title: Clinical Characteristics and Treatment Response of Peripheral Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center.
abstract: OBJECTIVE
  Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA.
  
  
  METHODS
  Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Sixty-one of these patients were followed-up for more than 1 year and received corticosteroid therapy with or without intravenous cyclophosphamide pulse therapy for 6 to 18 months. Medical records of the 61 patients including demographic data, clinical features, laboratory and pathological findings, treatments, and outcomes were reviewed.
  
  
  RESULTS
  Peripheral neuropathy as a manifestation of EGPA was present in 46 (75%) of the 61 patients. The mean follow-up duration of the patients with neuropathy was 6.4 years (range 1.2-18.8 years). The scores on the neurological functional disability scale before and after the combination treatment with corticosteroid and cyclophosphamide were 2.43±0.86 and 0.54±0.95 (mean±SD; p<0.001), respectively. The peripheral neuropathy relapsed in one patient.
  
  
  CONCLUSIONS
  The long-term clinical outcome of peripheral neuropathy in patients with EGPA receiving initial corticosteroid and cyclophosphamide combination therapy was favorable with a very low relapse rate.
authors.affiliation: Department of Neurology, The Catholic University of Korea College of Medicine, Bucheon St. Mary's Hospital, Bucheon, Korea.
authors.forename: Hye Jin
authors.initials: HJ
authors.lastname: Cho
authors.affiliation: Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Sehyo
authors.initials: S
authors.lastname: Yune
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.|Neuroscience Center, Samsung Medical Center, Seoul, Korea.
authors.forename: Jin Myoung
authors.initials: JM
authors.lastname: Seok
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.|Neuroscience Center, Samsung Medical Center, Seoul, Korea.
authors.forename: Eun Bin
authors.initials: EB
authors.lastname: Cho
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.|Neuroscience Center, Samsung Medical Center, Seoul, Korea.
authors.forename: Ju Hong
authors.initials: JH
authors.lastname: Min
authors.affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Yeon Lim
authors.initials: YL
authors.lastname: Seo
authors.affiliation: Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Byung Jae
authors.initials: BJ
authors.lastname: Lee
authors.affiliation: Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.|Neuroscience Center, Samsung Medical Center, Seoul, Korea. bjkim@skku.edu.
authors.forename: Byoung Joon
authors.initials: BJ
authors.lastname: Kim
authors.affiliation: Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. dcchoi@skku.edu.
authors.forename: Dong Chull
authors.initials: DC
authors.lastname: Choi
keywords: cyclophosphamide; eosinophilic granulomatosis with polyangiitis; peripheral neuropathy; prognosis