How is RPGN treated

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How is RPGN treated

What are the treatment options for patients with RPGN?

RPGN needs to be treated aggressively and early in its course to reduce the likelihood of ESKD. Glucocorticoid and immunosuppressive regimens are the mainstay of RPGN treatment.

The benefit of these agents is greatest in patients with ANCA-associated vasculitis. The most common complications of therapy are infections. Hemorrhagic cystitis, a complication of cyclophosphamide therapy, occurs less commonly with use of aggressive intravenous saline infusions to promote the excretion of metabolites toxic to bladder epithelial cells.

In addition, 2-mercaptoethanesulfonate (MESNA), an agent that binds and sequesters the metabolite responsible for injury to the uroepithelia, is often given with cyclophosphamide. Malignancies, especially bladder cancers and leukemias, can occur decades after treatment of GPA with cyclophosphamide. Randomized, clinical trials continue to be performed in order to determine optimal therapy for patients with either ANCA-associated vasculitis or lupus nephritis.

The reader is encouraged to review the most current literature and expert opinion to develop treatment regimens for patients with these diseases. The most current immunosuppressive protocols include several different immunosuppressive agents directed at different molecular targets to induce and maintain a remission.

Therapy with these agents is often prolonged, lasting months to more than 1 year, necessitating the need for expert diagnosis and almost always biopsy confirmation. Of course, all treatment protocols have significant risk for adverse events, and patients require close monitoring and drug dose adjustments to reduce drug toxicity. Consultation with an expert in the treatment of these patients is highly recommended.

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