Successful Treatment of Refractory IgA Vasculitis with Rituximab

Introduction: IgA vasculitis is a systemic small-vessel vasculitis characterized by IgA1-dominant immune complex deposition. Although glucocorticoids and immunosuppressive agents are commonly used for treatment, there is no consensus regarding optimal therapy for refractory or relapsing cases. Herein, we describe a case of severe, treatment-resistant IgA vasculitis with glomerulonephritis successfully managed with rituximab. Case Description: A previously healthy 16-year-old female presented with purpura on the lower extremities. Laboratory investigations revealed nephrotic-range proteinuria (5.0 g/day), microscopic hematuria, and elevated serum creatinine (2.0 mg/dL), indicating significant renal involvement. Skin biopsy confirmed the diagnosis of IgA vasculitis. Renal biopsy demonstrated mesangial hypercellularity, mesangial matrix expansion, and presence of cellular crescents in the glomeruli. Immunofluorescence staining revealed IgA deposition in the mesangial regions. Initial treatment with high-dose intravenous methylprednisolone followed by oral prednisolone in combination with mycophenolic acid, achieved only partial remission, as indicated by reduction in proteinuria to 1.3 g/day. Three months later, the patient experienced a relapse with proteinuria worsening to 5.0 g/day. Mycophenolic acid was discontinued, and rituximab was initiated in a weekly infusion for three consecutive weeks. Following rituximab therapy, the patient exhibited a marked clinical improvement, with proteinuria decreasing to 0.5 g/day and stabilization of renal function. No further relapses of glomerulonephritis have been observed, and corticosteroid tapering has been successfully achieved. Discussion: Rituximab, a monoclonal antibody targeting CD20-positive B lymphocytes, has demonstrated efficacy in modulating pathogenic IgA production and immune complex-mediated inflammation. Accumulating evidence from case reports and small cohort studies suggests that rituximab may be effective in cases of refractory or steroid-resistant IgA vasculitis with renal involvement. While randomized controlled trials are currently lacking, rituximab may serve as a viable second- or third-line therapeutic option. Further prospective studies are warranted to establish its role within the treatment paradigm for IgA vasculitis.