Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis.

Abstract

BACKGROUND:

Paraneoplastic pemphigus (PNP) involves multiple organs, but little is known about its neurological involvement.

OBJECTIVES:

To investigate the symptoms, prognosis and profiles of associated autoantibodies in myasthenia gravis (MG), and their correlations in patients with PNP.

METHODS:

Fifty-eight patients with PNP 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. Patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), connective tissue disease (CTD) and non-PNP MG (NP-MG), and healthy donors, served as controls. These autoantibodies in PNP were also compared in the presence or absence of dyspnoea or muscle weakness. Cox regression and log-rank tests were used for survival analysis.

RESULTS:

Overall 39% of patients with PNP experienced muscle weakness, and 35% were diagnosed with MG. Moreover, 35% had positive anti-AChR and 28% had anti-AChE antibodies, similarly to NP-MG (33% and 17%, respectively, P > 0·05). However, both were negative in all patients with PV, PF and CTD and healthy donors (P < 0·005). No other antibodies showed significant differences among groups. Anti-AChR and anti-AChE antibody levels were significantly increased in patients with PNP with dyspnoea, while anti-AChR, anti-titin and anti-RyR were significantly increased in patients with PNP with muscle weakness (P < 0·05). Nevertheless, levels and positive rates of these autoantibodies showed no significant differences between PNP with Castleman disease and thymoma. Although anti-AChE levels impacted survival duration (P  =  0·027, odds ratio 3·14), MG complications did not affect the overall survival percentage in PNP.

CONCLUSIONS:

MG is a complication of PNP. Anti-AChR and anti-AChE antibodies are prominent in patients with PNP, especially those with dyspnoea.

• Authors: R Wang, J Li, M Wang, H Hao, X Chen, R Li, X Zhu
• Journal: The British journal of dermatology (Br J Dermatol) Vol. 172 Issue 4 Pg. 968-75 (Apr 2015) ISSN: 1365-2133 [Electronic] England
• PMID: 25388377 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2014 British Association of Dermatologists.

Chemical References

• Autoantibodies
• Connectin
• Receptors, Cholinergic
• Ryanodine Receptor Calcium Release Channel
• MUSK protein, human
• Receptor Protein-Tyrosine Kinases
• Acetylcholinesterase

Topics

• Acetylcholinesterase (immunology)
• Adolescent
• Adult
• Aged
• Autoantibodies (metabolism)
• Connectin (immunology)
• Dyspnea (etiology, immunology, mortality)
• Female
• Humans
• Male
• Middle Aged
• Muscle Weakness (etiology, immunology, mortality)
• Myasthenia Gravis (etiology, immunology, mortality)
• Paraneoplastic Syndromes (complications, immunology, mortality)
• Pemphigus (complications, immunology, mortality)
• Prognosis
• Receptor Protein-Tyrosine Kinases (immunology)
• Receptors, Cholinergic (immunology)
• Ryanodine Receptor Calcium Release Channel (immunology)
• Thymoma (complications, immunology, mortality)
• Thymus Neoplasms (complications, immunology, mortality)
• Young Adult