title: Marked improvement of Churg-Strauss syndrome neuropathy by intravenous immunoglobulin and cyclophosphamide.
abstract: A 42-year-old Japanese man developed Churg-Strauss syndrome 7 years after being diagnosed with chronic eosinophilic pneumonia. Prominent eosinophilia, subcutaneous nodules, and neuropathy in the left leg were seen. A pathological diagnosis of necrotizing vasculitis was determined by a biopsy of a subcutaneous nodule. The leg pain was severe and there was prominent atrophy of the thigh and calf, but the muscle weakness was mild. Serum anti-myeloperoxidase anti-neutrophil cytoplasmic antibody was positive. Because the initial treatment with an intravenous methylprednisolone pulse at 1 g/day for 3 days was not sufficient, a onetime treatment with intravenous cyclophosphamide at 15 mg/kg and intravenous immunoglobulin therapy (IVIG) at 400 mg/kg/day for 5 days were administered. Peripheral eosinophilia improved and the leg pain significantly improved. IVIG was repeated 1 month later and symptoms gradually improved further. The early diagnosis of Churg-Strauss syndrome and the early initiation of IVIG with cyclophosphamide were thought to be important.
authors.affiliation: Department of Internal Medicine, Shioya Hospital, International University of Health and Welfare Tochigi, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Umeda
authors.affiliation: Department of Internal Medicine, Shioya Hospital, International University of Health and Welfare Tochigi, Japan.
authors.forename: Tateki
authors.initials: T
authors.lastname: Yamane
authors.affiliation: Department of Internal Medicine, Shioya Hospital, International University of Health and Welfare Tochigi, Japan.
authors.forename: Jin
authors.initials: J
authors.lastname: Takeuchi
authors.affiliation: Department of Pathology, Dokkyo Medical University Koshigaya Hospital Saitama, Japan.
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Imai
authors.affiliation: Department of Neurology, Dokkyo Medical University Tochigi, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Suzuki
authors.affiliation: Department of Nephrology, International University of Health and Welfare Hospital Tochigi, Japan.
authors.forename: Wako
authors.initials: W
authors.lastname: Yumura
keywords: ANCA; Churg–Strauss syndrome; eosinophils; immunoglobulin; vasculitis