Just Say “NO!” To Ammonia Kelley E. Capocelli, MD Clinical Pathology Conference April 29, 2005.
Clinical Pathology Conference
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Transcript of Clinical Pathology Conference
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Clinical Pathology Conference
42 yo man with recurrentpneumonia
J. R. Hartig, MD
November 28, 2006
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CPC: Goals
Its all about the discussion(If Im wrong!)
Its all about being correct(If the discussion is bad)
Its all about having the diagnosis in thedifferential
(My DDx will include all of Harrisons) Its all about getting even with the CMRs
(If this is something totally ridiculous)
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CPC: Goals
Discuss the approach that I used in thiscase
Discuss some uncommon pulmonaryconditions
Make an educated guess after narrowing
the differential
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Start from the start
42 yo white male with blood-tingedproductive cough
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Differential Diagnosis
Common things are common
Bronchitis
Viral URI
Rhinosinusitis (Allergies)
Pneumonia
GERD
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Start from the start
42 yo white male with blood-tingedproductive cough
Recurrent pneumonias
Several CAP during the past year
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Missouri State Motto
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Recurrent pneumonias
Pulmonary
Local
Diffuse
Immune
Gastrointestinal Problems
Neurological Conditions
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Recurrent pneumonias
Pulmonary
Local
Anatomic abnormality Bronchial compression
LAD
Neoplasm
Vascular anomaly
Foreign body Bronchiectasis
Bronchomalacia / stenosis
Tracheo-esopahgeal or bronchial fistulas
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Recurrent pneumonias
Pulmonary
Diffuse recurrence
Underlying pulmonary process Cystic fibrosis
Immotile cilia syndromes
Neurological dysfunctions
Dysphagia Seizures
Etoh/drugs
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Cystic Fibrosis
Autosomal recessive disease
1:2000 to 3000
CFTR protein abnormality
Multi-organ disease
mild mutations
Productive cough
No hint of disease earlier in life
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Recurrent pneumonias
Pulmonary
Diffuse recurrence
Underlying pulmonary process Cystic fibrosis
Immotile cilia syndromes
Neurological dysfunctions
Dysphagia Seizures
Etoh/drugs
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Recurrent pneumonias
Immune
Quantitative or Qualitative granulocytic
Chronic Granulomatous Disease
Multiple Myeloma
Common Variable Immune Deficiency
Chronic lymphocytic leukemia HIV
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Recurrent pneumonias
Gastrointestinal Problems
Aspiration (primary or secondary)
GERD
Zenkers diverticula
Achalasia
Neurologic Conditions
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More History
Chronic non-productive cough
Progressive dyspnea over the coarse of a
year
No fevers, chills, night sweats
Weight loss >20lbs
Antibiotics give short term relief
Steroids do not seem to help
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Some of the teasers
Works as a hairstylist
+MSM
27 pk/yr tobacco use
No drugs, No Etoh, No TB risks (?homeless)
ROS: no other signs of bleeding problems
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Exam
Marked hypoxia on room air (80%)
Chronically hypoxic (clubbing)
Pulmonary exam
Mild tachypnea
Bibasilar rales
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Clubbing
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Labs
Marked A-a gradient
Elevated bicarb
Hct 49
LDH 603
WBC 19K But not really lymphopenic
Numerous other negative labs
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Additional Testing
Normal Transthoracic echocardiogram
CXR and CT
Patchy ground glass opacities diffusely with ahilar predominance. No lymphadenopathy.
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Lucy!!! You got some splaining to
doYoung W chronic non-productive cough
Recurrent Pneumonias
Severe Dyspnea
Weight Loss
Antibiotics some help. Prednisone none. Chronic hypoxia
Hilar predominant radiographic changes
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Where to now?
Infections
Vascular
Malignancy
Pulmonary
Interstitial Lung Diseases (DiffuseParenchymal Lung Diseases)
Diffuse Alveolar Hemorrhage Syndromes
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Infections (Pneumonia)
Previously healthy host
Diagnosed with pneumonia
Elevated WBC (left shift)
Gets better with antibiotics
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Infections (Pneumonia)
Viral
Bacterial Nocardia
Mycobacterial M. tuberculosis
Non-tuberculous Mycobacterial infections
Fungal
Histoplasmosis Coccidioidomycosis
Other/Parasitic Pneumocystis jiroveci
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Nocardia
Aerobic gram + bacteria
16 species causing human disease
Immunocompromised hosts Pulmonary manifestations
Non-improving pneumonia
TB mimic on x-ray
Diagnosis Treatment
Sulfonamides
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Tuberculosis
Common in Alabama
Always in the differential on a CPC
No risk factors
? Homeless
PPD (-)
No hilar adenopathy
Too many other things are possible
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Nontuberculous Mycobacteria
M. avium and M. intracellulare (MAC)
Older individuals with COPD
Bronchiectasis (cystic fibrosis)
Clinically similar to TB
M. kansasii
Similar to TB; cavitation common
Diagnosis and Treatment
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Histoplasmosis
First described in 1906 Common among Ohio and Mississippi River
valleys Most individuals have few symptoms Pulmonary disease
Bronchopneumonia Hilar LAD
Diagnosis Urine 75% pulmonary disease Serology 90% acute pulmonary disease
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Histoplasmosis
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Pneumocystis carinii (P. jiroveci)
pneumonia HIV (-) individuals
Malignancy
Hematologic > solid
Steroids
Mean dose 30mg/d and 12 weeks therapy
Often seen after therapy is stopped
Diagnosis and Therapy
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Differential Diagnosis
Congenital
Cystic fibrosis (better not be)
Infections PCP
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Where to now?
Infections
Vascular
Malignancy
Pulmonary
Interstitial Lung Diseases (Diffuse
Parenchymal Lung Diseases)
Diffuse Alveolar Hemorrhage Syndromes
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Vascular/Vasculitis
No obvious bleeding abnormality
No specific evidence to indicate PTE or
pulmonary veno-occlusive disease
Wegeners granulomatosis
Churg-Strauss syndrome
Microscopic polyangiitis
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Wegeners granulomatosis
Lungs and kidneys
25% of cases have only respiratory
involvement Primarily have sinus involvement
90% antineutrophil cytoplasmic antibodies
X-ray usually demonstrate nodules or LAD
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Wegeners granulomatosis
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Vasculitis
Churg-Strauss syndrome
Usually starts with asthma (8-10 years)
Skin and nasal disease is common No specific labs: eosinophilia, anca, others
Microscopic polyangiitis
Also has predominance of anca (+)
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Differential Diagnosis
Congenital
Cystic fibrosis (better not be)
Infections PCP
Vasculitis
?
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Malignancy
Lymphoma
Bronchogenic carcinoma
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Differential Diagnosis
Congenital
Cystic fibrosis (better not be)
Infections PCP
Vasculitis/Malignancy
?
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Where to now?
Infections
Vascular
Malignancy Pulmonary
Interstitial Lung Diseases (Diffuse
Parenchymal Lung Diseases) Diffuse Alveolar Hemorrhage Syndromes
Other
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Interstitial Lung DiseaseDiffuse Parenchymal Lung Disease
Idiopathic Pulmonary Fibrosis (UIP)
Other Idiopathic Interstitial Pneumonia
Desquamative IPAcute IP
Nonspecific IP
Respiratory bronchiolitis assoc -ILD
Cryptogenic Organizing Pneumonia
Known causes (Hypersensitivity, CTD)
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Idiopathic Pulmonary Fibrosis
Present in 5th or 6th decade of life
Progressive dyspnea and non-prod cough
: of 2:1 Elevated LDH
Follows relentless coarse difficult to treat
Radiographically Peripheral and bibasilar reticulonodular
opacities with traction bronchiectasis
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Idiopathic Pulmonary Fibrosis
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Desquamative IP
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Desquamative IP
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Respiratory bronchiolitis assoc -ILD
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Cryptogenic Organizing Pneumonia
Clinically presents in the 5th to 6th decade
Idiopathic form of BOOP
Persistent non-productive cough
Dyspnea
Weight loss (10lbs average)
Steroids help; antibiotics generally do not
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Cryptogenic Organizing Pneumonia
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Hypersensitivity Pneumonitis
Certainly could explain the radiographicfindings
Lack of specific agent (work exposure?) Chronic recurrent exposure
Generally improves with corticosteroids
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Differential Diagnosis
Congenital
Cystic fibrosis (better not be)
Infections
PCP
Vasculitis/Malignancy ?
DPLDs
Idiopathic Pulmonary Fibrosis
Desquamative Interstitial Pneumonia
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Diffuse Alveolar HemorrhageSyndromes
Anti-GBM disease (Goodpastures)
Idiopathic Pulmonary Hemosiderosis
Hct is normal
UA is normal
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Other
Sarcoidosis
Pulmonary Langerhans cell histiocytosis
Pulmonary Alveolar Proteinosis
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Sarcoidosis
Worldwide disease young adults
Multisystem granulomatous disorder
Characteristics: Bilateral hilar adenopathy
Pulmonary infiltrates
Skin/eye abnormalities Treatment
Generally involves steroids
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Pulmonary Langerhans cellHistiocytosis
Younger smoking patients (20-30s)
Caucasian
Fever, weight loss, other symptoms Spontaneous pneumothorax
Variable clinical coarse
PFTs Reticular nodular infiltrates and honeycombing
in upper lung zones
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Pulmonary Alveolar Proteinosis
Accumulation of lipoproteinaceous in thedistal airways
No inflammation or architectural changesAlveolar macrophage dysfunction
Impaired response to GM-CSF
Increased risk of superinfections
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Pulmonary Alveolar Proteinosis
Clinical Presentation
Typically age 30-50
Male to female 2:1 Insidious onset
Symptoms
Progressive dyspnea on exertion
Fatigue
Weight loss
Non-productive cough is common
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Pulmonary Alveolar Proteinosis
Radiographic findings
Bat-wing distribution
Air bronchograms are rareCrazy paving
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Pulmonary Alveolar Proteinosis
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Pulmonary Alveolar Proteinosis
Laboratory findings:
Polycythemia
Increased LDH Hypergammaglobulinemia
Marked hypoxia
serum anti-GM-CSF titer elevation
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Pulmonary Alveolar Proteinosis
Diagnosis
Bronchoalveolar lavage or biopsy
Treatment Whole lung lavage
Prognosis
??
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Differential Diagnosis
Congenital
Cystic fibrosis (better not be)
Infections
PCP
Vasculitis/Malignancy ?
DPLDs Idiopathic Pulmonary Fibrosis
Desquamative Interstitial Pneumonia
DAH Sarcoid
PAP
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Final Answer
Procedure
Bronchoscopy with BAL and TBBx
Diagnosis Pulmonary Alveolar Proteinosis