PMID- 25388377
OWN - NLM
STAT- MEDLINE
DCOM- 20151228
LR  - 20150401
IS  - 1365-2133 (Electronic)
IS  - 0007-0963 (Linking)
VI  - 172
IP  - 4
DP  - 2015 Apr
TI  - Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic 
      pemphigus and their correlations with symptoms and prognosis.
PG  - 968-75
LID - 10.1111/bjd.13525 [doi]
AB  - 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.
CI  - (c) 2014 British Association of Dermatologists.
FAU - Wang, R
AU  - Wang R
AD  - Department of Dermatology, Peking University First Hospital, 8 Xishiku Street, 
      Xicheng District, Beijing, 100034, China; Beijing Key Laboratory of Molecular 
      Diagnosis of Dermatoses, Beijing, China.
FAU - Li, J
AU  - Li J
FAU - Wang, M
AU  - Wang M
FAU - Hao, H
AU  - Hao H
FAU - Chen, X
AU  - Chen X
FAU - Li, R
AU  - Li R
FAU - Zhu, X
AU  - Zhu X
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20150305
PL  - England
TA  - Br J Dermatol
JT  - The British journal of dermatology
JID - 0004041
RN  - 0 (Autoantibodies)
RN  - 0 (Connectin)
RN  - 0 (Receptors, Cholinergic)
RN  - 0 (Ryanodine Receptor Calcium Release Channel)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
RN  - EC 3.1.1.7 (Acetylcholinesterase)
SB  - IM
CIN - Br J Dermatol. 2015 Apr;172(4):849-50. PMID: 25827731
MH  - Acetylcholinesterase/immunology
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Autoantibodies/*metabolism
MH  - Connectin/immunology
MH  - Dyspnea/etiology/immunology/mortality
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Muscle Weakness/etiology/immunology/mortality
MH  - Myasthenia Gravis/etiology/*immunology/mortality
MH  - Paraneoplastic Syndromes/complications/*immunology/mortality
MH  - Pemphigus/complications/*immunology/mortality
MH  - Prognosis
MH  - Receptor Protein-Tyrosine Kinases/immunology
MH  - Receptors, Cholinergic/immunology
MH  - Ryanodine Receptor Calcium Release Channel/immunology
MH  - Thymoma/complications/immunology/mortality
MH  - Thymus Neoplasms/complications/immunology/mortality
MH  - Young Adult
EDAT- 2014/11/13 06:00
MHDA- 2015/12/29 06:00
CRDT- 2014/11/13 06:00
PHST- 2014/11/05 00:00 [accepted]
PHST- 2014/11/13 06:00 [entrez]
PHST- 2014/11/13 06:00 [pubmed]
PHST- 2015/12/29 06:00 [medline]
AID - 10.1111/bjd.13525 [doi]
PST - ppublish
SO  - Br J Dermatol. 2015 Apr;172(4):968-75. doi: 10.1111/bjd.13525. Epub 2015 Mar 5.