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?

  1. A Rapidly progressive glomeruionephri7s
  2. B Henoch Schönlein purpura
  3. C Goodpastures syndrome Correct answer
  4. D Lupus nephri7s

Explanation

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).

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