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Clinical characterization and successful treatment of 6 patients with Churg-Strauss syndrome-associated neuropathy
Title: | Clinical characterization and successful treatment of 6 patients with Churg-Strauss syndrome-associated neuropathy |
Authors: | Nakamura, Masakazu Browse this author | Yabe, Ichiro Browse this author →KAKEN DB | Yaguchi, Hiroaki Browse this author | Kishimoto, Riichiro Browse this author | Mito, Yasunori Browse this author | Fujiki, Naoto Browse this author | Houzen, Hideki Browse this author | Tsuji-Akimoto, Sachiko Browse this author | Niino, Masaaki Browse this author | Sasaki, Hidenao Browse this author |
Keywords: | Churg-Strauss syndrome | neuropathy | conduction block | ANCA | vasculitis | intravenous immunoglobulin | corticosteroid |
Issue Date: | Oct-2009 |
Publisher: | Elsevier |
Journal Title: | Clinical Neurology and Neurosurgery |
Volume: | 111 |
Issue: | 8 |
Start Page: | 683 |
End Page: | 687 |
Publisher DOI: | 10.1016/j.clineuro.2009.07.004 |
PMID: | 19647930 |
Abstract: | Objective: To confirm the reported findings and clarify unknown clinical features of Churg-Strauss syndrome (CSS) -associated neuropathy and design appropriate treatment.
Patients and Methods: We assessed the clinical features of 6 patients with CSS-associated neuropathy.
Results: Mononeuritis multiplex was present in 4 cases and polyneuropathy in the remaining cases. Both groups progressed to sensori-motor polyneuropathy in an acute or subacute course. All cases showed bronchial asthma and eosinophilia. Two cases with serum antineutrophil cytoplasmic antibodies to myeloperoxidase (MPO-ANCA) had an acute clinical course and severe symptoms. Nerve conduction studies (NCS) of these 2 cases revealed conduction blocks at the initial stage, although NCS finally indicated sensori-motor axonopathy at the involved extremities. For treatment, high-dose corticosteroid therapy for 4 cases, and cyclophosphamide combined with corticosteroids for one case, were effective. For the remaining case, intravenous immunoglobulin (IVIg) at the chronic phase resulted in a slow improvement of neuropathy in the symptomatic aspect. There was no relapse of neuropathy with low dose corticosteroid treatment for 14-24 months after the initial treatment, except one case. There was also no relapse in the other case that was treated with moderate-dose steroids.
Conclusion: Our study showed that CSS-associated neuropathy is a treatable disorder and that the first choice therapy is high-dose corticosteroid. In cases where corticosteroids are ineffective or for severe cases, immunosuppressive therapy (cyclophosphamide) with steroids should be considered, and IVIg might be a treatment option. |
Type: | article (author version) |
URI: | http://hdl.handle.net/2115/39261 |
Appears in Collections: | 医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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Submitter: 矢部 一郎
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