A 48-year-old man presents with hemoptysis, hematuria, and rapidly progressive glomerulonephritis (creatinine rising from 1.0 to 4.2 mg/dL in 2 weeks). CXR shows bilateral pulmonary infiltrates. Urinalysis shows RBC casts. cANCA (anti-PR3) is positive at high titer. Renal biopsy shows pauci-immune crescentic glomerulonephritis. Induction therapy is:
- A Azathioprine and oral prednisolone
- B IV methylprednisolone plus cyclophosphamide or rituximab ✓
- C Plasma exchange alone
- D Mycophenolate mofetil and prednisolone
Explanation
Granulomatosis with polyangiitis (GPA, formerly Wegener's) with pulmonary-renal syndrome and rapidly progressive GN requires urgent induction with high-dose IV methylprednisolone pulses (3 × 500–1000 mg) followed by oral prednisolone plus either cyclophosphamide or rituximab. RAVE and RITUXVAS trials established rituximab non-inferiority to cyclophosphamide for induction. Plasma exchange is considered if severe lung hemorrhage or dialysis-dependent renal failure (PEXIVAS trial updated: benefit primarily for lung hemorrhage severity). Azathioprine and MMF are maintenance (not induction) agents.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.