Pediatrics
A 5-year-old child presents with features of glomerulonephri7s.
A week later, she developed haemoptysis. Which of the following is the most likely diagnosis?
الشرح
Core Concept: Goodpasture's syndrome (Anti-GBM disease) is a rare autoimmune disease caused by anti-glomerular basement membrane (anti-GBM) antibodies attacking the alveolar and glomerular capillaries, leading to a pulmonary-renal syndrome.
Clinical Presentation:
- Classic symptoms: Hemoptysis (pulmonary hemorrhage) and rapidly progressive glomerulonephritis (hematuria, acute renal failure).
- Complications: End-stage renal disease (ESRD) and life-threatening respiratory failure.
Diagnosis:
- Diagnostic algorithm: Clinical presentation of pulmonary-renal syndrome, followed by serology and biopsy.
- First-line diagnostic plan: Serology showing anti-GBM antibodies, ANCA (to rule out vasculitis), and ANA.
- Gold standard: Renal or lung biopsy showing linear IgG deposition along the basement membrane on immunofluorescence.
Management:
- Acute management: Plasmapheresis to rapidly remove circulating antibodies, combined with high-dose corticosteroids and cyclophosphamide.
- Long-term/Definitive management: Immunosuppression maintenance; dialysis or kidney transplant if ESRD develops (transplant is delayed until anti-GBM antibodies are undetectable).