The document discusses interstitial lung disease (ILD), including its common features, types, causes, diagnostic approach and treatment. It describes various ILD types such as idiopathic pulmonary fibrosis and sarcoidosis. Imaging and biopsy are used to diagnose ILD and determine prognosis. Treatment involves identifying and removing environmental causes, suppressing inflammation, and managing complications like right heart failure.

1. Approach to INTERSTITIAL LUNG DISEASE Prof. Dr. MAGHESHKUMAR Unit Dr. Devendra Patil

2. 52 / F comes with complains of Cough with minimum mucoid expectoration 6-7 yrs DOE gradually progressive 3-4 yrs HOPI :- No H/o fever, No h/o pul TB No h/o palpitations,PND , orthopnea, O/e: Tachypnoea and Bibasilar Inspiratory Crackles Clubbing +nt. X ray was advised and it showed some B/L interstitial opacities

3. How to suspect an INTERSTITIAL LUNG DISEASE. How to find its Cause How to differentiate using imaging and simpler procedure rather than doing a TBLB or Open lung biopsy Which ILDs have good prognosis Whats the Supportive Treatment

4. COMMON FEATURES OF ILD History : Chronic non productive cough with progressive exertional dysnoea. Examination :- Tachypnoea +/- Respiratory distress Cynosis and clubbing Bibasilar Inspiratory crackles f/s/o pul HT and cor pulmonale IMAGING : - Interstitial pattern PFT:- Restrictive pattern DLco :- Reduced

5. IDIOPATHIC INTERSTITIAL PNEUMONIA NS- UIP AIP COP/BOOP DIP RB-ILD IPF Smoking related Due to KNOWN CAUSE Environmental Pneumoconiosis HP Gases n fumes Iatrogenic Drugs Irradiation Microbes DCTD GRANULOMATOSIS sarcoidosis Langerhans cell histiocytosis Wegener's granulomatosis, Churg-Strauss Syndrome RARE ILD alv.proteinosis alv.microlithiasis amyloidosis eosinophilic pneumonia lymphangioleiomyomatosis idiopathic pulmonary hemosiderosis INTERSTITIAL LUNG DISEASE

6. INTERSTITIAL LUNG DISEASE On basis of PFT and DLco Is it due to environmental / iatrogenic factors Avoid those factors and monitor response Is it due to a systemic disease Or microbial origin No response Serology Skin Biopsy Sputum c/s HRCT and BAL TBLB or Open Lung Biopsy Can Diagnosis and prognosis be established HISTORY

7. ILD with obstructive component Sarcoidosis Hypersensitivity pneumonitis Langerhans cell granulomatosis Lymphangioleiomyomatosis Tuberous sclerosis Combined COPD and ILD RELATIVE CONTRA INDICATIONS FOR A LUNG BIOPSY Honey combing or evidence of end stage disease Severe pulmonary dysfunction Major operative risk

8. Environment Dependent ILD MINING INDUSTRY : Coal workers pneumoconiosis Silicosis Asbestosis HYPERSENSITVE PNEUMONITIS GAS or FUME Exposure

9. Coal miners pneumoconioisis Rounded opacities between 1 and 5 mm (upper and middle zones) small irregular and linear opacities Progressive massive fibrosis almost always starts in an upper zone Calcification is not a feature Cavitation of PMF can occur Caplan's syndrome is the name given to the combination of rheumatoid disease and several round nodules (usually 1 to 5 cm in diameter) in the lungs of a coal miner.

10. SILICOSIS Clues to diagnosis Micronodular pattern Simple silicosis : Upper lobes Small multiple nodules Egg shell calcification Complicated : >1 cm nodules Acute silicosis : small nodular pattern with ground glass appearance ( crazy paving ) PMF : nodules coalesce to large masses BAL : dust particles on polarised light

11. Clues to diagnosis X Ray: reticular interstitial pattern pleural plaques ( lower lung field , cardiac border and diaphragm ) Irrregular linear opacities first noted in lower lung fields. HRCT : Distinct subpleural curvilinear opacities 5-10 mm length parallel to pleural surface BAL: Asbestos bodies ASBESTOSIS

12. HISTORY of exposure to an offending antigen Temporal association +nt characteristic signs and symptoms PFT and Imaging ( ILD pattern ) presence of granulomatous inflammation Absence of eiosinophilia BAL : marked lymphocytosis > 50% HYPERSENSITIVITY PNEUMONITIS

13. Suspect a CTD if Musculosketetal pain Weakness Fatigue Joint pains and swelling Photosensitivity Raynauds phenomenon Pleuritis Dry eyes or mouth INTERSTITIAL LUNG DISEASE in CTD

14. SYSTEMIC SLEROSIS Lung manifestation may be first SS sign in 55% Lung involvement +nt in 90 % ( detected by PFT ) Vascular Involvement is not vasculitis but intimal hypertrophy ( CREST ) RA MC lung manifestation : Fibrosing alveolitis Male predominance Pleural disease Pleuro pulmonary nodules (may cavitate to produce pneumothorax ) Caplan Syndrome SLE ILD is rare . Pleural involvement is common POLYMYOSITIS / DERMATOMYOSITIS ILD in 10 % a combination of patchy consolidation with a peripheral reticular pattern being highly characteristic.

15. HRCT in RA bibasilar peripheral reticular pattern, intralobular interstitial thickening distortion of the lung parenchyma Bilateral is present, predominantly on the left side bibasilar peripheral reticular pattern, pleural effusion thickening of the interlobular septa,

16. INTERSTITIAL LUNG DISEASE in VASCULITIC DISORDERS Vasculitic Disorders Lung Involvement ANCA Interstial Pattern seen Wegener granulomatosis Common c-ANCA >> p-ANCA 80–90% Diffuse Alveolar Hemorrage with nodules ,cavitation Microscopic polyangiitis Common Common p-ANCA > c-ANCA 80% DAH Churg-Strauss syndrome Common p-ANCA > c-ANCA 30–50% DAH with transient infiltates Goodpasture syndrome Common p-ANCA 10% DAH Takayasu arteritis Common Negative “

17. X ray : consolidation, typically resolving within a matter of days, multiple abcesses HRCT : ground-glass partial alveolar filling. Hb : anaemia ( iron defeciency ) BAL :- frank blood-staining in sequential lavage (acute presentation) and numerous macrophages containing iron, identified by Perl's stain Dlco :- may be increased in acute conditions but is chronically low MC seen is Wegeners Granulomatosis ILD in VASCULITIC DISORDERS Suspect if Mononeuritis mutiplex Renal involvement Skin lesions haemoptysis

18. DRUG and IRRADIATION and GAS DRUGS Amiodarone Bleomycin Busulphan Carmustine Chlorambucil Cyclophosphamide Cytosine arabinoside Lomustine ….) RADIATION

19. IDIOPATHIC INTERSTITIAL PNEUMONIA NS- UIP AIP COP/BOOP DIP RB-ILD IPF Smoking related Due to KNOWN CAUSE Environmental Pneumoconiosis HP Gases n fumes Iatrogenic Drugs Irradiation Microbes DCTD GRANULOMATOSIS sarcoidosis Langerhans cell histiocytosis Wegener's granulomatosis, Churg-Strauss Syndrome RARE ILD alv.proteinosis alv.microlithiasis amyloidosis eosinophilic pneumonia lymphangioleiomyomatosis idiopathic pulmonary hemosiderosis INTERSTITIAL LUNG DISEASE

20. UIP or IPF MC of all chronic ILD Typical c/f presentation Median survival approximately 3 years, depending on stage at presentation. B/L Reticular bibasilar and subpleural opacities. minimal ground-glass and variable honeycomb change. Type I pneumocytes are lost, and there is proliferation of alveolar type II cells. "Fibroblast foci" of actively proliferating fibroblasts and myofibroblasts.

21. Disease Age M:F C/F Imaging Prognosis REMARKS Respiratory bronchiolitis- associated interstitial lung disease younger Heavy smokers with similar complains Like UIP with Airtrapping Emphysematous change survival greater than 10 years Spontaneous remission 20%. ILD with Obstructiv pattern Acute interstitial pneumonitis Hamman-Rich syndrome. young Apparently normal indistinguishable from that of idiopathic ARDS ARDS Diffuse b/l airspace consolidation with areas of ground-glass attenuation POOR Most severe formof ILD Pneumonia

22. Disease Age M:F C/F Imaging Prognosis REMARKS Nonspecific interstitial pneumonitis (NSIP) 40-50 May be indistinguishable from UIP Like But uniform in time, suggesting response to single injury UIP Honeycombing is rare. Prognosis good but depends on the extent of fibrosis at diagnosis greater than 10 years. But Surgical Biopsy is needed to confirm. Cryptogenic organizing pneumonitis (bronchiolitis obliterans organizing pneumonia [BOOP]) 50–60 Abrupt onset, frequently weeks to a few months following a flu-like illness. constitutional symptoms are common Ground glass infiltrate subpleural consolidation and bronchial wall thickening and dilation. Xray – interstitial pattern with nodules Good Rule out infection and treat with steroids

23. Acute interstitial pneumonitis

24. Nonspecific interstitial pneumonitis (NSIP)

25. Cryptogenic organizing pneumonitis (bronchiolitis obliterans organizing pneumonia [BOOP])

26. Smoking related ILD Respiratory bronchiolitis- associated interstitial lung disease

27. IDIOPATHIC INTERSTITIAL PNEUMONIA NS- UIP AIP COP/BOOP DIP RB-ILD IPF Smoking related Due to KNOWN CAUSE Environmental Pneumoconiosis HP Gases n fumes Iatrogenic Drugs Irradiation Microbes DCTD GRANULOMATOSIS sarcoidosis Langerhans cell histiocytosis Wegener's granulomatosis, Churg-Strauss Syndrome RARE ILD alv.proteinosis alv.microlithiasis amyloidosis eosinophilic pneumonia lymphangioleiomyomatosis idiopathic pulmonary hemosiderosis INTERSTITIAL LUNG DISEASE

28. Sarcoidosis Incidental X-ray (20-30 %) Cough , chest discomfort ( upto 50 – 60 % ) Skin lesions ( 20 -25 % )

30. SARCOIDOSIS ctd…. BAL :- lymphocytosis CD4 : CD8 > 3.5 is most specific PFT :- Restrictive pattern But Obstructive component present in many Biopsy :- non caseating granulomas lymphocytosis Sr. ACE levels:- Hyper calciuria or Hypercalcemia

31. RARE ILD

32. Primary Alveolar Microlithiasis perilobular and bronchovascular distribution of microliths and subpleural consolidation with calcifications in the right lung SAND STORM appearance

33. Pulmonary Alveolar Proteinosis diffuse reticulo-alveolar infiltrates BAT WING distribution BAL :- milky effulent foamy macrophages with lipoproteinous intraalveolar material thickened interlobular septa “ crazy paving” ground glass fashion, sharply demarked from normal lung creating a “geographic” pattern.

34. TREATMENT Removal of offending agent if noted Aggressive suppression on inflammatory response Supportive management ( O 2 or ) Treatment of Right heart Failure Treatment of Infections Combined effort from family , doctors , physioherapists.

35. CYCLOPHOSPHAMIDE or AZATHIOPRINE IPF Other ILD as 2 nd line drugs 1-2 mg / kg /day with or without steroids STEROIDS BOOP CTD – ILD Eiosinophilic pneumonia Inorganic Dust ILD Vasculitic ILD Organic Dust Dose :- 0.5 – 1 mg / kg prednisone for 4 – 12 weeks and then gradual tapering of the dose with repeated monitoring for flare up activity

36. THANK - - YOU References: Harrisons 16/e Atlas Of ILD by OP Sharma Oxford’s Text book of Medicine 4/e