1. Pulmonology Case Presentation
Dr. GJ Visagie

2. 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

3. 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!!!

4. Initial Thoughts Pulmonary embolism COPD with respiratory failure
Obesity hypoventilation syndrome
Infective lung process

5. 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

6. Further investigations CXR

7. Flow volume curve

8. Flow Volume (Cont)

9. 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

10. HRCT

11. Additional Blood investigations
ANCA Negative
ANF Negative
RF Negative
S-Ace Negative
HIV Negative

12. Summary of patient Patient with Interstitial lung disease
Type 1 respiratory failure
Long smoking history
Obstructive sleep apnea
Obesity stage 3

13. Smoking related interstitial lung disease

14. Introduction

15. Introduction

16. Diseases associated with smoking
Desquamative interstitial Pneumonia
Respiratory bronchiolitis-associated interstitial lung disease
Pulmonary Langerhans cell histiocytosis

17. 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%

18. DIP histology
Increased number of pigmented macrophages in alveolar spaces
Thickened alveolar septae
Overall architecture maintained with very little honeycombing

19. DIP Lung functions Restrictive pattern Decreased diffusion capacity
Less pronounced than with idiopathic pulmonary fibrosis

20. 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

21. 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

22. 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!

23. 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

24. 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

25. RB-ILD Lung Functions Normal or mixed obstructive restrictive pattern
DLCO2 impaires
Total lung volumes may be
Normal
Increased
Decreased

26. RB-ILD Radiology
CXR
Reticular or reticulo nodular opacities in +/- 60%
Ground glass
HRCT
Areas of ground glass attenuation
Fine nodules
Associated emphysematous changes

27. 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

28. 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

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

30. 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%

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

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

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

34. PLCH Diagnosis History and CT findings may be diagnostic
Surgical biopsy
Broncho alveolar lavage
> 5% of CD1a positive cells

35. Treatment and Prognosis
Natural history is uncertain
Treatment
? Smoking cessation
? Corticosteroids
Other Cytotoxic drugs

36. Ryu JH, Colby TV, Hartman TE, Vassallo R, Smoking related interstitial lung diseases: A concise review, Eur Resp J 2001; 17:

37. Lung diseases related to smoking
Lung Cancer
Chronic Obstructive airways disease
Interstitial lung diseases

38. Conclusion

39. References
Ryu JH, Myers JL, Capizzi SA et al, Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease, Chest 2005;127;
Ryu JH, Colby TV, Hartman TE, Vassallo R, Smoking related interstitial lung diseases: A concise review, Eur Resp J 2001; 17:
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: