Dr. GJ Visagie. 43 yo Female Known with Obesity Class 3 Recently lost 45 Kg Obstructive sleep...
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Transcript of Dr. GJ Visagie. 43 yo Female Known with Obesity Class 3 Recently lost 45 Kg Obstructive sleep...
PULMONOLOGY CASE PRESENTATION
Dr. GJ Visagie
Patient
43 yo FemaleKnown with
Obesity Class 3 Recently lost 45 Kg Obstructive sleep apnea Pulmonary hypertension and Cor Pulmonale Polycystic ovarian Syndrome Significant smoking history Benign intracranial hypertension
Patient (Continued)
Admitted to neurology with headache While in ward she experienced a episode
of chest pain. Caused by pulmonary hypertension In unit it was noticed that the patient had
a low oxygen saturation Pulmonology was consulted!!!
Initial Thoughts
Pulmonary embolism COPD with respiratory failure Obesity hypoventilation syndrome Infective lung process
Physical examination
Overweight middle aged female Hypertrichosis of face Central cyanosis Plethoric Comfortable with no distress CVS: Loud P2 and heaving Right ventricle Resp: Fine late inspiratory crackles Abd: Central obesity
Further investigationsCXR
Flow volume curve
Flow Volume (Cont)
Blood investigations
Arterial blood gas pH 7,37 pO2 52,6 pCO2 37,6 SaO2 87%
Full Blood count HB 17,8 WCC 6,08 Plt 190
Thyroid function
Lipid profile
Raised GGT
HRCT
Additional Blood investigations
ANCA Negative ANF Negative RF Negative S-Ace Negative HIV Negative
Summary of patient
Patient with Interstitial lung disease Type 1 respiratory failure Long smoking history Obstructive sleep apnea Obesity stage 3
Smoking related interstitial lung disease
Introduction
Introduction
Diseases associated with smoking
Desquamative interstitial Pneumonia Respiratory bronchiolitis-associated
interstitial lung disease Pulmonary Langerhans cell histiocytosis
Desquamative interstitial pneumonia (DIP)
90% of patients with this diagnosis have smoked
Male predominance Clinical and radiologically non specific
findings Dyspnea and cough Inspiratory crackles 50-60% Clubbing 25%-50%
DIP histology
Increased number of pigmented macrophages in alveolar spaces
Thickened alveolar septae Overall architecture maintained with very
little honeycombing
DIP Lung functions
Restrictive pattern Decreased diffusion capacity Less pronounced than with idiopathic
pulmonary fibrosis
Radiological appearance of DIP
• Ground Glass appearance
• Correlate with macrophage accumulation in alveoli and alveolar ducts
• Chest X-ray may be normal in up to 22% of biopsy proven cases
Treatment and Prognosis
DIP There has been a +/- 30% mortality rate
reported in these patients ? Smoking cessation Some improvement on Steroid therapy but
data is unclear Measurement of improvement ? Sustained improvement Correlate to smoking cessation
Role of other immuno-therapy
Respiratory bonchiolitis
Respiratory bronchiolitis, First described as an incidental post mortem finding in smokers by Niewoehner
Extremely common in Smokers Usually without symptoms of
accompanying lung disease May account for 20% of subclinical
radiographic changes in smokers Small portion of these patients may have
accompanying interstitial lung disease!
Respiratory bonchiolitis-associated interstitial lung disease (RB-ILD)
Characterised by Pigmented macrophages Mild interstitial changes in interstitium
surrounding respiratory bronchioles and alveoli Virtually all cases are smoking related Histologically less extensive and diffuse
compared to DIP
RB-ILDClinical picture
Symptoms usually start in the 4th-5th decade
Slightly more males than females involved
Cough and dyspnea
50% have crackles and very few have clubbing
RB-ILDLung Functions
Normal or mixed obstructive restrictive pattern
DLCO2 impaires Total lung volumes may be
Normal Increased Decreased
RB-ILDRadiologyCXR• Reticular or reticulo
nodular opacities in +/- 60%
• Ground glass
HRCT• Areas of ground
glass attenuation• Fine nodules• Associated
emphysematous changes
Treatment and Prognosis
Good Prognosis compared to DIP No deaths have been attributed to RB-ILD No Progression to Lung fibrosis
Treatment Stop smoking ? Role of corticosteroids
Pulmonary Langerhans’ cell histiocytosis (PLCH)
Langerhans Cell histiocytosis Non malignant disorder Abnormal proliferation of dendritic cells May affect multiple organs or involve only single
organ system Spectrum of severity (Children usually more
severely affected: Letterer Siwe disease, Hand schuller christian syndrome)
Lung involvement Usually in isolation Associated with Smoking
PLCHHistology
Nodular sclerosing lesions containing Langerhans’ cells
Bronchiolocentric distribution
Progression Fibrosis and cystic changes
PLCHClinical features
Adults in 3rd and 4th decade Sex distribution is uniform > 90% of patients are smokers or ex-
smokers Dyspnea (35-87%) Cough (50-70%) Previous Pneumothorax (10%) Crackles and clubbing very rare Wheezes may be present Cystic bone lesions (Skull, pelvis,ribs)
10%
PLCHLung functions
Obstructive and restrictive changes may be seen
Difficult to distinguish effects of cigarette smoking
Abnormal diffusion capacity Abnormal exercise tolerance
Radiology of PLCHCXR• Nodular or
reticular nodular
• Middle and upper lung zones
• Cystic changes increased lung volumes
Radiology of PLCHHRCT• Upper lung
involvement• Sparing of lung
bases• Complex cysts• Nodules and
cysts
PLCHDiagnosis
History and CT findings may be diagnostic
Surgical biopsy Broncho alveolar lavage
> 5% of CD1a positive cells
Treatment and Prognosis
Natural history is uncertain Treatment
? Smoking cessation ? Corticosteroids Other Cytotoxic drugs
Ryu JH, Colby TV, Hartman TE, Vassallo R, Smoking related interstitial lung diseases: A concise review, Eur Resp J 2001; 17:122-132
Lung diseases related to smoking
Lung Cancer Chronic Obstructive airways disease Interstitial lung diseases
Conclusion
References
1. Ryu JH, Myers JL, Capizzi SA et al, Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease, Chest 2005;127;178-184
2. Ryu JH, Colby TV, Hartman TE, Vassallo R, Smoking related interstitial lung diseases: A concise review, Eur Resp J 2001; 17:122-132
3. Hidalgo A, Franquet T, Gimenez A et al, Smoking-related interstitial lung diseases: radiologic-pathologic correlation, Eur Radiol 2006; 16: 2463–2470
4. Atilli AK, Kazarooni EA, Gross BH et al, Smoking-related Interstitial Lung Disease: Radiologic- Clinical-Pathologic Correlation, RadioGraphics 2008; 28: 1383–1398
5. Caminati A, Harari S, Smoking-related Interstitial Pneumonias and Pulmonary Langerhans Cell Histiocytosis, Proc Am Thorac 2006; 3: 299–306
6. Selman M, The Spectrum of Smoking-Related Interstitial Lung Disorders: The Never-Ending Story of Smoke and Disease, Chest 2003; 124: 1185-1187