Granulomatosis with Polyangiitis Glomerulonephritis

Thank you Dr. Jang for an excellent presentation on granulomatosis with polyangiitis presenting as lower extremity edema from renal involvement.

Teaching Points:

GPA can involve multiple organs including nasal ulcers, cartiglage destruction, tracheal stenosis, alveolar hemorrhage, glomerulonephritis, leukoclastic angiitis
Diagnosis is made by biopsy and positive ANCA
Treatment involves 2 components: induction of remission and maintenance to prevent relapse. Induction is with steroids and rituximab or cyclophosphamide. Maintenance regimens include methoetrexate, azathioprine, rituximab, and tapering of glucocortidoids.

Further Reading:
Ritximab vs Cyclophosphamide for ANCA – Associated Vasculitis (RAVE trial)
Rituximab vs Azathioprine for Maintenance in ANCA-Associated Vasculitis (MAINRITSAN trial)
Azathioprine or Methotrexate Maintenance for ANCA-Associated Vasculitis (WEGENT trial)

Didactic: Morning Report