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Interstitiallungdiseases(ILD).docVIP

Interstitiallungdiseases(ILD).doc

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Interstitiallungdiseases(ILD)

Interstitial Lung Disease Egan’s Chapter 21 PAGE  PAGE 3 Interstitial Lung Diseases (ILD) Egan’s Chapter 21 Definition of interstitial lung diseases A group of over 130 diseases that first cause an inflammatory process in the alveolar walls (an alveolitis) but later or simultaneously an irreversible fibrosis, a increase in collagenous or elastic fiber in the interstitium. Incidence—81/100,000 Etiology Most are unknown Those of known etiology Pneumoconioses or Occupational Lung Diseases (Silicosis, Coal Miner’s, Asbestosis etc. see Box 21-1 in Egan) Also drug induced Chemotherapeutic or cytotoxic agents (Anti-tumor) Gold Some antibiotics (Nitrofurantoin, Sulfasalazine, Bleomycin etc. See Egan, Box 21-2 for full list) Radiation induced Pathology Alveolitis (possibly reversible) Fibrosis (scar tissue collageneous fibers—irreversible) When extensive-responsible for honeycomb appearance on x-ray Granulomas – cluster of phagocytic or macrophagic cells. Found in ILD where there is an immune response to an exogenous agent or in sarcoidosis where exogenous agent is unknown or nonexistent Pathophysiology Decreased compliance Secondary to increased elastic tissue in interstitium Faster respiratory rate, lower tidal volume Decrease in all lung volumes Lowered DLCO – mostly due to decreases in a-c membrane surface area? (thickening of fibrotic membrane) Some small airway dysfunction with low V/Q Hypoxemia with hypocapnia or normal CO2 Increased A-a gradient, which worsens with exercise secondary to diffusion problems Pulmonary hypertension secondary to decreased p.c. bed and chronic hypoxemia Cor pulmonale if g. (above) is severe and prolonged Clinical features Dyspnea, first on exertion and then at rest Velcro crackles (bilaterally, predominately in bases) Clubbing Tachypnea Jugular venous distension (JVD) late if pulmonary hypertension causes cor pulmonale Chest X-ray Reticulonodular or interstitial pattern In some types of ILD – hilar adenopathy Honeycombin

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