PMID- 24156976
OWN - NLM
STAT- MEDLINE
DCOM- 20150330
LR  - 20161018
IS  - 1768-3122 (Electronic)
IS  - 0248-8663 (Linking)
VI  - 35
IP  - 7
DP  - 2014 Jul
TI  - [Myasthenia gravis and autoantibodies: Pathophysiology of the different 
      subtypes].
PG  - 413-20
LID - S0248-8663(13)00674-7 [pii]
LID - 10.1016/j.revmed.2013.09.012 [doi]
AB  - Myasthenia gravis is characterized by muscle weakness and abnormal fatigability. 
      It is an autoimmune disease caused by the presence of antibodies against 
      components of the muscle membrane localized at the neuromuscular junction. In 
      most cases, the autoantibodies are directed against the acetylcholine receptor 
      (AChR). Recently, other targets have been described, such as muscle-specific 
      kinase protein (MuSK) or lipoprotein related protein 4 (LRP4). The origin of the 
      autoimmune response is not known, but thymic abnormalities and defects in immune 
      regulation certainly play a major role in patients with anti-AChR antibodies. 
      Genetic predisposition probably influences the occurrence of the disease. Sex 
      hormones seem to play a role in the early form of the disease. Muscle weakness is 
      fluctuating and worsens with exercise. Myasthenia gravis could be classified 
      according to the location of the affected muscles (ocular versus generalized), 
      the age of onset of symptoms, thymic abnormalities and profile of autoantibodies. 
      These criteria are used to optimize the management and treatment of patients. In 
      this review, we analyze the latest concepts of the pathophysiology of myasthenia 
      gravis according to the different subgroups of the disease, including a 
      description of the role of immunological, genetic and environmental factors. The 
      potential viral hypothesis of this disease is discussed. Finally, we also discuss 
      the biological assays available to validate the diagnosis.
CI  - Copyright (c) 2013 Societe nationale francaise de medecine interne (SNFMI). 
      Published by Elsevier SAS. All rights reserved.
FAU - Berrih-Aknin, S
AU  - Berrih-Aknin S
AD  - Unite mixte de recherche (UMR), CNRS UMR7215/Inserm U974/UPMC UM76/AIM, therapie 
      des maladies du muscle strie, groupe hospitalier Pitie-Salpetriere, 105, 
      boulevard de l'Hopital, 75651 Paris cedex 13, France. Electronic address: 
      sonia.berrih-aknin@upmc.fr.
FAU - Le Panse, R
AU  - Le Panse R
AD  - Unite mixte de recherche (UMR), CNRS UMR7215/Inserm U974/UPMC UM76/AIM, therapie 
      des maladies du muscle strie, groupe hospitalier Pitie-Salpetriere, 105, 
      boulevard de l'Hopital, 75651 Paris cedex 13, France.
LA  - fre
PT  - English Abstract
PT  - Journal Article
PT  - Review
TT  - Myasthenie et auto-anticorps : physiopathologie des differentes entites.
DEP - 20131021
PL  - France
TA  - Rev Med Interne
JT  - La Revue de medecine interne
JID - 8101383
RN  - 0 (Autoantibodies)
RN  - 0 (LDL-Receptor Related Proteins)
RN  - 0 (LRP4 protein, human)
RN  - 0 (Receptors, Cholinergic)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Autoantibodies/*blood
MH  - Humans
MH  - LDL-Receptor Related Proteins/immunology
MH  - Myasthenia Gravis/*physiopathology
MH  - Neuromuscular Junction/immunology
MH  - Receptor Protein-Tyrosine Kinases/immunology
MH  - Receptors, Cholinergic/immunology
MH  - Thymus Gland/physiopathology
OTO - NOTNLM
OT  - Acetylcholine receptor
OT  - Inflammation
OT  - LRP4
OT  - MuSK
OT  - Recepteur de l'acetylcholine
OT  - Thymus
EDAT- 2013/10/26 06:00
MHDA- 2015/03/31 06:00
CRDT- 2013/10/26 06:00
PHST- 2013/09/21 00:00 [received]
PHST- 2013/09/23 00:00 [accepted]
PHST- 2013/10/26 06:00 [entrez]
PHST- 2013/10/26 06:00 [pubmed]
PHST- 2015/03/31 06:00 [medline]
AID - S0248-8663(13)00674-7 [pii]
AID - 10.1016/j.revmed.2013.09.012 [doi]
PST - ppublish
SO  - Rev Med Interne. 2014 Jul;35(7):413-20. doi: 10.1016/j.revmed.2013.09.012. Epub 
      2013 Oct 21.