A 36-Year-Old Woman With a Worsening Cough and Hearing Loss

Nicole Davey-Ranasinghe, MD; Constantine K. Saadeh, MD


September 05, 2019

The primary treatment for EGPA is systemic glucocorticoids. In addition, an immunosuppressive agent is typically added for advanced refractory disease. Various disease-modifying agents have been used as glucocorticoid-sparing or maintenance therapy in patients with EGPA. Variable responses to methotrexate, azathioprine, mycophenolate mofetil, and leflunomide in the setting of EGPA have been reported; however, evidence is lacking to support these therapies.

Other therapies, including intravenous immunoglobulin, rituximab, interferon alpha, and anti-immunoglobulin E, have all been used in EGPA. Anti–interleukin-5 (IL-5) antibodies are available for the treatment of asthma and have affected the treatment options for patients with EGPA. Mepolizumab, a humanized monoclonal antibody to IL-5, is approved by the US Food and Drug Administration (FDA) for the treatment of EGPA. Mepolizumab has been assessed in multiple case reports and in a multicenter randomized, placebo-controlled trial.[4] Mepolizumab resulted in increased frequency of remission and allowed for tapering of prednisolone or prednisone compared with placebo. The dosage of mepolizumab for EGPA is 300 mg every 4 weeks, which is higher than the traditional asthma dosage.

This patient was treated with prednisone at a dosage of 60 mg daily (approximately 0.8 mg/kg). Her symptoms improved after 4 weeks, and a slow steroid taper was initiated. Monitoring of her vasculitis, both clinically and with repeat imaging and laboratory testing, is scheduled. An anti–IL-5 regimen may be initiated, if it is needed as a steroid-sparing agent. She concomitantly received Pneumocystis jirovecii pneumonia prophylaxis. Ongoing close monitoring for all patients with EGPA in a multidisciplinary fashion is essential.


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