Nelson Textbook of Pediatrics 22nd Edition | Pulmonary fibrosis in children is rare and usually secondary to underlying conditions. Causes: surfactant dysfunction (SFTPC, ABCA3), hypersensitivity pneumonitis (chronic), connective tissue diseases (SLE, scleroderma, JIA, dermatomyositis), drug-induced (bleomycin, methotrexate, nitrofurantoin, amiodarone), radiation fibrosis, post-infectious (adenovirus, severe pneumonia), chronic aspiration, sarcoidosis, Langerhans cell histiocytosis, and idiopathic (very rare in children). Clinical: dyspnea, hypoxemia, crackles, digital clubbing, failure to thrive. PFTs: restrictive pattern (↓FVC, ↓TLC, ↓DLCO). HRCT: reticulation, honeycombing, traction bronchiectasis. Diagnosis: HRCT, lung biopsy (gold standard). Treatment: supportive (oxygen), treat underlying cause, corticosteroids, immunosuppression (mycophenolate, rituximab, cyclophosphamide), antifibrotics (nintedanib, pirfenidone – off-label in children), lung transplantation.
🌐 paeds.online — Pakistan's Pediatric Platform| Cause | Treatment Approach |
|---|---|
| Surfactant dysfunction (SFTPC, ABCA3) | Hydroxychloroquine, azithromycin, supportive care, lung transplantation |
| Hypersensitivity pneumonitis (chronic) | Antigen avoidance, corticosteroids, immunosuppression |
| Connective tissue disease-associated | Treat underlying disease, mycophenolate, rituximab, cyclophosphamide |
| Drug-induced | Discontinue offending drug, corticosteroids |
| Idiopathic pulmonary fibrosis (IPF) | Antifibrotics (nintedanib, pirfenidone – off-label in children), lung transplantation |