Management of Vasculitic Glomerulonephritis

Abstract

Small-vessel vasculitides (SVV) are a group of complex and chronic systemic autoimmune disorders associated with significant morbidity. Renal involvement is a major determinant of long-term outcome in all SVV. Depending on the disorder, SVV can be associated with frequent, and often life-threatening, relapses and increased burden from long-term exposure to toxic therapies and accumulating, irreversible organ damage. Prompt diagnosis or determination of active disease and quick initiation of appropriate treatment are therefore paramount in the management of SVV. For rapidly progressive glomerulonephritis or other severe, life-threatening manifestations in either anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) or cryoglobulinemic vasculitis (CV), we recommend intravenous pulse methylprednisolone, plasmapheresis, and intravenous monthly cyclophosphamide. Hemodialysis is also used when indicated. Dose adjustments are made based on the patient’s weight, degree of renal impairment, and tolerability. Corticosteroids and plasmapheresis are tapered according to the patient’s response. Although a clearer role for rituximab in the treatment of AAV and CV is emerging, we reserve rituximab for disease relapse or disease refractory to the aforementioned treatments. We also use rituximab in less severe disease and for maintenance of remission in AAV and CV when indicated. Corticosteroids are a mainstay of therapy in IgA nephropathy (IgAN) and vasculitis (IgAV). In rapidly progressive glomerulonephritis due to IgAN or IgAV, we recommend intravenous cyclophosphamide. Again, dose adjustments are made based on the patient’s weight, degree of renal impairment, and tolerability.