Marked improvement of Churg–Strauss syndrome neuropathy by intravenous immunoglobulin and cyclophosphamide

Respirology Case Reports

Akira Umeda³  Tateki Yamane³  Jin Takeuchi³  Yasuo Imai¹  Keisuke Suzuki² 

[1] Department of Pathology, Dokkyo Medical University Koshigaya Hospital, Saitama, Japan;Department of Neurology, Dokkyo Medical University, Tochigi, Japan;Department of Internal Medicine, Shioya Hospital, International University of Health and Welfare, Tochigi, Japan

关键词: ANCA;    Churg–Strauss syndrome;    eosinophils;    immunoglobulin;    vasculitis;   

DOI  :  10.1002/rcr2.56

来源: Wiley

【 摘 要 】

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.

【 授权许可】

CC BY-NC-ND   
© 2014 The Authors. Respirology Case Reports published by John Wiley & Sons Ltd on behalf of The Asian Pacific Society of Respirology.

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