Nelson Textbook of Pediatrics 22nd Edition | Lymphoproliferative lung disorder characterized by hyperplasia of lymphoid follicles along bronchi and bronchioles. Associated with immune dysregulation: common variable immunodeficiency (CVID), granulomatous and lymphocytic interstitial lung disease (GLILD), COPA mutations (autosomal dominant, follicular bronchiolitis, arthritis, nephritis), autoimmune disease (SLE, JIA). Clinical: chronic cough, crackles, hypoxemia, failure to thrive, recurrent infections. Diagnosis: HRCT (nodules, ground-glass opacities, hilar lymphadenopathy), lung biopsy (lymphoid follicles). Treatment: corticosteroids, IVIG for CVID, immunomodulators (rituximab, azathioprine, mycophenolate).
🌐 paeds.online — Pakistan's Pediatric Platform| Condition | Key Features | Treatment |
|---|---|---|
| Follicular bronchitis (isolated) | Lymphoid follicles on biopsy, no immune deficiency | Corticosteroids, supportive care |
| Follicular bronchitis with CVID | Hypogammaglobulinemia, recurrent infections, GLILD | IVIG replacement + corticosteroids, rituximab, azathioprine, mycophenolate |
| COPA syndrome | Autosomal dominant, follicular bronchiolitis, arthritis, nephritis |