Publication Date:
2014-11-16
Description:
Background Paraneoplastic pemphigus (PNP) involves multiple organs, but little is known about its neurological involvement. Objectives To investigate myasthenia gravis (MG) symptoms, prognosis, profiles of associated autoantibodies and their correlations in PNP patients. Methods 58 PNP patients were assessed for myasthenic symptoms and laboratory evidence. Serum autoantibodies against acetylcholine receptor (AChR), acetylcholinesterase (AChE), titin, ryanodine receptor (RyR) and muscle specific kinase (MuSK) were measured by enzyme linked immunosorbent assay. Pemphigus vulgaris (PV), pemphigus foliaceus (PF), connective tissue diseases (CTD), non-PNP MG (NP-MG) and healthy donors served as controls. These autoantibodies in PNP were also compared in presence or absence of dyspnea or muscle weakness. Cox regression and Log-rank test were used for survival analysis. Results 39.2% PNP experienced muscle weakness, and 35.3% were diagnosed as MG. Moreover, 35.1% had positive anti-AChR and 28.1% had anti-AChE, similarly in NP-MG (33.3% and 17.4%, respectively, P 〉0.05). However, both were negative in all PV, PF, CTD and healthy donors ( P 〈0.005). Neither anti-titin, anti-RyR nor anti-MuSK showed significant differences among all groups. Anti-AChR and anti-AChE levels were significantly increased in PNP having dyspnea, while anti-AChR, anti-titin and anti-RyR were significantly increased in PNP having muscle weakness ( P 〈0.05). Nevertheless, levels and positive rates of these autoantibodies showed no significant differences between PNP with Castleman's disease and thymoma. Although anti-AChE levels impacted survival duration ( P =0.027,OR=3.145), MG complication did not affect overall survival percentage of PNP. Conclusions MG is a complication of PNP. Anti-AChR and anti-AChE antibodies are prominent in PNP patients, especially those having dyspnea. This article is protected by copyright. All rights reserved.
Print ISSN:
0007-0963
Electronic ISSN:
1365-2133
Topics:
Medicine
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