Rituximab induction without maintenance for granulomatosis with polyangiitis and dialysis - Case report and...

ANCA-associated vasculitis (AAV) may lead to irreversible organ damage, particularly end-stage renal disease (ESRD) requiring dialysis. The chances of renal recovery diminish with prolonged dialysis. We describe a case of a 32-year-old woman admitted for pulmonary infiltrates and acute renal failure. Autoimmune workup revealed an elevated titer of proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA). The diagnosis of granulomatosis with polyangiitis (GPA) was confirmed by renal biopsy. The patient received induction therapy with IV rituximab (375 mg/m² per week for 4 weeks) along with systemic high-dose IV corticosteroids and one pulse of IV cyclophosphamide (1000 mg). Rapid deterioration of her kidney function led to pulmonary edema requiring intensive care (ICU) hospitalization. Dialysis and plasmapheresis were initiated. Significant clinical improvement ensued, but the patient remained dialysis dependent. No immunosuppressive maintenance therapy other than prednisone was given. Chronic dialysis was discontinued successfully after eight months. At a follow-up of 30 months since her hospitalization, the patient is in complete remission without relapses. We suggest that rituximab induction without maintenance therapy for GPA ESRD may be adequate.