Interstitial Lung Disease (ILD)| Jindal Chest Clinic

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

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

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

AETIOLOGICAL CLASSIFICATION 1. Occupational and environmental exposures 2. Connective tissue diseases 3. Drugs and Poisons 4. Infections (residual scars) 5. Miscellaneous systemic dis 6. IDIOPATHIC (or PRIMARY)

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

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

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

Iatrogenic ILD 1. 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, 2. Therapeutic radiation 3. Oxygen toxicity

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Drugs and Chemicals 1. Chemotherapeutic agents Busulphan, Metho, Bleo 2. Antibiotics 3. Misc. drugs: Dilantin, Gold, Procainamide, Aminodarone 4. Fumes of Zn, Cu, Mn, Cd, Fe, Mg, Ni, Se, brass 5. Aerosols: Fats 6. Vapours: Resins, TDI, Hg 7. Paraquat

SYSTEMIC DISEASES 1. Sarcoidosis 2. Vasculitides Wegener’s GM Churg Strauss Syn 3. Good Pasture’s syn 4. Idiopathic haemosiderosis 5. Histiocytosis X 6. Lymphangitis carcinomatosis 7. Ch. Pulm oedema / uraemia 8. Alveolar proteinois

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)

DIAGNOSIS • Clinical features • Laboratory investigations: Hematological, biochemical, immunological etc.

• Radiology: CXR, HRCT • Pulmonary function tests • Bronchoscopy: BAL, lung biopsy • Surgical lung biopsy

Diagnosis Issues 1.

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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

DIFF. DIAGNOSIS 1. Causes of breathlessness . Left heart failure . Pulm. Thromboembolism . Miscellaneous-anemia 2. Respiratory Diseases . COPD . Bronchiectasis . Ch. Pneumonias- eosinophilic

SYMPTOMS • • • •

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

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

INVESTIGATIONS 1.

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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.)

RADIOLOGICAL STUDIES 1. What is most helpful?

Diagnosis Disease extent Progression 2. Radiological findings: distribution and pattern 3. Correlation 4. Cost-effectiveness

CLASSICAL RADIOGRAPH • Basilar / diffuse shadows reticular/nodular/RN • Mixed patterns ?

• Honey combing • Decreased volume • Pulm hypertension

ATYPICAL PATTERNS 1.

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Upper zone predominance Granulomatous dis.

Pneumoconioses Increased volume LAM Sarcoidosis Pleural involvement CTD, sarcoidosis Asbestosis, Drugs Miliary pattern : TBL Granulomatoses

5. Hilar / mediastinal LN Sarcoidosis Lymphoma / Lymphangitis 6. Pneumothorax Histocytosis X / LAM 7. Kerley B lines 8. Normal CXR

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

PULM. FUNCTION TEST 1. Restrictive pattern Reduced TLC, VC FRC and RV 2. Flow rates: Reduced due to decreased VC . Obstructive/mixed pattern : smoking/other causes 3. Compliance : Low 4. DLCO – Reduced 5. Blood gases

BRONCHO ALVEOLAR LAVAGE 1.

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Nonspecific Narrow down diff. diagnosis Defines stage of disease Assessment of progression Assessment of treatment response

ACTIVITY ASSESSMENT 1.

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Symptomatology Chest radiography Pulm. Function tests BAL ? TBLB Scanning – Ga-67, TC99m DTPA

TREATMENT

Objectives of Treatment of ILDs 1.

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

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

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.

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

Supportive Treatment 1. Underlying cause – Identification and management 2. Oxygen therapy 3. Management of pulmonary hypertension and cardiac failure • Pulm. Vasodilators • Diuretics 4. Antibiotics for infections 5. Miscellaneous

End Stage Disease 1.

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

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 antiinflammatory drugs