Interstitial Lung Disease | Jindal Chest Clinics

Overview on Interstitial Lung Disease (ILD), including causes, symptoms, diagnostics, and management strategies. For more information, please contact us: 9779030507.

1. Interstitial Lung Disease

2. Interstitial Lung Disease Diseases of lung interstitium Not a single disease – over 250 different diseases with similar clinical and/or radiological features Also known as : Diffuse Parenchymal Lung Disease or Diffuse Lung disease because of Involvement of air spaces , vessels, airways, ?pleura - i.e. diffuse involvement Idiopathic pulmonary fibrosis (IPF) or Idiopathic Interstitial Pneumonias (IIPs) are sometimes used synonymously with ILD

3. Characteristics Common clinical, radiological, physiological and histo pathologic features Hetero genous conditions - Inflammatory - Granulomatous - Infections - Depositions - Oedema

4. Causes and Pathogenesis Exact etiology , not know – Idiopathic Secondary to other known causes Inflammation, damage and fibrosis Repair mechanisms (for inflammation) - aberrant Diffuse involvement Progressive

5. AETIOLOGICAL CLASSIFICATION Occupational and environmental exposures Connective tissue diseases Drugs and Poisons Infections (residual scars) Miscellaneous systemic dis IDIOPATHIC (or PRIMARY)

6. Primary ILDs - IIPs UIP: Usual Interstitial Pneumonia or IPF AIP: Acute Interstitial Pneumonia DIP: Desquamative Interstitial Pneumonia NSIP : Non-specific Interstitial Pneumonia LIP : Lipoid Interstitial Pneumonia OP: Organizing pneumonias

7. Secondary I.L.Ds Acquired /Systemic Inhertied CTDs • Familial Granulomatoses • Tuberous sclerosis Pneumoconiosis • Neurofibromatosis Infections • Metabolic storage disease R.B-I.L.D • H-P syndrome Hypersenstivity Miscellaneous Pneumonias • Idio . pulm . haemosiderosis Iatrogenic • Veno -occlusive disease Drugs, Radiations • L.A.M. Toxic gases,fumes • Histiocytosis

8. Connective Tissue Disorders PSS Skin and digital changes Rh arth Polyarticular arthritis SLE Multisystem disease PM-DM Skin and muscle Sjogren Dryness in eyes, mouth, glands Ank Spond Seroneg . Spondylo -arthritis Mixed CTD SLE, SSC and PM-DM Relapsing PC Polychondritis (nose, ear) Behcet Dis. Aphthous ulcers; genital

9. Iatrogenic ILD Drugs Cytotoxic: Bleomycin , mitoC , busulfan , BCNU Noncytotoxic : • Aspirin • Antibiotics – NFT, sulfasalazine • Anti arrythmics : Amiodasone , BB • Anti-inflammatory: NSAIDs, gold • Anticonvulsants: Dilantin, CM • Vasodilators: Hydralazine • Narcotics: Opiates, heroin • Miscellaneous: Penicillamine , vitamins Therapeutic radiation Oxygen toxicity

10. Drugs and Chemicals Chemotherapeutic agents Busulphan , Metho , Bleo 2. Antibiotics Misc. drugs: Dilantin, Gold, Procainamide, Aminodarone Fumes of Zn, Cu, Mn , Cd, Fe, Mg, Ni, Se, brass Aerosols: Fats Vapours : Resins, TDI, Hg Paraquat

11. SYSTEMIC DISEASES Sarcoidosis Vasculitides Wegener’s GM Churg Strauss Syn Good Pasture’s syn Idiopathic haemosiderosis Histiocytosis X Lymphangitis carcinomatosis Ch. Pulm oedema / uraemia Alveolar proteinois

12. Honey Comb Lung Irreversible end-stage disease Cystic space formation Dense fibrosis Metaplastic cuboidal epithelium Mucus formation Interstitial smooth m. proliferation Pulmonary vascular change Malignant change (potential)

13. DIAGNOSIS Clinical features Laboratory investigations: Hematological, biochemical, immunological etc. Radiology: CXR, HRCT Pulmonary function tests Bronchoscopy: BAL, lung biopsy Surgical lung biopsy

14. Diagnosis Issues Diffuse vs. Local Primary vs. Secondary Cause of secondary ILD Acute vs Chronic Disease activity and progression Responsive vs Nonresponsive ( to tmt ) 7. Presence of complications

15. DIFF. DIAGNOSIS Causes of breathlessness . Left heart failure . Pulm . Thromboembolism . Miscellaneous-anemia Respiratory Diseases . COPD . Bronchiectasis . Ch. Pneumonias- eosinophilic

16. SYMPTOMS Progressive breathlessness Cough (non productive) Onset: Insidious / acute Symptoms / history of underlying disease/exposure

17. PHYSICAL EXAMINATION Tachypnoea Reduced chest expansion / intercostal retraction Exercise induced cyanosis Clubbing Breath sounds – well heard Bibasilar end-inspiratory day (Velcro) crackles Pulm hypert / cor pulmonale Signs of CTD or other causes responsible for ILD

18. INVESTIGATIONS Haematological – leucocytosis , Polycythaemia / anaemia Biochem : (renal, Liver) Raised ESR, hypergammaglob . Immunological tests: LE cell, Rhematoid factor, Antinuclear Ab., Immune complexes Cardiac function Others: ACE, LDH, antibodies (etc.)

19. RADIOLOGICAL STUDIES What is most helpful? Diagnosis Disease extent Progression Radiological findings: distribution and pattern Correlation Cost-effectiveness

20. CLASSICAL RADIOGRAPH Basilar / diffuse shadows reticular/nodular/RN Mixed patterns ? Honey combing Decreased volume Pulm hypertension

21. ATYPICAL PATTERNS Upper zone predominance: Granulomatous dis., Pneumoconioses Increased volume: LAM , Sarcoidosis Pleural involvement: CTD , sarcoidosis , Asbestosis , Drugs Miliary pattern : TBL, Granulomatoses

22. 5. Hilar / mediastinal LN Sarcoidosis Lymphoma / Lymphangitis Pneumothorax Histocytosis X / LAM Kerley B lines Normal CXR

23. HIGH RESOLUTION CT Better resolution / more accuracy Earlier detection Better assessment of extent and distribution Occult adenopathy Selection of biopsy site

24. PULM. FUNCTION TEST Restrictive pattern Reduced TLC, VC FRC and RV Flow rates: Reduced due to decreased VC . Obstructive/mixed pattern : smoking/other causes Compliance : Low DLCO – Reduced Blood gases

25. BRONCHO ALVEOLAR LAVAGE Nonspecific Narrow down diff. diagnosis Defines stage of disease Assessment of progression Assessment of treatment response

26. ACTIVITY ASSESSMENT Symptomatology Chest radiography Pulm . Function tests BAL ? TBLB Scanning – Ga-67, TC 99m DTPA

27. TREATMENT

28. Objectives of Treatment of ILDs Symptomatic relief Slow down disease progression Prevent/ Treat complications Prolong survival Improve Quality of Life Prevent drug-induced problems End of Life Care

29. Treatment Principles I . Secondary ILDs Treatment of ILD of a known primary cause essentially comprises the treatment of the primary disorder. Symptomatic Anti-inflammatory Supportive II. Primary ILD (Idiopathic Interstitial Pneumonias) and Pulmonary Fibrosis

30. Treatment of IIP/IPF Of all IIPs, Idiopathic Pulmonary Fibrosis (IPF) i.e. U.I.P. is the most common form. It is associated with an extremely poor prognosis for survival in most patients. Life expectancy after diagnosis varies, but is on average less than 5 years.

31. Current Drug Treatment 1. Corticosteroids 2. Immunosuppressive drugs: Azathioprine , Cyclophosphamide , Cyclosporine 3. Antifibrotic drugs: Pirfenidone , Colchicine , D- penicillamine , Pentoxyfylline 4. Anti-oxidants: N . Acetyl-cysteine

32. Supportive Treatment Underlying cause – Identification and management Oxygen therapy Management of pulmonary hypertension and cardiac failure Pulm . Vasodilators Diuretics 4. Antibiotics for infections 5. Miscellaneous

33. End Stage Disease Lung Transplantation Palliative End of Life Care Domicilliary Oxygen Symptomatic relief Discontinuation of steroids and immunosuppressive drugs

34. Sarcoidosis Multi-system granulomatous disorder Unknown etiology Pulmonary features > 80% (Interstitial infiltrates/fibrosis) Extrapulmonary (Eyes , skin, liver , spleen, nervous systme , cardiac etc): Approx . 30% Increasing recognition Diagnosis primarily on lung biopsy; Serum ACE Treatment: Corticosteroids and other anti-inflammatory drugs

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