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Anti-GBM disease — ESENeph MCQ

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HardGlomerulonephritisAnti-GBM diseaseESENeph

A 28-year-old man has 5 days of haemoptysis, breathlessness and dark urine. Creatinine is 412 micromol/L, potassium 5.8 mmol/L and bicarbonate 16 mmol/L. Urine microscopy shows red cell casts; chest radiograph shows bilateral alveolar infiltrates. Anti-GBM antibody is strongly positive and renal biopsy shows linear IgG staining along the glomerular basement membrane. What is the most appropriate management?

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Correct answer: C — Plasma exchange, corticosteroid and cyclophosphamide

Anti-GBM disease with pulmonary haemorrhage and severe renal injury is a nephrological emergency requiring plasma exchange to remove antibody, plus corticosteroid and cyclophosphamide to suppress production. Steroid alone is inadequate for antibody-mediated pulmonary-renal disease. Rituximab may have niche use but is not the standard first-line regimen in this presentation. The clinical pearl is that linear IgG staining distinguishes anti-GBM disease from pauci-immune ANCA vasculitis. The competing options do not fit the renal phenotype, temporal relationship, treatment threshold or safety constraint described. Management still requires confirmation of current medicines, renal trajectory and relevant contraindications, followed by timely biochemical and clinical reassessment. Escalate urgently if life-threatening electrolyte disturbance, respiratory compromise, shock, neurological deterioration or a dialysis indication develops.

Reference: KDIGO 2021 Glomerular Diseases Guideline; UKKA/Renal Association guidance: https://kdigo.org/guidelines/gd/