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Langford: Conventional therapy still has a role in granulomatosis with polyangiitis, microscopic polyangiitis

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CLEVELAND — Conventional therapy still has a role in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis; however, there are many factors that need to be examined for each patient, according to Carol A. Langford, MD, MHS, director for Vasculitis Care and Research in the Department of Rheumatic and Immunologic Diseases at the Cleveland Clinic, said in her presentation at the Primary Vasculitides Pre-symposium of the Biologic Therapies VII Summit.

“As far as today’s therapies, which include conventional agents, [they] do provide valuable options for the treatment of our patients,” Langford said.

Carol A. Langford

Langford said management of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) should take into account the following three facts: patients with GPA and MPA can still have long-term survival; relapses occur in 50% to 70% of patients; and all therapies have toxicity, both in the short- and long-term.

Currently, treatment of severe disease includes 3-month to 6-month induction therapy of cyclophosphamide plus glucocorticoids or rituximab plus glucocorticoids followed by at least 2 years of maintenance with methotrexate, azathioprine, mycophenolate mofetil or rituximab, Langford said. According to Langford, cyclophosphamide should be used as induction therapy for patients with either a creatinine level of at least 4 mg/dL and who require mechanical ventilation, for patients who have severe disease with intolerance to rituximab or who have worsening severe disease despite rituximab. In addition, cyclophosphamide can be an option for patients who are newly diagnosed. In contrast, rituximab should be used for patients who either have relapsing severe disease; who are newly diagnosed; who have leukopenia or thrombocytopenia; who have urinary retention; who have a history of malignancy or who have an infection. For maintenance therapy, she noted rituximab and azathioprine were found to be equivalent options in a study published in the New England Journal of Medicine. Langford cited a long-term follow-up study which showed rituximab had fewer relapses than azathioprine for maintenance therapy during the span of 18 months; however, relapses still occurred in 58% of patients who were on rituximab. Overall, she said the best approach to manage long-term maintenance in severe disease is unclear.

For non-severe disease, Langford said there should be 3-month to 6-month induction therapy of methotrexate plus glucocorticoids followed by at least 2 years of maintenance with just methotrexate.

“Looking forward, I think there are many exciting things that we leave on the horizon, but there are many exciting things that we do have available to offer our patients today,” Langford said. – by Will Offit

Reference:

Langford CA. GPA and MPA: Is there still role for conventional therapy? Presented at: the Primary Vasculitides Pre-symposium of the Biologic Therapies VII Summit; April 4-8, 2017; Cleveland.

Disclosure: Langford reports clinical trial funding support from Bristol-Myers Squibb, Genentech and GlaxoSmithKline.