open access

https://hdl.handle.net/2115/39261

Clinical characterization and successful treatment of 6 patients with Churg-Strauss syndrome-associated neuropathy

英語

corticosteroid

2009-10

Elsevier

Clinical Neurology and Neurosurgery

111(8)

683-687

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.

学術雑誌論文

Accepted Manuscript

0303-8467