Pulmonology Case Presentation Dr. GJ Visagie

Patient 43 yo Female Known 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 investigations CXR

Flow volume curve

Flow Volume (Cont)

Blood investigations Arterial blood gas Full Blood count 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-ILD Clinical 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-ILD Lung Functions Normal or mixed obstructive restrictive pattern DLCO2 impaires Total lung volumes may be Normal Increased Decreased

RB-ILD Radiology CXR 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) 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

Nodular sclerosing lesions containing Langerhans’ cells PLCH Histology Nodular sclerosing lesions containing Langerhans’ cells Bronchiolocentric distribution Progression Fibrosis and cystic changes

PLCH Clinical 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%

PLCH Lung functions Obstructive and restrictive changes may be seen Difficult to distinguish effects of cigarette smoking Abnormal diffusion capacity Abnormal exercise tolerance

Radiology of PLCH CXR Nodular or reticular nodular Middle and upper lung zones Cystic changes increased lung volumes

Radiology of PLCH HRCT Upper lung involvement Sparing of lung bases Complex cysts Nodules and cysts

PLCH Diagnosis 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 Ryu JH, Myers JL, Capizzi SA et al, Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease, Chest 2005;127;178-184 Ryu JH, Colby TV, Hartman TE, Vassallo R, Smoking related interstitial lung diseases: A concise review, Eur Resp J 2001; 17:122-132 Hidalgo A, Franquet T, Gimenez A et al, Smoking-related interstitial lung diseases: radiologic-pathologic correlation, Eur Radiol 2006; 16: 2463–2470 Atilli AK, Kazarooni EA, Gross BH et al, Smoking-related Interstitial Lung Disease: Radiologic- Clinical-Pathologic Correlation, RadioGraphics 2008; 28: 1383–1398 Caminati A, Harari S, Smoking-related Interstitial Pneumonias and Pulmonary Langerhans Cell Histiocytosis, Proc Am Thorac 2006; 3: 299–306 Selman M, The Spectrum of Smoking-Related Interstitial Lung Disorders: The Never-Ending Story of Smoke and Disease, Chest 2003; 124: 1185-1187