Nelson Textbook of Pediatrics 22nd Edition | Granulomatosis with polyangiitis (GPA, Wegener): c-ANCA (PR3), cavitary nodules, sinusitis, glomerulonephritis. Sarcoidosis: noncaseating granulomas, BAL CD4/CD8 >4, bilateral hilar lymphadenopathy, erythema nodosum. Berylliosis: chronic beryllium disease, BeLPT positive. GLILD: granulomatous and lymphocytic interstitial lung disease in CVID. Diagnosis: HRCT, ANCA, ACE, BeLPT, lung biopsy. Treatment: corticosteroids, cyclophosphamide/rituximab for GPA, antigen avoidance for berylliosis.
🌐 paeds.online — Pakistan's Pediatric Platform| Disease | Key Diagnostic Finding | Treatment | Granulomatosis with polyangiitis (GPA) | c-ANCA (PR3) in 90%, cavitary nodules, glomerulonephritis | Cyclophosphamide or rituximab + corticosteroids (induction); methotrexate or azathioprine (maintenance) |
|---|---|---|
| Sarcoidosis | BAL CD4/CD8 >4, noncaseating granulomas, elevated ACE | Corticosteroids for progressive lung disease, ocular disease, hypercalcemia |
| Berylliosis (Chronic beryllium disease) | History of beryllium exposure, BeLPT positive | Antigen avoidance, corticosteroids |
| GLILD (with CVID) | CVID + granulomas + lymphocytic interstitial pneumonia | IVIG, corticosteroids, rituximab, azathioprine |