Antibodies

A21: CLINICAL AND MECHANISTIC STUDIES OF LUNG FIBROSIS

Pacific Ballroom 15-17 (North Tower, First Floor) Marriott Marquis San Diego Marina

13

Rare Disease Term




Refractory Rapidly Progressive Interstitial Lung Disease in Anti-Melanoma Differentiation Antibody with Clinically Amyopathic Dermatomyositis



MDA-5 Positive Dermatomyositis Complicated by Pneumomediastinum



Organizing Pneumonia as the Presenting Symptom of Anti-MDA5 Amyopathic Dermatomyositis



Successful Application of Rituximab and ECMO in a Patient with Rapidly Progressive Interstitial Lung Disease Related to Anti-MDA-5 Antibody Positive Amyopathic Dermatomyositis



Semi-Quantitative CT Chest Scoring System for Anti-Synthetase Syndrome Associated ILD

A41: ILD SCIENTIFIC ABSTRACTS: DIAGNOSIS, OUTCOMES, AND PPFE




Recognizing the Pattern: Anti- MDA 5 Associated Clinically Amyopathic Dermatomyositis (CADM) with Rapidly Progressive Interstitial Lung Disease (RP-ILD) Presenting as Multi-Focal Pneumonia



A Pulmonary Prodrome of Anti-PL7 Positive Anti-Synthetase Syndrome



Triple the Trouble: A Case Report B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


Why Won't this Pneumonia Go Away B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


Rapidly Fatal Anti Synthetase Syndrome Presenting with Pneumonia B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


A Catastrophic Presentation of Antisynthetase Syndrome with Rapidly Progressing Interstitial Lung Disease and Its Antibodies



Rapidly Progressive Interstitial Lung Disease in Amyopathic Dermatomyositis: When Skin Meets Lung




Synthesising the Correct Diagnosis in the Work-Up of Interstitial Lung Disease: Myositis Panel May Be Key


14

Rare Disease Term




Management of Spontaneous Pneumomediastinum in Dermatomyositis with Cyclosporine



A Fatal Case of Anti-MDA5 Antibody Positive Clinically Amyopathic Dermatomyositis (CADM) Associated with Rapidly Progressive Interstitial Lung Disease



Myositis Associated ILD in a Patient with Autoantibody to PMScl D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center

1:30 pm-3:30 pm;Oral Presentation 2:45 pm-3:00 pm

Polymyositis and Dermatomyositis in Lung Transplantation: The National U.S. Experience

D93: LUNG TRANSPLANT: CLINICAL RESEARCH Room 32 A-B (Upper Level)San Diego Convention Center

DIFFUSE ALVEOLAR HEMORRHAGE



Asymmetric Migrating Diffuse Alveolar Hemorrhage A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center



Sometimes It Can Be Lupus: Diffuse Alveolar Hemorrhage (DAH) in SLE



Diffuse Alveolar Hemorrhage After a Nephrectomy... What's the Association?

B45: CRITICAL CARE CASE REPORTS: ACID-BASE, ELECTROLYTES, ENDOCRINE, METABOLIC, AND RENAL ABNORMALITIES



Diffuse Alveolar Hemorrhage Induced by Vaping B51: CIGARETTES, E-CIGARETTES, AND HOOKAHS Area B (Hall A-B2, Ground Level)San Diego Convention Center



A Novel Presentation of Pulmonary Hemorrhage in Infancy C67: PEDIATRIC CASE REPORTS: INTERSTITIAL LUNG DISEASE



Diffuse Alveolar Hemorrhage Following Generalized Tonic Clonic Seizure in a 14 Year-Old Boy




Goodpasture Syndrome in a 15 Month Old Presenting with Anemia and Pulmonary Infiltrates




Double-Positive Goodpasture's Syndrome in a Child: Use of Rituximab




P908 9:15 am-4:15 pm;11:15 am-1:00 pm

Pulmonary Renal Syndrome: Diffuse Alveolar Hemorrhage in Association with ANCA Negative Pauci-Immune Glomerulonephritis

D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level) San Diego Convention Center

15

Rare Disease Term




Amiodarone and Fluconazole: A Deadly Combination D35: DRUG INDUCED LUNG DISEASE: CASE REPORTS Area J (Hall A-B2, Ground Level)San Diego Convention Center

DIFFUSE IDIOPATHIC NEUROENDOCRINE CELL HYPERPLASIA



Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia(DIPNECH): Our Institutional Experience with Somatostatin Analog Therapy





A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia Diagnosed with Transbronchial Lung Cryobiopsy




DIPNECH: A Lung Lined by Tumor D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center


Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH), An Under Recognized Clinical Entity

D38: COUGH: CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): What Is It And What Should You Know?

D62: TYPES, GENOTYPES, AND PHENOTYPES: THE THREE "T's" OF THORACIC ONCOLOGY


DUCHENNE MUSCULAR DYSTROPHY



Comparison of Longitudinal Changes in Expiratory Respiratory Function Endpoints and Inspiratory Flow Reserve (IFR) in Patients with Duchenne Muscular Dystrophy (DMD)

A47: NEUROMUSCULAR DISEASE AND RESPIRATION Area L (Hall A-B2, Ground Level)San Diego Convention Center



The Natural History of Declining Pulmonary Function in Children with Duchenne Muscular Dystrophy

C76: CHEST WALL, RESPIRATORY MUSCLES: NEURAL AND VENTILATORY CONTROL



Diagnostic Differences in Work of Breathing (WOB) Indices And Respiratory Breathing Patterns in Healthy And Duchenne Muscular Dystrophy (DMD) Children




Effect of idebenone on respiratory function across various subgroups in patients with Duchenne Muscular Dystrophy (DMD)




Reliability Of Inspiratory Flow Reserve (IFR) Endpoints And Robustness Of Longitudinal Analyses Assessing The Effect Of Idebenone On Ifr In Patients With Duchenne Muscular Dystrophy (DMD)



16

Rare Disease Term


EOSINOPHILIC DISORDERS — PULMONARY



Retrospective Case Series of Thirty-Two Patients with Biopsy-Proven Chronic Eosinophilic Pneumonia




Idiopathic Hyper-Eosinophilic Syndrome Presenting with a Cavitary, Eosinophilic Lung Abscess



Up In Smoke: A Case of Eosinophilic Pneumonia After Hookah Use B51: CIGARETTES, E-CIGARETTES, AND HOOKAHS Area B (Hall A-B2, Ground Level)San Diego Convention Center



Acute Eosinophilic Pneumonia in the Setting of Hypogammaglobulinemia

C35: CLINICAL STUDIES IN IMMUNODEFICIENCY Area H (Hall A-B2, Ground Level)San Diego Convention Center


The Jamaican Shuffle C51: CRITICAL CARE CASE REPORTS: YOU GIVE ME FEVER — INFECTION AND SEPSIS



Treatment of Childhood Hypereosinophilic Syndrome with Mepolizumab





An Unusual Presentation of Chronic Eosinophilic Pneumonia D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center


A Case of Dapsone Induced Eosinophilic Pneumonia D35: DRUG INDUCED LUNG DISEASE: CASE REPORTS Area J (Hall A-B2, Ground Level)San Diego Convention Center

EXPOSURE-RELATED IDIOPATHIC INTERSTITIAL PNEUMONIA



Idiopathic Pulmonary Fibrosis in a Rail Yard Worker A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center


Domestic and Non-Occupational Exposures in Developing Countries: A Case of Desert Lung Syndrome

A54: INDOOR AND OUTDOOR AIR POLLUTION Area A (Hall A-B2, Ground Level)San Diego Convention Center


Cocaine and Hard Metals: Partners in Crime or Guilty by Association?

A54: INDOOR AND OUTDOOR AIR POLLUTION Area A (Hall A-B2, Ground Level)San Diego Convention Center



Biomass Smoke-Associated Interstitial Lung Disease B24: AN UPDATE ON INDOOR AND OUTDOOR AIR POLLUTION



Hard Metal Lung Disease Presenting in a Surface Coal Miner B49: OCCUPATIONAL EXPOSURES Area B (Hall A-B2, Ground Level)San Diego Convention Center

17

Rare Disease Term




Drilling Down on Hard Metal Lung Disease: An Unusual Case of a Dental Lab Technician with Rapidly Progressive Interstitial Lung Disease


FAMILIAL INTERSTITIAL PNEUMONIA AND OTHER GENETIC ILD


116 9:15 am-11:15 am;Poster Viewing 9:15 am-9:45 amPoster Discussion 9:45 am-11:15 pm

RTEL1 Mutations Are Associated with Heterogeneous Pulmonary and Extra-Pulmonary Phenotypes



Surfactant Protein C Deficiency and Rheumatoid Arthritis Interstitial Lung Disease in Pregnancy — A Fatal Combination



The MUC5B Promoter Variant Is an Independent Risk Factor of Idiopathic Interstitial Pneumonitis (IIP) in Families with Multiple Cases of IIP



Understanding the Cellular Dysfunction Caused by Pathogenic Surfactant Protein C Mutant I73T

A68: MOLECULAR DETERMINANTS OF REMODELING IN LUNG FIBROSIS



Assessing Pulmonary Fibrosis Progression in a Model of Surfactant Protein C Mutation with Ultra-Short Echo-Time Magnetic Resonance Imaging

A73: DIFFUSE PARENCHYMAL LUNG DISEASE: NOVEL MECHANISMS, BIOMARKERS, AND THERAPEUTICS



Re-Organization Of Air Spaces In The Surfactant Protein C Aging Lung And Lipid Homeostasis




Clinical Features and Outcomes Associated with Telomere Pathway Mutations in Familial Interstitial Pneumonia




Expression Of An Interstitial Lung Disease Associated Surfactant Protein C SFTPC) Mutation Produces Dynamic Alterations In The Alveolar Type 2 Epithelial Cell Transcriptome And A Fibrotic Lung Phenotype In Mice

B107: MECHANISTIC ADVANCES IN PULMONARY FIBROSIS




Computed Tomography Images and Electron Microscopy: Clues for an ABCA-3 Deficiency Case Report in Colombia




At the Opposite End of the Spectrum: Filamin A-Associated Interstitial Lung Disease



18

Rare Disease Term




A Novel Mutation in the SFPTC Gene in a Newborn with Persistent Tachypnea




Heterozygous Surfactant Protein B Disease Leading to Respiratory Failure




Desquamative Interstitial Pneumonia in a Child: A Case Report C67: PEDIATRIC CASE REPORTS: INTERSTITIAL LUNG DISEASE



ABCA3 Mutation Associated with Interstitial Lung Disease in a Newborn: Case Report





Multiplexed Direct Genomic Selection to Identify Non-coding and Copy Number Variation in ABCA3 among Neonates with Respiratory Failure and Children with Interstitial lung Disease (chILD) with Monoallelic ABCA3 Variants




Abnormal Surfactant Protein Expression and Activation of Anti-Apoptotic Pathways in Severe Interstitial Lung Disease (ILD) in Rubinstein-Taybi Syndrome (RTS)




Fibrosing Mediastinitis Due to Histoplasma: A Rare but Potentially Treatable Entity

D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center


Myhre Syndrome in Montana, A Case Report D39: DYSPNEA: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center


Silicosis-Associated Fibrosing Mediastinitis a Rare Cause of Pulmonary Hypertension




Is Venous Thromboembolism a Risk Factor for Pulmonary Hypertension in Fibrosing Mediastinitis?




Multidisciplinary Team Dedicated to Suspected Heritable Pulmonary Fibrosis One Year Experience.


FOLLICULAR BRONCHIOLITIS



Follicular Bronchiolitis Mimicking Mycobacterial Infection: Case Report


19

Rare Disease Term




Follicular Bronchiolitis Associated with Behcet's Disease D32: ILD: CASE REPORTS II Area H (Hall A-B2, Ground Level)San Diego Convention Center

GLYCOGEN STORAGE AND METABOLIC DISORDERS



Niemann-Pick Disease Presenting as Familial Interstitial Lung Disease: Report of 2 Adult Siblings




When the Phrenic Nerve Fails You C54: CRITICAL CARE CASE REPORTS: CAUSES AND COMPLICATIONS OF ACUTE RESPIRATORY FAILURE


HEMOGLOBINOPATHY



Low Oxygen Affinity Hemoglobinopathy: A Rare Sixth Cause of Hypoxemia


HEPATOPULMONARY SYNDROME



A Rare Case of Idiopathic Noncirrhotic Portal Hypertension Causing Hepatopulmonary Syndrome





Epoprostenol for Worsening Hypoxemia of Hepatopulmonary Syndrome After Lung Transplantation

C50: CRITICAL CARE CASE REPORTS: THE LIVER, PANCREAS, AND INTESTINES




Pentoxifylline Reduces Intrapulmonary Shunt in a Patient with Hepatopulmonary Syndrome

D39: DYSPNEA: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center


Hepatopulmonary Syndrome Masquerading as Interstitial Lung Disease


HEREDITARY HEMORRHAGIC TELANGIECTASIS



Long-Term Management of HHT-Related PAVM: Lessons Learned on the Need for More HHT Centres of Excellence



Hereditary Hemorrhagic Telangiectasia and a Recurrent Brain Abscess



HERMANSKY-PUDLAK SYNDROME



Platelet Delta Granule Evaluation in Lung Transplant Recipients with HPS-1 to Study Platelet Biogenesis


20

Rare Disease Term




Galectin-3 Interacts with CHI3L1 Axis and Contributes to Hermansky-Pudlak Syndrome Lung Disease

C72: PULMONARY FIBROSIS: MECHANISMS AND MODELS



The Endocannabinoid/CB<sub>1</sub>R System is Overactive in Bleomycin-induced Pulmonary Fibrosis in Pale Ear (HPS1) mice




Circulating Fibrocytes Predict Biomarkers Of Immune Dysregulation And Fibrogenesis In A Genetic Interstitial Lung Disease





An Unusual Cause of Pulmonary Fibrosis D32: ILD: CASE REPORTS II Area H (Hall A-B2, Ground Level)San Diego Convention Center

HYPERSENSITIVITY PNEUMONITIS



Evidence for an Organizing Adaptive Immune Response in Explant Lungs with Chronic Hypersensitivity Pneumonitis




Chronic Hypersensitivity Pneumonitis: A Prospective Cohort Analysis of an Under-Recognized Clinical Entity



Hypersensitivity Pneumonitis and Sarcoidosis A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center


Pet Groomers Survey Responses A54: INDOOR AND OUTDOOR AIR POLLUTION Area A (Hall A-B2, Ground Level)San Diego Convention Center



Clinical Relevance Of Antinuclear Antibodies (ANA) In Patients With Hypersensitivity Pneumonitis (HP): A Single Centre Experience.




The Little Farmer’s Lung: Hypersensitivity Pneumonitis Due to Multiple Fungal Triggers in a 6-Year-Old




The Moldy Birds: An Adolescent with Progressive Dyspnea C67: PEDIATRIC CASE REPORTS: INTERSTITIAL LUNG DISEASE




A Complex Case of Interstitial Lung Disease Related to Nontuberculous Mycobacteria and Hot Tub Exposure



Fibrotic Hypersensitivity Pneumonitis Induced by Oxaliplatin: A Unexpected but Serious Side Effect in Treating Colorectal Cancer

D35: DRUG INDUCED LUNG DISEASE: CASE REPORTS Area J (Hall A-B2, Ground Level)San Diego Convention Center

21

Rare Disease Term




A Case of Ziprasidone-Induced Hypersensitivity Pneumonitis: A Previously Unreported Side Effect of an Atypical Antipsychotic

D46: CRITICAL CARE CASE REPORTS: TOXICOLOGY AND POISONINGS


IDIOPATHIC PULMONARY HEMOSIDEROSIS



A Recurrent Hemizygous Deletion at the MHC Region Impacting MICA, LINC01149, HCP5, HCG26 Associates with Idiopathic Pulmonary Hemosiderosis



IGG4-RELATED DISEASE



Lung Involvement in Immunoglobulin G4 Related Disease A39: SARCOIDOSIS AND RARE LUNG DISEASE SCIENTIFIC ABSTRACTS




IgG4 Related Lung Disease Found in a Patient with a Pancreatic Mass



Revealing the Mystery of a Previously Diagnosed Inflammatory Pseudotumor of the Lung



Evaluation of Lung Lesions in LATY136F Mutant Mice B32: CLINICAL STUDIES IN AUTOIMMUNE LUNG DISEASE Area G (Hall A-B2, Ground Level)San Diego Convention Center


A New Foe: IgG4-Related Disease Presenting as Isolated Pulmonary Nodules



Dual Manifestations of Immunoglobulin G4 Related Disease in Thorax



Unusual Presentation of IgG4 Related Disease B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center

INTERSTITIAL LUNG DISEASES — CHILDHOOD OTHER



The National Registry for Childhood Interstitial and Diffuse Lung Diseases: An Initial Report from the ChILD Research Network

B97: IMPORTANCE OF LONGITUDINAL COHORT STUDIES IN PEDIATRICS

Room 6 C/F (Upper Level)San Diego Convention Center



Recurrent Life Threatening Pneumothorax Associated with Diffuse Subpleural Cystic Lung Disease in a Trisomy 21 Infant with Congenital Heart Disease: A Unique Case Report!




Diagnosis and Treatment of Cryptogenic Organizing Pneumonia in a Child on Extracorporeal Membrane Oxygenation Support



22

Rare Disease Term




Combined Heterozygous TBX4 Loss and Single ABCA3 Mutation in the Pathogenesis of Childhood Interstitial Lung Disease




Tracheostomy Bleeding Unfolding into an Unusual Etiology C67: PEDIATRIC CASE REPORTS: INTERSTITIAL LUNG DISEASE




Assessment of Late Pulmonary Outcomes and Exercise Capacity among Pediatric Survivors of High-Risk Neuroblastoma




Childhood Interstitial Lung Disease in a Large Tertiary Care Center: Implications of a Family Needs Assessment Survey



LIGHT CHAIN DEPOSITION DISEASE



Pulmonary Only Light Chain Deposition Disease in a Young Healthy Male



Light-Chain Deposition Disease: A Rare Cause of Cystic Lung Disease



Cystic Lung Disease Due to Primary Light Chain Deposition D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center

LIPOID PNEUMONIA



Progressive Chronic Hypoxemia After Chronic Constipation: A Case Report



A Case of Lipoid Pneumonia with Exogenous Source A73: DIFFUSE PARENCHYMAL LUNG DISEASE: NOVEL MECHANISMS, BIOMARKERS, AND THERAPEUTICS




A Case of Exogenous Lipoid Pneumonia Caused by Sesame Oil Pulling Successfully Treated with Whole Lung Lavage




Lipoid Pneumonia, An Unexpected Diagnosis in a Healthy Middle-Aged Patient


LYMPHANGIOLEIOMYOMATOSIS



Identification of Novel Biomarkers for Lymphangioleiomyomatosis by Proteomics from Serum Exosomes



23

Rare Disease Term




Pseudoneutropenia in Lymphangioleiomyomatosis Patients Receiving Sirolimus




Serum Levels of VEGF-C Are Elevated in Sporadic but Not Tuberous Sclerosis Associated-LAM, Correlate with the Need for Supplemen-tal Oxygen, and Impact Lung Function Response to Sirolimus



Clinical Characteristics in Lymphangioleiomyomatosis-Related Pulmonary Hypertension: An Observational Study




A Rare Combination of Lymphangioleiomyomatosis with Hypersensitivity Pneumonia



Does Chest Computed Tomography Body Positioning Change the Cyst Score for Lymphangioleiomyomatosis (LAM)?




Effect of Model-Based Iterative Reconstruction in Chest Computed Tomography on Lymphangioleiomyomatosis (LAM) Cyst Score Quantification




Correlations between Lung Parenchymal Changes on Computed Tomographic Imaging and both Disease Progression and Response to Sirolimus Therapy in Lymphangioleiomyomatosis




Single Cell RNAseq of Lymphangioleiomyomatosis (LAM) Lungs Reveals Unique Populations Consistent with LAM Cells and Lymphatic Endothelial Cells

A96: THE STEM AND PROGENITOR CELL NICHES AS THERAPEUTIC TARGETS

Grand Ballroom 5-6 (North Tower, Lobby Level)Marriott Marquis San Diego Marina


Potential Serum Biomarkers in Lymphangioleiomyomatosis A109: NOVEL EXPERIMENTAL MODELS AND APPROACHES IN LUNG BIOLOGY




Lymphangioleiomyomatosis-Derived Smooth Muscle Cells (LAM-SMCs) Destabilize Endothelial Cell (EC) Networks While ECs Promote Growth of LAM-SMCs

B28: CELL AND MATRIX MICROENVIRONMENTS MODULATE LUNG DISEASE



Lung Function Response And Changes Of Lung Cysts To Rapamycin For Lymphangioleiomyomatosis: Long-term Follow-up Observational Study

B103: ILD: MANAGEMENT Room 6 D (Upper Level)San Diego Convention Center

24

Rare Disease Term




Hospitalization Patterns in Patients with Lymphangioleiomyomatosis (LAM) in the United States




Quantification Of Cyst Ventilation Using Hyperpolarized 129Xe MRI Correlates With Pulmonary Function Tests In Patients With Lymphangioleiomyomatosis.

C97: DIFFUSE PARENCHYMAL LUNG DISEASES: EVALUATION, OUTCOMES, AND TRIALS

Grand Ballroom 5-6 (North Tower, Lobby Level)Marriott Marquis San Diego Marina


9:15 am-11:15 am;Oral Presentation 10:00 am-10:15 am

The NHLBI Lymphangioleiomyomatosis Registry: Prognostic Radiologic and Physiologic Biomarkers Emerge from a 15-Year Prospective Longitudinal Analysis

D14: ILD: CLINICAL RESEARCH Room 3 (Upper Level)San Diego Convention Center


Lymphangioleiomyomatosis (LAM) of the Uterus: Risk of Pulmonary LAM?



A Rare Case of Cocaine User with Sporadic Lymphangioleiomyomatosis



Pneumothorax in a Woman with Tuberous Sclerosis — the Trigger to Diagnosis of Lymphangioleiomyomatosis



Pulmonary Lymphangioleiomyomatosis: A Prime of Life Misfortune D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center


Genetic Features Of Metastatic Tsc2-null Cells: Relevance To Pulmonary LAM

D52: DECONSTRUCTING TUMORS AND MALIGNANCIES Area D (Hall A-B2, Ground Level)San Diego Convention Center


IGF2 As A Potential Novel Therapeutic Target For Rapamycin-insensitive LAM Patients



Translationally Controlled Tumour Protein: A Novel Therapeutic Target for Lymphangioleiomyomatosis?



PD-L1 As a Notential Therapeutic Target For Patients With Advanced or Refractory-to-Rapamycin Pulmonary lymphangioleiomyomatosis (LAM)



Targeted Serum Proteomic Analysis Identifies Novel Biomarkers in LAM



Tuberous Sclerosis and Lymphangioleiomyomatosis are Neurocristopathies


25

Rare Disease Term


LYMPHATIC ANOMALIES: GENERALIZED LYMPHATIC ANOMALY (GLA), GORHAM-STOUT DISEASE (GSD)



Assessment of Alveolar-Airspace Dimensions in a Case of Pediatric Generalized Lymphatic Anomaly





Chylous Effusion in an Infant: Presenting Symptom of Lymphangiomatosis





Lymphangiomatosis D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center


Plastic Bronchitis: A Tale of Lymphatic Anomalies D38: COUGH: CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


Vanishing Bone Disease D39: DYSPNEA: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

LYMPHOVENOUS MALFORMATION



A Curious Bronchoscopic Finding in a Patient with Tracheal Submucosal Lymphovenous Malformation



MARFAN’S SYNDROME



Sildenafil Ameliorates Airspace Widening And Early Pulmonary Artery Dilation In The Mouse Model Of Marfan Syndrome

B66: WHAT'S NEW IN VASCULAR AND PLEURAL DISEASE Area J (Hall A-B2, Ground Level)San Diego Convention Center



Massive Hemoptysis in a Patient with Marfan’s Syndrome D38: COUGH: CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center

MOUNIER-KUHN SYNDROME



Mounier-Kuhn Syndrome Complicated by Mycobacterium Abscessus Infection in an Active Duty Solider

A56: NON-TUBERCULOUS MYCOBACTERIA: CASE REPORTS




Tracheobroncomegaly "Mounier-Kuhn Syndrome" D37: CF AND NON-CF BRONCHIECTASIS AND IMMUNE DEFICIENCIES: CASE REPORTS



A Large Airway Problem: A Case of an Elderly Female with Mounier-Kuhn Syndrome




Mounier Kuhn Syndrome; a Magnified Incidental Finding D39: DYSPNEA: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

26

Rare Disease Term


NEUROENDOCRINE CELL HYPERPLASIA OF INFANCY



Lung Shape From Chest Computed Tomography Scan Predicts Diagnosis of Neuroendocrine Cell Hyperplasia of Infancy



NEUROFIBROMATOSIS



Neurofibromatosis 1 Presenting with a Large Mediastinal Mass B72: CASE REPORTS IN THORACIC ONCOLOGY IV Area D (Hall A-B2, Ground Level)San Diego Convention Center


A New Diagnosis of Neurofibromatosis Type 1 in a Young Adult Male Presenting with Palpitations and Posterior Mediastinal Mass

B73: CASE REPORTS IN THORACIC ONCOLOGY V Area D (Hall A-B2, Ground Level)San Diego Convention Center

NON-CF BRONCHIECTASIS



Characteristics of Alaska Native Children with Chronic Suppurative Lung Disease: A Prospective 10-year Study





Effect of Inhaled Liposomal Ciprofloxacin on Severe Pulmonary Exacerbations in Patients with Bronchiectasis and Chronic Pseudomonas aeruginosa Lung Infections



Safety and Tolerability of ARD-3150, Inhaled Liposomal Ciprofloxacin, in Patients with Bronchiectasis and Chronic Pseudomonas aeruginosa Infection: Results from two Phase 3 trials


NON-LANGERHANS HISTIOCYTOSES



Interesting Case of Mediastinal Adenopathy B37: INTERVENTIONAL PULMONARY: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


Bilateral Pulmonary Nodules and Intravascular Pulmonary Histiocytosis: A Rare Presentation of Hemophagocytic Lymphohistiocytosis Secondary to Epstein-Barr Virus Infection

B46: CRITICAL CARE CASE REPORTS: HEMATOLOGY, ONCOLOGY, RHEUMATOLOGY, AND IMMUNOLOGY




So It’s Not a Pulmonary Embolism? A Rare Case of Cardiac Rosai-Dorfman Disease



NON-TUBERCULOUS MYCOBACTERIAL INFECTION — PULMONARY



In Vitro Determination of Inhaled NO Regimen for Treatment of Pulmonary Non-Tuberculous Mycobacteria Infection

A58: NON-TUBERCULOUS MYCOBACTERIA: OFF THE BEATEN TRACK


27

Rare Disease Term




Patient-Reported Health Status Measured by St. George’s Respiratory Questionnaire Improved with Sputum Culture Conversion in Patients with Nontuberculous Mycobacterial (NTM) Lung Disease Caused by Mycobacterium Avium Complex (MAC)

A58: NON-TUBERCULOUS MYCOBACTERIA: OFF THE BEATEN TRACK


ORGANIZING PNEUMONIA



Acute Fibrinous and Organizing Pneumonia After Allogeneic Stem Cell Transplant



A Rare Radiographic Presentation of Cryptogenic Organizing Pneumonia: A Diffuse Micronodular Pattern Mimicking Miliary Lung Infiltration



Frankincense for Organizing Pneumonia: A Steroid-Sparing Gift? A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center


Recurrent Acute Fibrinous and Organizing Pneumonia in a 40-Year-Old Woman




A Case of Acute Interstitial Pneumonia with Autoimmune Features Presenting as Granulomatous Organizing Pneumonia



An Atypical Presentation of Cryptogenic Organizing Pneumonia B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


Cocaine Associated Organizing Pneumonia: A Fatal Low to Getting High




Cryptogenic Organizing Pneumonia as a Precursor of Primary Hodgkin's Disease: An Unreported Entity

B74: CASE REPORTS IN THORACIC ONCOLOGY VI Area D (Hall A-B2, Ground Level)San Diego Convention Center



Not so “Cryptogenic”: A Case of Organizing Pneumonia (OP) Associated with Common Variable Immunodeficiency (CVID)




Focal Organizing Pneumonia in a Severe Hemophiliac: A Diagnostic Dilemma



Fatal Acute on Chronic Respiratory Failure Due to Cryptogenic Organizing Pneumonia in the Setting of Systemic Lupus Erythematosus


28

Rare Disease Term




Cryptogenic Organizing Pneumonia After Allogeneic Hematopoietic Stem Cell Transplant, Difficult to Diagnose, Easy to Treat



A Rare Case of Cocaine-Induced Organizing Pneumonia D34: LUNG TRANSPLANT AND DRUG INDUCED LUNG DISEASE: CASE REPORTS



Acute Fibrinous and Organizing Pneumonia (AFOP) Associated with Chemotherapy Drug Reaction: A Case Report



Bortezomib Induced Organizing Pneumonia in a Patient with Underlying Interstitial Lung Disease: The (Im)Perfect Setup


OSSIFICATION AND CALCIFICATION — PULMONARY



Pulmonary Alveolar Microlithiasis with Satisfactory Diagnosis by Bone Scintigraphy of the Lung



Osteogenesis and Osteolysis in Pulmonary Alveolar Microlithiasis A109: NOVEL EXPERIMENTAL MODELS AND APPROACHES IN LUNG BIOLOGY



Mechanisms of Microlith Clearance Induced by Dietary Phosphate Restriction in Pulmonary Alveolar Microlithiasis

A109: NOVEL EXPERIMENTAL MODELS AND APPROACHES IN LUNG BIOLOGY




Pulmonary Ossification: Not a Bone of Contention B37: INTERVENTIONAL PULMONARY: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center



EGTA Therapeutic Lung Lavage For Pulmonary Alveolar Microlithiasis

C43: TOPICS IN IN INTERVENTIONAL PULMONARY AND PLEURAL DISEASES



Ivory Lungs: A Rare Case of Pulmonary Alveolar Microlithiasis Bridged to Transplant by AV ECMO

C52: CRITICAL CARE CASE REPORTS: GOOD VIBRATIONS — MECHANICAL VENTILATION FROM NIV TO ECMO



Pulmonary Alveolar Microlithiasis: A Case of an Early Presentation C67: PEDIATRIC CASE REPORTS: INTERSTITIAL LUNG DISEASE




Primary Pulmonary Calciphylaxis Found Six-Months After Simultaneous Liver-Kidney Transplantation



29

Rare Disease Term


PLACENTAL TRANSMOGRIFICATION



Placental Transmogrification of the Lung in a Patient with Anti-Synthetase Syndrome




Giant Cystic Lesions with a Rare Pulmonary Diagnosis C67: PEDIATRIC CASE REPORTS: INTERSTITIAL LUNG DISEASE


PLASTIC BRONCHITIS



Rare Case of Idiopathic Plastic Bronchitis in Adult A48: DYSPNEA: CASE REPORTS I Area L (Hall A-B2, Ground Level)San Diego Convention Center



Idiopathic Plastic Bronchitis: A Rare Presentation B37: INTERVENTIONAL PULMONARY: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


A Case of Plastic Bronchitis B37: INTERVENTIONAL PULMONARY: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center



Plastic Bronchitis in an Adult Lung Transplant Patient with Refractory Donor-Related Asthma

C32: LUNG TRANSPLANTATION: CLINICAL STUDIES Area H (Hall A-B2, Ground Level)San Diego Convention Center


Old Disease-New Perspectives: Plastic Bronchitis C64: PEDIATRIC CASE REPORTS: AIRWAY DISEASES AND BRONCHOSCOPY




Characterizing Bronchoalveolar Lavages and Bronchial Casts in Patients with Congenital Heart Disease and Plastic Bronchitis at Time of Percutaneous Lymphatic Intervention




"Doctor, I Coughed Up My Lung!" D38: COUGH: CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


“Casting” for a Diagnosis: A Case of Plastic Bronchitis in an Adult Patient Following Cardiopulmonary Bypass


PLEUROPARENCHYMAL FIBROELASTOSIS (PPFE)



Pleuroparenchymal Fibroelastosis Following Autologous Bone Marrow Transplant



What You Don’t Know Can Hurt You: A Rare Form of Pulmonary Fibrosis in the Setting of Chronic Cyclophosphamide


30

Rare Disease Term




Pleuroparenchymal Fibroelastosis a Case Report A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center


Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis




Clinical Features and Prognosis of Pleuroparenchymal Fibroelastosis in Korean





Familial Pleuroparenchymal Fibroelastosis D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center

P1068 9:15 am-4:15 pm; Poster Viewing11:15 am-1:00 pm

Pleuroparenchymal Fibroelastosis (PPFE): A Rare Disease Entity, Secondary to Methotrexate?


PRIMARY CILIARY DYSKINESIA



Neonatal Chest X-ray Findings in Patients with Primary Ciliary Dyskinesia




Where Is The NO In Primary Ciliary Dyskinesia (PCD)? Somatic Cell Heme Oxidizes N0 in Ciliated Human Epithelial Cells Homozygous For The PCD Mutation, DNAH5




Investigation of “Read-Through” of Nonsense Mutations as a Treatment for Primary Ciliary Dyskinesia




NIOX VERO® Nasal Measurement Mode Differentiates Individuals with PCD from Healthy Individuals with Two Breathing Methods




Primary Ciliary Dyskinesia: Longitudinal Study of Lung Disease Progression by Ultrastructural Defect and Genotype

B97: IMPORTANCE OF LONGITUDINAL COHORT STUDIES IN PEDIATRICS

Room 6 C/F (Upper Level)San Diego Convention Center



Different Mutations in Cilia Preassembly Gene HEATR2 Are Associated with Variable Clinical Phenotypes and Defects in Proteostasis

C18: BEST OF PEDIATRICS Room 1 A-B (Upper Level)San Diego Convention Center



The Clinical Features of Bronchiectasis Associated with Alpha-1 Antitrypsin Deficiency, Common Variable Immunodeficiency, and Primary Ciliary Dyskinesia


31

Rare Disease Term




Longitudinal Analysis of Pulmonary Exacerbations: Results from ORBIT-3 and ORBIT-4 trials of ARD-3150 in Patients with Non Cystic Fibrosis Bronchiectasis and Chronic Infection with Pseudomonas aeruginosa



Mutations in C11ORF70 Cause Primary Ciliary Dyskinesia with Randomization of Left-Right Body Asymmetry Due to Outer and Inner Dynein Arm Defects

D110: EPITHELIAL FUNCTION IN HEALTH AND DISEASE Pacific Ballroom 24-26 (North Tower, First Floor)Marriott Marquis San Diego Marina

PULMONARY ALVEOLAR PROTEINOSIS



Differential Diagnosis of Pulmonary Alveolar Proteinosis: Lung Biopsy or Blood Test?



Secondary Pulmonary Alveolar Proteinosis Predominant in the Transplanted Left Lung in Patients with Idiopathic Interstitial Pneumonia: An Autopsy Case



Pulmonary Alveolar Proteinosis: A Compositional and Biophysical Functional Analysis of its Effects on Lung Surfactant





Generation of cDNA Library Encoding GM-CSF Autoantibody Sequences In Autoimmune Pulmonary Alveolar Proteinosis by Next Generation Sequencing



Nocardia Survival and Virulence in Pulmonary Alveolar Proteinosis B32: CLINICAL STUDIES IN AUTOIMMUNE LUNG DISEASE Area G (Hall A-B2, Ground Level)San Diego Convention Center


A Formal Toxicology Study of Inhaled rhGM-CSF in Healthy, Non-Human Primates




Autoimmune Pulmonary Alveolar Proteinosis: Presentation, Clinical Manifestations and Current Therapeutic Approaches




The Effect of Inhaled Granulocyte-macrophage colony stimulating factor(GM-CSF) for Patients with Mild-to-moderate Autoimmune Pulmonary Alveolar Proteinosis (APAP) in China

B103: ILD: MANAGEMENT Room 6 D (Upper Level)San Diego Convention Center

32

Rare Disease Term




Long-term Evaluation of Pulmonary Macrophage Transplantation Therapy in Csf2ra Gene-Deficient Mice, a Clinically Relevant Model of Hereditary Pulmonary Alveolar Proteinosis

C18: BEST OF PEDIATRICS Room 1 A-B (Upper Level)San Diego Convention Center


Induction Of Antibody Against Granulocyte/macrophage Colony Stimulating Factor (gm-csf) Through Repeated Inhalation Of Recombinant Human Gm-csf In No Human Primates.



923 9:15 am-11:15 am;Poster Viewing 9:15 am-9:45 am

Restoration Of Gm-csf Signaling And Pulmonary Macrophage Trans-plantation Therapy Of Hereditary Pulmonary Alveolar Proteinosis Is Safe, Efficacious, And Durable In Csf2ra Deficient Mice




Non-Familial Pulmonary Alveolar Proteinosis in a Pregnancy Complicated by Stenotrophomonas Maltophilia

C35: CLINICAL STUDIES IN IMMUNODEFICIENCY Area H (Hall A-B2, Ground Level)San Diego Convention Center


Pulmonary Alveolar Proteinosis Single Center Case Series Experience

C43: TOPICS IN IN INTERVENTIONAL PULMONARY AND PLEURAL DISEASES




Rare Cause of Secondary Pulmonary Alveolar Proteinosis D32: ILD: CASE REPORTS II Area H (Hall A-B2, Ground Level)San Diego Convention Center


Relapsing Pulmonary Alveolar Proteinosis Treated with GM-CSF D32: ILD: CASE REPORTS II Area H (Hall A-B2, Ground Level)San Diego Convention Center


Pulmonary Alveolar Proteinosis: A Rare Disease, First-Line Treatment Resulting in a Near-Fatal Complication


PULMONARY ARTERY ANEURYSM



Pulmonary Artery Aneurysm: A 10 Year Community Hospital Experience

A64: LET'S GO TRIPPIN': DIAGNOSTICS AND RISK ASSESSMENT IN PULMONARY HYPERTENSION




Giant Pulmonary Artery Aneurysms in a Patient with Pulmonary Arterial Hypertension



PULMONARY INTERSTITIAL GLYCOGENOSIS



Acute Respiratory Failure and Pulmonary Hemorrhage in the Newborn Period: An Unusual Presentation of Pulmonary Interstitial Glycogenosis



33

Rare Disease Term


PULMONARY LANGERHANS CELL HISTIOCYTOSIS



Pulmonary Langerhans Cell Histiocytosis in a Patient with DRESS Syndrome and Acute Respiratory Failure

A31: ALLERGY AND ASTHMA CASE REPORTS Area H (Hall A-B2, Ground Level)San Diego Convention Center


Pneumothorax in Adult Pulmonary Langerhans Cell Histiocytosis A39: SARCOIDOSIS AND RARE LUNG DISEASE SCIENTIFIC ABSTRACTS



Pulmonary Langerhans Cell Histiocytosis-Cladribine Treatment A39: SARCOIDOSIS AND RARE LUNG DISEASE SCIENTIFIC ABSTRACTS



Mystery of Waxing and Waning Lung Nodules A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center


Late Onset Pulmonary Langerhans Cell Histiocytosis (PCLH) and Concurrent Diagnosis of Lung Cancer



Pulmonary Langerhans Cell Histiocytosis in a Heavy Marijuana Smoker



Genetic Alterations in Adult Pulmonary Langerhans Cell Histiocytosis A71: THE EPIGENOME, GENOME AND NON-CODING RNAs IN LUNG DISEASE




Upper Lobe Cystic Lung Disease Mimicking Pulmonary Langerhans Cell Histiocytosis in a Veteran with Burn Pit Exposure

B49: OCCUPATIONAL EXPOSURES Area B (Hall A-B2, Ground Level)San Diego Convention Center



A Lung Cyst in a Sea of Histiocytes: Systemic Langerhans Cell Histiocytosis Diagnosed Via EBUS



A Common Presentation of a Rare Disease: Pulmonary Langerhans Cell Histiocytosis



Pulmonary Langerhans Cell Histiocytosis(PLCH): A Rare Cause of Chronic Cough



Molecular and Cellular Mechanisms of Pulmonary Langerhans Cell Histiocytosis


PULMONARY CAPILLARY HEMANGIOMATOSIS AND PULMONARY VENO-OCCLUSIVE DISEASE (PVOD)



Hypoxemia and Ground-Glass Opacities: A Case of Pulmonary Capillary Hemangiomatosis



34

Rare Disease Term




Cardiac Tamponade: As a Manifestation of Pulmonary Veno-Occlusive Disease (PVOD)




Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension



P432 9:15 am - 4:15 pm;11:15 am - 1:00 pm

Extracorporal Membrane Oxygenation, an Effective Therapy for Pulmonary Veno-Occlusive Disease and Pulmonary Capillary Hemangiomatosis




Pulmonary Capillary Hemangiomatosis with Clinical Improvement on AntiangiogenicTreatment with Doxycycline



PULMONARY WALDENSTRÖM MACROGLOBULINEMIA



Lung Consolidation as a Manifestation of Lymphoplasmacytic Lymphoma with Waldenström's Macroglobulinemia

B70: CASE REPORTS IN THORACIC ONCOLOGY II Area D (Hall A-B2, Ground Level)San Diego Convention Center

RARE CONGENITAL ANOMALIES



A Case of Unilateral Pulmonary Artery Hypoplasia in an Adult Patient A48: DYSPNEA: CASE REPORTS I Area L (Hall A-B2, Ground Level)San Diego Convention Center


Practically Living with One Lung: A Rare Adult Presentation of Congenital Pulmonary Airway Malformation




Intrapulmonary Sequestration: A Rare Congenital Accessory B52: BACTERIAL INFECTION CASE REPORTS Area A (Hall A-B2, Ground Level)San Diego Convention Center


Recurrent Pneumonia in a Healthy Adult: A Rare Case of Congenital Pulmonary Airway Malformation

B52: BACTERIAL INFECTION CASE REPORTS Area A (Hall A-B2, Ground Level)San Diego Convention Center


Living with One Lung B58: WHO'S BAD: CASE REPORTS IN PULMONARY VASCULAR MEDICINE I




An Incidentally Discovered Congenital Pulmonary Airway Malformation




Bilateral and Multilobar Congenital Pulmonary Airway Malformation Type 2: A Rare Case




Bronchial Atresia Associated with a Bronchogenic Cyst C65: PEDIATRIC CASE REPORTS: NEONATAL LUNG DISEASE


35

Rare Disease Term




Charlotte’s Web (of Bowels in the Chest) C65: PEDIATRIC CASE REPORTS: NEONATAL LUNG DISEASE



Congenital Pulmonary Airway Malformation in a Female Infant — Management Challenges in a Developing Country




Do Not Miss the Nuances: Failure to Thrive and Wheezing. A Case of Scimitar Syndrome

C66: PEDIATRIC CASE REPORTS: CARDIOVASCULAR AND CRITICAL CARE DISEASE



Partial Anomalous Pulmonary Venous Return: Different Management Approaches

C69: USE YOUR ILLUSION: NOVEL THERAPEUTIC APPROACHES IN PULMONARY HYPERTENSION




Right Side Aortic Arch with Aberrant Left Subclavian Artery D49: SURFING SAFARI: CASE REPORTS IN PULMONARY VASCULAR MEDICINE II



Severe Pulmonary Artery Hypertension in the Setting of Isolated Unilaterally Absent Pulmonary Artery

D50: CLOT ON THE ROCKS: CASE REPORTS OF PE AND CTEPH


RARE IDIOPATHIC INTERSTITIAL PNEUMONIAS — OTHER



A Rare Case of Bronchiolocentric Interstitial Fibrosis A40: ILD: CASE REPORTS I Area K (Hall A-B2, Ground Level)San Diego Convention Center


Telomere Length and the MUC5B Promoter Polymorphism in Patients with Unclassifiable Interstitial Lung Disease




An Unusual Presentation of CMV Pneumonitis C53: CRITICAL CARE CASE REPORTS: YOU GIVE ME (MORE) FEVER - INFECTION AND SEPSIS




Early Diagnosis and Treatment of Acute Interstitial Pneumonia D33: ILD: CASE REPORTS III Area H (Hall A-B2, Ground Level)San Diego Convention Center


Interstitial Lung Disease in a Patient with Quiescent Ulcerative Colitis


RARE PLEURAL DISEASE



A Unusual Case of Dyspnea: A Pancreaticopleural Fistula A48: DYSPNEA: CASE REPORTS I Area L (Hall A-B2, Ground Level)San Diego Convention Center


Pulmonary Extramedullary Hematopoiesis in a Patient with Polycythemia Vera


36

Rare Disease Term




A Novel Use of Intrapleural t-PA/DNase Therapy for Loculated Chylothorax



Multi-Modality Management of a Lone Mediastinal Silicosis Presenting as an Extremely Rare Case of Chylothorax



Chylous Pleural Effusion: A Rare Complication of Chronic Lymphocytic Leukemia



An Exudative Urinothorax with Low Creatinine and Low pH B39: PLEURAL DISEASE: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


Fungus Among Us: A Rare Case of Infected Chylothorax B39: PLEURAL DISEASE: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


Bilothorax: Rare Cause of Respiratory Failure After Percutaneous Liver Biopsy



New Onset Atrial Fibrillation Masking a Nephropleural Fistula B52: BACTERIAL INFECTION CASE REPORTS Area A (Hall A-B2, Ground Level)San Diego Convention Center


An Extremely Rare Case of Transudate Chylothorax Secondary to Carcinoid Tumor

B72: CASE REPORTS IN THORACIC ONCOLOGY IV Area D (Hall A-B2, Ground Level)San Diego Convention Center



Chylothorax After Abdominal Surgery — A Fatal Outcome C50: CRITICAL CARE CASE REPORTS: THE LIVER, PANCREAS, AND INTESTINES



Coprothorax: A Feculent Pleura C54: CRITICAL CARE CASE REPORTS: CAUSES AND COMPLICATIONS OF ACUTE RESPIRATORY FAILURE



Tension Faecopneumothorax: A Rare Complication of Ruptured Morgagni Diaphragmatic Hernia: Case Report and Review of Literature





It’s Never Lupus: A Rare Case of Methimazole Induced Pleural Effusion



Left Sided Catamenial Pneumothorax : A Rare Entity D36: PLEURAL DISEASE: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center


Talcoma: The Confounding of a Differential Diagnosis in a Patient with a Pleural-Based Mass


37

Rare Disease Term




Another Case of Pleural Based Malignancy or Perhaps Not: An Unusual Presentation of Gunshot Injury



Extramedullary Hematopoesis of the Pleura: A Rare Cause of Massive Hemothorax



Pancreaticopleural Fistulae; a Rare Pulmonary Complication of an Intra-Abdominal Disease



Chylothorax Due to Esophageal Duplication Cyst: Case Report D36: PLEURAL DISEASE: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center


Pyocholethorax: A Rare Complication of Percutaneous Transhepatic Biliary Drainage



A Case of Cryptogenic Bilateral Fibrosing Pleuritis, an Extremely Rare Form of Interstitial Lung Disease



Transudative Chylothorax: A Portal Hypertension Problem? D36: PLEURAL DISEASE: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center


Spontaneous Pneumomediastinum (Hamman’s Syndrome): A Case Report


RARE TUMORS — AIRWAY



Long Term Efficacy of Radio Frequency Ablation for Superficial Lesions of the Tracheobronchial Tree


P296 9:15 am-4:15 pm; 11:15 am-1:00 pm

Incidental Finding of Endobronchial Schwannoma in Lung Transplant Candidate Undergoing Lung Nodule Evaluation



Abscopal Effect Following CT Guided Permanent Seed Brachytherapy for Metastatic Adenoid Cystic Carcinoma to Lung



An Atypical Bronchopulmonary Carcinoid Tumor Manifesting as Abdominal Cramping



A Rare Cause of Hemoptysis: Inflammatory Myofibroblastic Tumor of the Trachea



Bilateral Primary Endobronchial Leiomyosarcoma B71: CASE REPORTS IN THORACIC ONCOLOGY III Area D (Hall A-B2, Ground Level)San Diego Convention Center

38

Rare Disease Term




Rare Presentation of Sclerosing Pneumocytoma Manifesting as an Endobronchial Lesion



Dual Primary Lung Cancers. Mucoepidermoid Carcinoma and Adenocarcinoma, a Rare Entity



Tracheobronchial Inflammatory Myofibroblastic Tumor in a Young Adult with Subsequent Sarcoidosis



Unusual Presentation of Pulmonary MALT-Lymphoma as an Occlusive Mainstem Endobronchial Mass

B75: CASE REPORTS IN THORACIC ONCOLOGY VII Area D (Hall A-B2, Ground Level)San Diego Convention Center


Obstructing Hyalinizing Clear Cell Primary Tracheal Carcinoma Presenting with a Non-Fixed Expiratory Spirogram




Pediatric Endobronchial Tumor Leading to Recurrent Pneumonia C64: PEDIATRIC CASE REPORTS: AIRWAY DISEASES AND BRONCHOSCOPY



Interesting Presentation of a Rare Endobronchial Lesion C64: PEDIATRIC CASE REPORTS: AIRWAY DISEASES AND BRONCHOSCOPY



When Wheezing Isn’t Asthma: A Case of Mucoepidermoid Carcinoma of the Trachea




A Lump in His Throat: Refractory Recurrent Respiratory Papillomatosis with Pulmonary Parenchymal Involvement Improved with Systemic Bevacizumab

C68: PEDIATRIC CASE REPORTS: IMMUNODEFICIENCY AND INFECTION




Use of Endotracheal Stent for Hemostasis of a Bleeding Glomus Tumor



Endobronchial Liposuction: Excision of Endobronchial Lipoma in Patient with Pulmonary Hamartoma



Extranodal NK/T-Cell Lymphoma of the Trachea Causing Airway Compromise



Carcinoma Ex Pleomorphic Adenoma of the Airway and Parenchyma D31: INTERVENTIONAL PULMONARY: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

RARE TUMORS — PARENCHYMAL



Benign Pulmonary Nodular Lymphoid Hyperplasia (NLH) in an HIV Infected Patient: A Diagnostic Dilemma


39

Rare Disease Term




Pulmonary Nodular Lymphoid Hyperplasia Associated with Multiple Sclerosis



Pulmonary Inflammatory Myofibroblastic Tumor (IMT) with Remarkable Response to Corticosteroids



Using EBUS to Sample Pulmonary Artery Tumors B37: INTERVENTIONAL PULMONARY: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


Extranodal Natural Killer/T Cell Lymphoma, Nasal Type: A Rare Pulmonary Presentation



A Case of Suspected Metastatic Choriocarcinoma Versus Ectopic Beta-HCG Producing Adenocarcinoma Causing Acute Hypoxic Respiratory Distress and Thyroid Storm

B46: CRITICAL CARE CASE REPORTS: HEMATOLOGY, ONCOLOGY, RHEUMATOLOGY, AND IMMUNOLOGY



A Giant Pain in the Shoulder B69: CASE REPORTS IN THORACIC ONCOLOGY I Area D (Hall A-B2, Ground Level)San Diego Convention Center


A Unique Presentation of a Rare Primary Epithelial-Myoepithelial Carcinoma of Lung



Primary Lung Diffuse Large B-Cell Lymphoma with Hepatic Metastases: A Rare Neoplasm



Hemoptysis as a Presentation of Composite Hemangioendothelioma B69: CASE REPORTS IN THORACIC ONCOLOGY I Area D (Hall A-B2, Ground Level)San Diego Convention Center


Pulmonary Artery Sarcoma Presenting as a Pulmonary Embolism B70: CASE REPORTS IN THORACIC ONCOLOGY II Area D (Hall A-B2, Ground Level)San Diego Convention Center


Unusual Presentation of an Uncommon Lung Tumor: A Case Report B70: CASE REPORTS IN THORACIC ONCOLOGY II Area D (Hall A-B2, Ground Level)San Diego Convention Center


“Hepatoid Adenocarcinoma of the Lung with Normal Alpha-Fetoprotein Level”



Incidental Diagnosis of Primary Pulmonary Synovial Sarcoma (PPSS) in an Elderly Patient with Pulmonary Fibrosis Complicated by Radiation Pneumonitis



Squamous Cell Carcinoma in a Young Patient with a Chronic Infectious Disease


40

Rare Disease Term




Pulmonary Large Cell Neuro-Endocrine Cancer: A Diagnostic Difficulty and Therapeutic Dilemma



Biphasic Pulmonary Blastoma Invading the Mediastinum and Pleura with Brain Metastasis in an Adult Female


P297 9:15 am-4:15 pm;11:15 am-1:00 pm

Chronic Hemoptysis: Manifestation of a Rare, Obscure, and Fatal Diagnosis

B70: CASE REPORTS IN THORACIC ONCOLOGY II Area D (Hall A-B2, Ground Level) San Diego Convention Center


The New Miliary Kid on the Block B71: CASE REPORTS IN THORACIC ONCOLOGY III Area D (Hall A-B2, Ground Level)San Diego Convention Center


Lung Inflammatory Pseudotumor in an Adult Smoker B71: CASE REPORTS IN THORACIC ONCOLOGY III Area D (Hall A-B2, Ground Level)San Diego Convention Center


An Undifferentiated Malignancy B71: CASE REPORTS IN THORACIC ONCOLOGY III Area D (Hall A-B2, Ground Level)San Diego Convention Center


Myelolipoma: A Rare Tumor in a Rare Location B72: CASE REPORTS IN THORACIC ONCOLOGY IV Area D (Hall A-B2, Ground Level)San Diego Convention Center


Brotherly Love: A Case of Pulmonary Plasma Cell Granuloma in Twin Brothers



A Nut-ty Case of Lung Cancer B72: CASE REPORTS IN THORACIC ONCOLOGY IV Area D (Hall A-B2, Ground Level)San Diego Convention Center


A Rare Malignancy of the Lung: A Case of Solitary Plasmacytoma B72: CASE REPORTS IN THORACIC ONCOLOGY IV Area D (Hall A-B2, Ground Level)San Diego Convention Center


Near Complete Right Lung Obliteration Due to Rare Pulmonary NUT Midline Carcinoma



Lung Cancer in Very Young Males B73: CASE REPORTS IN THORACIC ONCOLOGY V Area D (Hall A-B2, Ground Level)San Diego Convention Center


Pulmonary Artery Sarcoma Diagnosed by Endovascular Catheter-Guided Biopsy: A Case Report



An Unusual Tumor with Unusual Metastasis B73: CASE REPORTS IN THORACIC ONCOLOGY V Area D (Hall A-B2, Ground Level)San Diego Convention Center

41

Rare Disease Term




Sleeping Beauty: A Case of a 15-Year-Old Pulmonary Epithelioid Hemangioendothelioma



Pneumocytoma: A Benign Cause of a Malignant Diagnosis B73: CASE REPORTS IN THORACIC ONCOLOGY V Area D (Hall A-B2, Ground Level)San Diego Convention Center


Primary Pulmonary Angiosarcoma: Rare and Deadly B73: CASE REPORTS IN THORACIC ONCOLOGY V Area D (Hall A-B2, Ground Level)San Diego Convention Center


Rapid Progressive Pulmonary Malignant Perivascular Epithelioid Cell Tumor: A Case Report and Literature Review



A Rare Entity of Primary Pulmonary Hodgkin Lymphoma with Cavitation



A Rare Case of Metastatic Choriocarcinoma Presenting One Week After a Term Pregnancy



Pulmonary Sarcomatoid Carcinoma: An Often Missed Diagnosis with Poor Prognosis



Expanding Lung Mass in a Patient with Chronic Myelomonocytic Leukemia




Metastatic Angiosarcoma Presenting as Diffuse Alveolar Hemorrhage

C54: CRITICAL CARE CASE REPORTS: CAUSES AND COMPLICATIONS OF ACUTE RESPIRATORY FAILURE



Presentation of Pulmonary Kaposi Sarcoma in the Absence of Cutaneous Manifestations: A Diagnostic Challenge for Physicians

C59: UNUSUAL PULMONARY INFECTIONS Area K (Hall A-B2, Ground Level)San Diego Convention Center


Recurrent Pneumonia—When to Think Outside the Box C64: PEDIATRIC CASE REPORTS: AIRWAY DISEASES AND BRONCHOSCOPY



Pulmonary Alveolar Proteinosisand Pulmonary Kaposi Sarcoma in a Young Adult with HIV

C68: PEDIATRIC CASE REPORTS: IMMUNODEFICIENCY AND INFECTION




Lymphocytic Interstitial Pneumonitis with Lung-Localised MALT LymphomaPresenting as Infiltrates on Chest X-Ray in an Asymptomatic Patient



Endemic Kaposi Disease (KD) with Pulmonary Localization: A Case Report



42

Rare Disease Term


RARE TUMORS — PLEURAL



Pleural Lipoma with Secondary Changes — A Diagnostic Challenge B39: PLEURAL DISEASE: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


Primary Synovial Sarcoma of the Pleura: A Rare Cause of a Lung Mass



An Autopsied Case of Neurofibromatosis Type 1(von Recklinghau-sen's Disease) Associated Malignant Peripheral Nerve Sheath Tu-mor Carcinomatous Pleurisy with Giant Intrathoracic Meningoceles




Recurrence of Resected Solitary Fibrous Tumor D36: PLEURAL DISEASE: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center


A Case of Pleuropulmonary Nodular Lymphoid Hyperplasia Presenting with Recurrent Exudative Pleural Effusions



Deadly Imposter: A Rare Pleural Tumor Masquerading as Pulmonary Abscess



Malignant Pleural Effusion as the First Presentation of Thyroid Cancer



Doege Potter Syndrome: Solitary Fibrous Tumor of the Pleura Associated with Hypoglycemia


RELAPSING POLYCHONDRITIS



Long-Term Outcome of Metallic Stenting for Airway Involvement in Relapsing Polychondritis




Unrelenting Tracheobronchial Stenosis in Relapsing Polychondritis D31: INTERVENTIONAL PULMONARY: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center

SCLERODERMA LUNG DISEASE



Safety and Suitability of a Direct Thrombin Inhibitor, Dabigatran Etexilate, in Scleroderma-Associated Interstitial Lung Disease (SSc-ILD) Patients



43

Rare Disease Term



609 9:15 am-11:15 am;Poster Viewing 9:15 am -10:00 amPoster Discussion 10:00 am-11:15 am

Hemodynamics and Prognosis in Scleroderma-Associated PAH A27: YOU GOT ANOTHER THING COMING: DIAGNOSIS AND PROGNOSTICATION IN PULMONARY HYPERTENSION



M10, a 10 Amino Acid Peptide, Regulates the Extracellular Matrix Expression via a Dual Mechanism in Scleroderma Associated Lung Fibrosis

A68: MOLECULAR DETERMINANTS OF REMODELING IN LUNG FIBROSIS




Re-evaluation of The Parenchymal CT Features of Scleroderma Lung Disease Using Volumetric HRCT



Small Airway Disease a Potential Feature of Scleroderma LungDisease



Patients With Scleroderma Are At Increased Risk For Sleep Disorder Breathing (sdb)

B67: RISK AND PREVALENCE OF SLEEP DISORDERED BREATHING




Molecular Evidences for Antifibrotic Potentials of Nintedanib in Interstitial Lung Disease Associated with Systemic Sclerosis





Predicting Mortality in Systemic Sclerosis-Related Interstitial Lung Disease in Two Independent Cohorts

D14: ILD: CLINICAL RESEARCH Room 3 (Upper Level)San Diego Convention Center

SHRINKING LUNG SYNDROME



The Other "Baby Lung": Shrinking Lung Syndrome in the Absence of Confirmed Connective Tissue Disease



Rare Case of Systemic Lupus Erythematosus Shrinking Lung Syndrome in a Young Male Patient



Shrinking into Thin Air? A Case Report of Vanishing Lung Syndrome B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center

SICKLE CELL DISEASE



Airway Inflammatory Patterns in Sickle Cell Disease A62: SICKLE CELL DISEASE Area F (Hall A-B2, Ground Level)San Diego Convention Center



Platelets shed extracellular vesicles to promote IL-1α Dependent Lung Vaso-Occlusion in Sickle Cell Disease

B15: IMMUNE PATHWAYS IN LUNG INJURY AND REPAIR Pacific Ballroom 22 (North Tower, First Floor)Marriott Marquis San Diego Marina

44

Rare Disease Term


SILICOSIS



Comparison of Decellularization between Normal and Silicotic Lungs on Lung Function and Extracellular Matrix Components

B29: LUNG ECM ENVIRONMENT: NOVEL FINDINGS AND MODELS IN FIBROSIS



Complicated Silicosis Mimicking Primary Lung Malignancy on Imaging

B49: OCCUPATIONAL EXPOSURES Area B (Hall A-B2, Ground Level)San Diego Convention Center

SJOGREN’S SYNDROME



An Unusual Cause of Pleural Effusion D36: PLEURAL DISEASE: CASE REPORTS II Area G (Hall A-B2, Ground Level)San Diego Convention Center


Primary Sjögren’s Syndrome-Associated Interstitial Lung Disease—A Descriptive Study


SWEET SYNDROME



Sweet Syndrome with Pulmonary Involvement: High Index of Suspicion Important for Differentiation from Pneumonia and Timely Treatment




Acute Respiratory Failure: A Rare Manifestation of Sweet Hydrothorax


THORACIC ENDOMETRIOSIS/CATAMENIAL PNEUMOTHORAX



A Rare Case of Thoracic Endometriosis Presenting with Hemopneumothorax



Catamenial Hemothorax a Rare Cause of Recurrent Pleural Effusion B39: PLEURAL DISEASE: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center


A Rare Case of Tension Hydropneumothorax from Disseminated Endometriosis


TRACHEOBRONCHOPATHIA OSTEOCHONDROPLASTICA



Tracheobronchopathia Osteochondroplastica: Rare Cause of Dyspnea, Cough, and Bronchitis

D38: COUGH: CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center

45

Rare Disease Term


VASCULITIS — ANCA-ASSOCIATED



Clinical Utility of Serial Measurements of Antineutrophil Cytoplasmic Antibodies Targeting Proteinase 3 by Automated Bead-Based Assay




Prolidase Deficiency and ANCA-Associated Vasculitis in a 16-Month Old Boy



VASCULITIS — EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA)



Hypoxia and Eosinophilia in a Patient with 'Difficult Asthma' A31: ALLERGY AND ASTHMA CASE REPORTS Area H (Hall A-B2, Ground Level)San Diego Convention Center


Glucocorticoid Sparing Effects of Reslizumab in the Treatment of Eosinophilic Granulomatosis with Polyangiitis

A34: ASTHMA CLINICAL STUDIES I Area H (Hall A-B2, Ground Level)San Diego Convention Center


Low Dose Mepolizumab in Eosinophilic Granulomatosis with Polyangiitis



A Novel Tool to Measure Clinical Activity and Outcomes in Eosinophilic Granulomatosis with Polyangiitis




Eosinophilic Granulomatosis with Polyangiitis Patient Characteristics and Clinical Response to Mepolizumab Compared to Placebo



Immunosuppressive Therapy Ameliorates Severe Pulmonary Hypertension and Refractory Vasospastic Angina Due to Eosinophilic Granulomatosis with Polyangiitis

B47: CRITICAL CARE CASE REPORTS: CARDIOVASCULAR DISEASES AND ECHOCARDIOGRAPHY



Eosinophilic Granulomatosis with Polyangiitis Presenting with Cardiac Tamponade

B47: CRITICAL CARE CASE REPORTS: CARDIOVASCULAR DISEASES AND ECHOCARDIOGRAPHY




Clinical and Economic Characteristics of Patients Diagnosed with Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome) in the United States




ANCA-Negative Eosinophilic Granulomatosis with Polyangitis Presenting as Hemoptysis in a Pediatric Patient



VASCULITIS — GRANULOMATOSIS WITH POLYANGIITIS (GPA)



Diffuse Cobblestone Appearance of the Tracheobronchial Tree: A Rare Presentation of Granulomatosis with Polyangiitis


46

Rare Disease Term




A Rare Case of Polyangiitis Overlap Syndrome: Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis



The Benefit of Plasmapheresis on Alveolar Hemorrhage in Pulmonary-Renal Syndrome: A Case of Granulomatosis with Polyangiitis (GPA)



Auto-Pneumonectomy with Compensatory Lobar Hyper-Expansion and Extreme Mediastinal Shift in Granulomatosis with Polyangiitis



A Case of Granulomatosis with Polyangiitis Masquerading as Fungal Pneumonia



Flu Vaccine Associated ANCA Vasculitis: A Rare Entity B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center



Massive Hemoptysis and Diffuse Alveolar Hemorrhage from Granulomatosis with Polyangiitis Successfully Rescued by Extracorporeal Membrane Oxygenation

C52: CRITICAL CARE CASE REPORTS: GOOD VIBRATIONS — MECHANICAL VENTILATION FROM NIV TO ECMO



All that Wheezes Is Not Asthma: An Atypical Presentation of Granulomatosis with Polyangiitis in an 8-Year-Old Female



VASCULITIS — MICROSCOPIC POLYANGIITIS



Bloody Lungs and Broken Kidneys Banded in One Blight B32: CLINICAL STUDIES IN AUTOIMMUNE LUNG DISEASE Area G (Hall A-B2, Ground Level)San Diego Convention Center


Microscopic Polyangiitis Complicated Due to Mucormycosis B46: CRITICAL CARE CASE REPORTS: HEMATOLOGY, ONCOLOGY, RHEUMATOLOGY, AND IMMUNOLOGY




Presentation of MPO-ANCA Positive Microscopic Polyangiitis with Usual Interstitial Pneumonia


VASCULITIS — OTHER



Beware of the Rare: Fatal Hemoptysis in Hughes-Stovin Syndrome B34: AUTOIMMUNE LUNG DISEASE CASE REPORTS Area G (Hall A-B2, Ground Level)San Diego Convention Center


Hughes-Stovin Syndrome and Chronic Thromboembolic Pulmonary Hypertension: A Case Report



47

Rare Disease Term




A Cough that Is More than Asthma: A Rare Case of Bronchiectasis C64: PEDIATRIC CASE REPORTS: AIRWAY DISEASES AND BRONCHOSCOPY


P1019 9:15 am-4:15 pm;11:15 am-1:00 pm

Regrets Over a Missed Opportunity to Have a Biological Child — One Couple’s Journey After Lung Transplantation for Cystic Fibrosis





Williams-Campbell Syndrome: An Elusive Cause of Bronchiectasis D37: CF AND NON-CF BRONCHIECTASIS AND IMMUNE DEFICIENCIES: CASE REPORTS


XIPHOIDYNIA



Excision of the Xiphoid Process for Chronic Pain: Patient Characteristics and Results



YELLOW NAIL SYNDROME



Rare Presentation of a Rare Disease B39: PLEURAL DISEASE: CASE REPORTS I Area G (Hall A-B2, Ground Level)San Diego Convention Center

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YOU’RE INVITED TO 2 BOEHRINGER INGELHEIM GURU BAR SESSIONS

May 20, 2018, 12:30 PMGURU BAR 1, EXHIBIT HALL

INTRODUCING RAD ROUNDS—UIP TO IPF: A Digital Application to Identify Usual Interstitial

Pneumonia on HRCT

Join us to learn about the recently launched Rad Rounds —UIP to IPF mobile app. The app’s radiology content and usual interstitial pneumonia (UIP) diagnostic

algorithm will be detailed by an expert pulmonologist and radiologist.

TO INSTALL THIS APP ON YOUR iPHONE OR ANDROID PHONE:Text RADIPF to 313131 to receive a download link for your iPhone

Text RADUIP to 313131 to receive a download link for your Android phone

CONCOMITANT IDIOPATHIC PULMONARY FIBROSIS AND EMPHYSEMA:

Strategies to Improve Patient Management

May 21, 2018, 12:30 PMGURU BAR 3, EXHIBIT HALL

Come learn about the presence of concomitant emphysema in some patients with IPF and discuss management strategies for patients with CPFE with an

expert pulmonologist.

Copyright ©2018, Boehringer Ingelheim Pharmaceuticals, Inc. All rights reserved. (02/18) PC-US-102966CPFE, combined pulmonary fi brosis and emphysema; HRCT, high-resolution computed tomography; IPF, idiopathic pulmonary fi brosis.

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JOIN US FOR AN INDUSTRY-ORGANIZED SYMPOSIUM

Sponsored by Boehringer Ingelheim Pharmaceuticals, Inc.

Copyright ©2018, Boehringer Ingelheim Pharmaceuticals, Inc. All rights reserved. (02/18) PC-US-102965

Sunday, May 20, 20186:30 PM Dinner/Symposium

Hilton San Diego BayfrontIndigo Ballroom D/H

San Diego, CA

ADMISSION IS COMPLIMENTARY AND WILL BE ON A FIRST-COME, FIRST-SERVED BASIS. DINNER WILL BE PROVIDED.

An Industry-Organized Symposium at the ATS 2018 International Conference. All ATS 2018 International Conference attendees are invited to this non-CME/CNE educational

program sponsored by Boehringer Ingelheim Pharmaceuticals, Inc.

Attendance is limited to healthcare professionals only. Aspects of this program may be reportable under the physician payments Sunshine Act.

CME, continuing medical education; CNE, continuing nursing education.

PREPARED BY FCB

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American Thoracic Society International Conference · P1393 9:15 am-4:15 pm; Poster Viewing 11:15...

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