Echovirus polymyositis in patients with hypogammaglobulinemia: Failure of high-dose intravenous gammaglobulin therapy and review of the literature

Abstract

A 29-year-old man with X-linked hypogammaglobulinemia was treated with prednisone and methotrexate for polymyositis. Subsequently, it was established that disseminated echovirus 11 infection was causing the polymyositis. Treatment with large doses of intravenous gamma-globulin did not result in improvement. Viral cultures of blood, urine, and cerebrospinal fluid gave positive results throughout treatment and at postmortem examination. Multiple cultures of other tissues, including muscle, also gave positive results at postmortem examination. Severity of infection and treatment with prednisone and methotrexate prior to referral, diagnosis, and gammaglobulin treatment may explain the lack of response. A review of 23 cases of echovirus infection in patients with hypogammaglobulinemia revealed that the infection in these patients may cause meningoencephalitis or a polymyositis-like syndrome or both. Treatment with immunosuppressive agents, the standard therapy for polymyositis, is contraindicated, and intravenous or intraventricular gammaglobulin or both may be helpful.