Clinical Characteristics and Treatment Response ... - Semantic Scholar
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Clinical Characteristics and Treatment Response ... - Semantic Scholar
Oct 4, 2016 - Key Words eosinophilic granulomatosis with polyangiitis, peripheral neuropathy, ... teritis nodosa, microscopic polyangiitis, and EGPA), and it.
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 Hye-Jin Choa Sehyo Yuneb Jin Myoung Seokc,d Eun Bin Choc,d Ju-Hong Minc,d Yeon Lim Seoe Byung-Jae Leeb Byoung Joon Kimc,d* Dong-Chull Choib*
Background and Purpose 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.
Department of Neurology, The Catholic University of Korea College of Medicine, Bucheon St. Mary's Hospital, Bucheon, Korea b Departments of Medicine, cNeurology, and ePathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea d Neuroscience Center, Samsung Medical Center, Seoul, Korea
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