Presentation is loading. Please wait.

Presentation is loading. Please wait.

Dr. GJ Visagie.  43 yo Female Known with  Obesity Class 3 Recently lost 45 Kg  Obstructive sleep apnea  Pulmonary hypertension and Cor Pulmonale 

Similar presentations


Presentation on theme: "Dr. GJ Visagie.  43 yo Female Known with  Obesity Class 3 Recently lost 45 Kg  Obstructive sleep apnea  Pulmonary hypertension and Cor Pulmonale "— Presentation transcript:

1 Dr. GJ Visagie

2  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  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  Pulmonary embolism  COPD with respiratory failure  Obesity hypoventilation syndrome  Infective lung process

5  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

7

8

9  Arterial blood gas  pH7,37  pO252,6  pCO237,6  SaO287%  Full Blood count  HB17,8  WCC6,08  Plt190  Thyroid function  Lipid profile  Raised GGT

10

11  ANCANegative  ANFNegative  RFNegative  S-AceNegative  HIV Negative

12  Patient with  Interstitial lung disease  Type 1 respiratory failure  Long smoking history  Obstructive sleep apnea  Obesity stage 3

13

14

15

16  Desquamative interstitial Pneumonia  Respiratory bronchiolitis-associated interstitial lung disease  Pulmonary Langerhans cell histiocytosis

17  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  Increased number of pigmented macrophages in alveolar spaces  Thickened alveolar septae  Overall architecture maintained with very little honeycombing

19  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  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 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  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  Symptoms usually start in the 4 th -5 th decade  Slightly more males than females involved  Cough and dyspnea  50% have crackles and very few have clubbing

25  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  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  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

29 PLCH Histology  Nodular sclerosing lesions containing Langerhans’ cells  Bronchiolocent ric distribution  Progression  Fibrosis and cystic changes

30  Adults in 3 rd and 4 th 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  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  History and CT findings may be diagnostic  Surgical biopsy  Broncho alveolar lavage  > 5% of CD1a positive cells

35  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 Cancer  Chronic Obstructive airways disease  Interstitial lung diseases

38

39 1. 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– Atilli AK, Kazarooni EA, Gross BH et al, Smoking-related Interstitial Lung Disease: Radiologic- Clinical-Pathologic Correlation, RadioGraphics 2008; 28 : 1383– Caminati A, Harari S, Smoking-related Interstitial Pneumonias and Pulmonary Langerhans Cell Histiocytosis, Proc Am Thorac 2006; 3 : 299– Selman M, The Spectrum of Smoking-Related Interstitial Lung Disorders: The Never-Ending Story of Smoke and Disease, Chest 2003; 124 :


Download ppt "Dr. GJ Visagie.  43 yo Female Known with  Obesity Class 3 Recently lost 45 Kg  Obstructive sleep apnea  Pulmonary hypertension and Cor Pulmonale "

Similar presentations


Ads by Google