Ild diagnosis

interstitial lung disease

1. DIAGNOSIS OF INTERSTITIAL LUNG DISEASE ANUSHA CM

2. DEFINATION CLASSIFICATION PATHOGENESIS HISTORY AND RISK FACTORS SIGNS AND SYMPTOMS LABORTORY EVALUATION RADIOGRAPH BRONCHOSCOPY SURGICAL LUNG BIOPSY

3. PULMONARY INTERSTIUM Interstitial compartment is the portion of the lung sandwiched between the epithelial and endothelial basement membrane Expansion of the interstitial compartment by inflammation with or without fibrosis

4. DEFINITION ILD refers to a heterogeneous collection of more than one hundred distinct lung disorders that tend to be grouped together because they share clinical, radiographic, and pathologic features

5. PATHOGENESIS Few cells in the interstitium of the normal lung Injury to basement membrane shared by epithelium and endothelium Increased alveolar permeability and spillage of serum contents into the alveolar space and recruitment of fibroblasts Collagen deposition Also injury to small airways=respiratory bronchioles, alveolar ducts and terminal bronchioles

6. CLASSIFICATION

7. RISK FACTORS 1.AGE Age 20-40 years - Sarcoidosi s , CTD, LAM > 50years - Idiopathic pulmonary fibrosis

8. 2. SMOKING Diseases associated with smoker: EG ( histocytosis X) Desquamative interstitial pneumonitis Respiratory broncholitis Diseases less likely to be seen in smoker: Hypersensitivity pneumonitis Sarcoidosis

9. 3. OCCUPATION

10. 4. FAMILY HISTORY Autosomal dominant pattern: IPF Sarcoidosis Neurofibromatosis- I Hermansky–Pudlak syndrome Burt–Hogg–Dubé syndrome Autosomal recessive pattern : Gauchen’s disease Neimann pick

11. DRUG INDUCED ILD

12. SYMPTOMS Dyspnea - predominent symptom Cough(dry )- Second most frequent symptom. In sarcoidosis , HP or organising pneumonia Substernal chest pain - sarcoidosis . Wheezing- hypersensitivity pneumonitis , eosinophilic pneumonia, or sarcoidosis . Pleuritic chest pain- C onnective tissue disease, or pneumothorax in cystic lung diseases such as lymphangioleiomyomatosis (LAM) and Langerhans cell histiocytosis (LCH).

13. Hemoptysis - diffuse alveolar hemorrhage, pulmonary veno -occlusive disease, mitral stenosis , LAM, granulomatous vasculitides . New onset of hemoptysis in a patient with known ILD suggests a complicating malignancy. Gastrointestinal symptoms - indicative of underlying esophageal motility problems related to connective tissue disease like systemic sclerosis and polymyositis In particular, symptoms suggestive of acid reflux chest burning or pressure, cough after meals, regurgitation of food Bloating and diarrhea - inflammatory bowel disease or bacterial overgrowth due to bowel dysmotility in systemic sclerosis.

14. Arthralgias , morning stiffness, joint swelling and erythema , and deformities points towards Rheumatoid arthritis, Sjögren syndrome or mixed connective tissue disorder Swollen fingers (“sausage digits”) - systemic sclerosis and polymyositis . Raynaud’s phenomenon - Associated with digital ulcerations and, rarely, digital gangrene: suggestive of underlying scleroderma, mixed connective tissue disease, SLE, and antisynthetase syndrome

15. Ophthalmologic symptoms- dry eyes or the use of eye drops may uncover sicca syndrome, as seen in Sjögren syndrome and overlap connective tissue diseases. U veitis - SLE or sarcoidosis Neurologic symptoms - vasculitis or sarcoidosis . Oculocutaneous albinism and colitis points to Hermansky pudlak syndrome

16. SAUSAGE DIGITS IN SYSTEMIC SCLEROSIS

17. DURATION OF SYMPTOMS Acute (less than 3 weeks) (e.g. drug reaction , acute hypersensitivity Pneumonitis , chemical exposure) Subacute : 3-12 weeks (e.g. COP ) Insidious over months or years (e.g., IPF)

18. SIGNS Velcro crepts - B/L basal inspiratory - common in most forms of ILD. Less likely to be heard in sarcoidosis . Inspiratory squeaks and wheeze - B ronchiolitis / and obstruction Clubbing: most commonly seen in IPF but non-specific. Cor pulmonale Cyanosis in late stage of ILD. Skin thickening and acral cyanosis- scleroderma

19. Cutaneous vasculitis- churg strauss syndrome Edematous cyanotic skin- Dermatomyositis Palpable lymph nodes , hepatosplenomegaly - suggests Sarcoidosis, HIV infection or CTD

20. LAB TESTS CBC, MR, LFT, ANA PROFILE, c ANCA, p ANCA, RA factor Peripheral eosinophilia > 10% Churg -Strauss syndrome Chronic eosinophilic pneumonia Serum precipitating antibodies: Hypersensitivity pneumonitis

21. PULMONARY FUNCTION Most of the ILD have a restrictive defect. Mixed pattern: Sarcoidosis Hypersensitivity Pneumonitis (HP) Histocytosis X Lymphangioleiomyomatosis (LAM) Wegener’s granulomatosis Broncholitis obliterans organizing pneumonia (BOOP) rarely present with mixed pattern

22. Moderate - severe reduction in DLCO but normal lung volumes in a patient with ILD suggest: COPD with ILD Pulmonary Vascular Disease Pulmonary histocytosis X Lymphangioleiomyomatosis A reduction in DLCO is common but nonspecific. The severity of the DLCO reduction does not correlate well with disease stage. During follow up , esp in IPF about 5-10% reduction in FVC over 6 months is indicative of increased mortality

23. CHEST RADIOGRAPH Chest radiograph is normal in 10% of patients with ILD (particularly those with HP). A diffuse reticulonodular pattern , ground glass opacities or both are most common findings on radiograph

24. DISTRIBUTION OF ILD

25. HRCT FINDINGS High-resolution computed tomography (HRCT) of the chest is significantly more sensitive than chest radiograph for abnormalities in ILD. The characteristic radiographic features of IPF are collectively known as the “UIP pattern,” since these features have been demonstrated to confidently predict the presence of pathologic UIP when surgical biopsy is obtained This pattern consists of peripheral, subpleural, basilar-predominant reticular opacities in combination with basilar honeycombing and without features, such as ground-glass opacities, cysts or nodules, to suggest another form of ILD

26. Composite physiologic index (CPI) reflects the extent of fibrosis in HRCT An increase in CPI indicates progression of fibrosis and is associated with increased mortality

27. PATTERN OF ILD

28. GROUND GLASS PATTERN Hyper sensitivity Pneumontis PCP pneumonia D iffuse Interstitial Pneumonia Non Specific Intersititial Pneumonia Alveolar hemorrhage

29. CYSTS OR CYST LIKE LAM Bronchiectasis

30. NSIP pattern in a 64-year-old man. HRCT features of NSIP include extensive ground-glass areas in the lung (black arrows) and traction bronchiectasis.

31. COP pattern in a 53-year-old man. HRCT images show characteristic peripheral and bronchocentric consolidations. The “air bronchogram sign” is clearly recognisable in A. Tendency to migration and changing location are also typical imaging findings of COP

32.   Acute interstitial pneumonia- bilateral dependent ground glass opacity

33. Diffuse interstitial pneumonia- ground glass densities with mosaic pattern. These are reversible and disappear with smoking

34. RB-ILD : Ground-glass opacity and centrilobular nodules  

35. ATS/ERS International IPF guidelines

36. In particular, a peripheral reticular pattern, that is basilar predominant may be seen in IPF, NSIP, and connective tissue disease– associated ILD Nodular infiltrates may suggest sarcoidosis , hypersensitivity pneumonitis , and LCH The finding of diffuse cystic abnormalities leads to a specific differential diagnosis, including LAM, LCH, and LIP The term “ground glass” refers to areas of lung tissues with increased attenuation, but not enough to obscure or distort lung architecture, blood vessels, and lymphatics Alveolar opacities are a related finding and reflect more dense attenuation of lung tissue, sometimes containing air bronchograms

37. BRONCHOSCOPY Includes BAL, TBLB, TBNA for cytologic or histological analysis Endobronchial lesions: - Sarcoidosis - Wegener’s granulomatosis - Inflammation and stricture of the major airways Transbronchial bx : Sarcoidosis (75-80%) Lymphangitic carcinomatosis (80%) Eosinophilic pneumonia Pulmonary alveolar proteinosis Pulmonary histocytosis X Good pasture’s syndrome LCH Lymphoid interstitial pneumonia

38. BAL : normal count: - CD4:CD8 = 1.5 - Macrophage 85% - Lymphocyte 5-10% - Neutrophils < 2% - Eosinophils < 1 % Lymphocytic BAL (>30%): Sarcoidosis or hypersensitivity pneumonitis . Neutrophilic BAL (>60%): Infection or ARDS Eosinophilic BAL (>30%): Acute or chronic eosinophilic pneumonia; coccidioidomycosis

39. Elevated CD4/CD8 ratio >2 in early, active sarcoidosis (may be lower in chronic or quiescent disease) CD4/CD8 < 1 in hypersensitivity pneumonitis . The utility of BAL in the clinical assessment of disease progression or response to therapy still to be established.

40. In several patients with probable or possible IPF, bronchoscopy may be used to rule out alternative diagnoses like HP esp if VATS biopsy is too risky like in elderly patients However especially in IPF , bronchoscopy and BAL have the potential of triggering acute exacerbation of IPF , so that the indication for bronchopic evaluation should be discussed in critically in every individual patient

41. SURGICAL BIOPSY Not required to make the diagnosis in all patient with ILD. However, it is not frequently possible to reach a definitive diagnosis or to stage a disease without examination of lung tissue. In IPF , histologic analysis from surgical biopsy is no longer golden standard due to sampling error and no uniformity of disease pattern in advanced cases Video-assisted thoracoscopic lung biopsy is the preferred method of obtaining lung tissue. Consiquently in IPF , multidisciplinary discussion (MDD) involving the pulmonologist, pathologist and radiologist has become the gold standard for diagnosis

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