April 18, 2014

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


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  • Akira Umeda1,*
  • Tateki Yamane1
  • Jin Takeuchi1
  • Yasuo Imai2
  • Keisuke Suzuki3and
  • Wako Yumura4
  • Article first published online: 14 APR 2014DOI: 10.1002/rcr2.56
    © 2014 The Authors. Respirology Case Reportspublished by John Wiley & Sons Ltd on behalf of The Asian Pacific Society of Respirology. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

    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.

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