A 35-year-old woman presents with acute kidney injury (creatinine rising from 0.9 to 3.2 mg/dL over 3 days), haematuria, red cell casts on urine microscopy, proteinuria 3.2 g/24h, and systemic features of sinusitis and hemoptysis. ANCA (PR3-ANCA/c-ANCA) is positive at high titre. Which syndrome is present and what is the acute induction therapy?

• A Granulomatosis with polyangiitis (GPA); treat with IV methylprednisolone + IV cyclophosphamide or rituximab ✓
• B Goodpasture syndrome; treat with plasmapheresis, cyclophosphamide, and steroids
• C Microscopic polyangiitis; treat with prednisolone alone
• D IgA nephropathy; treat with ACE inhibitor

Explanation

Granulomatosis with polyangiitis (formerly Wegener's) is a PR3-ANCA/c-ANCA-associated small vessel vasculitis classically affecting upper respiratory tract (sinusitis, epistaxis), lungs (pulmonary nodules, hemoptysis), and kidneys (pauci-immune crescentic GN). Induction therapy per EULAR guidelines uses high-dose glucocorticoids (IV methylprednisolone then oral prednisolone) with cyclophosphamide or rituximab (RAVE trial showed equivalence; rituximab preferred in relapsing disease). Goodpasture syndrome has anti-GBM antibodies; GPA is ANCA-associated pauci-immune.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.