Idiopathic pulmonary fibrosis
Presents with progressive SOB, dry cough and fine inspiratory crackles and loud P2 on auscultation.
Diagnosis:-
1. Spirometry shows decreased FEV1, decreased FVC and normal or increased FEV1/FVC (i.e. FEV1/FVC > 70%); Spirometry shows restrictive patterns.
2. Chest x-ray shows reticulonodular pattern.
3. HRCT of the chest shows reticulonodular pattern (i.e. honeycombing appearance).
4. DLCO is decreased.
1. Spirometry shows decreased FEV1, decreased FVC and normal or increased FEV1/FVC (i.e. FEV1/FVC > 70%); Spirometry shows restrictive patterns.
2. Chest x-ray shows reticulonodular pattern.
3. HRCT of the chest shows reticulonodular pattern (i.e. honeycombing appearance).
4. DLCO is decreased.
Management:-
1. Oxygen supplementation.
2. Pirfenidone (i.e. anti-fibrotic agent that inhibits TGF-b mediated synthesis of collagen).
3. Nintedanib (i.e. tyrosine kinase inhibitor that blocks fibrogenic growth factors and inhibits fibroblasts).
4. Lung transplantation is the only curative treatment.
5. Stop smoking.
1. Oxygen supplementation.
2. Pirfenidone (i.e. anti-fibrotic agent that inhibits TGF-b mediated synthesis of collagen).
3. Nintedanib (i.e. tyrosine kinase inhibitor that blocks fibrogenic growth factors and inhibits fibroblasts).
4. Lung transplantation is the only curative treatment.
5. Stop smoking.
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