Myasthenia gravis.
Acetylcholinesterase
/ genetics
Adrenal Cortex Hormones
/ therapeutic use
Agrin
/ genetics
Anti-Inflammatory Agents, Non-Steroidal
/ therapeutic use
Autoantibodies
/ analysis
Biomarkers
/ analysis
Blepharoptosis
/ etiology
Collagen
/ genetics
Cortactin
/ genetics
Electromyography
/ methods
Humans
Kv1.4 Potassium Channel
/ genetics
LDL-Receptor Related Proteins
/ genetics
Muscle Proteins
/ genetics
Myasthenia Gravis
/ diagnosis
Receptor Protein-Tyrosine Kinases
/ genetics
Receptors, Cholinergic
/ genetics
Receptors, Nicotinic
/ genetics
Risk Factors
Ryanodine Receptor Calcium Release Channel
/ genetics
Journal
Nature reviews. Disease primers
ISSN: 2056-676X
Titre abrégé: Nat Rev Dis Primers
Pays: England
ID NLM: 101672103
Informations de publication
Date de publication:
02 05 2019
02 05 2019
Historique:
entrez:
4
5
2019
pubmed:
3
5
2019
medline:
17
9
2019
Statut:
epublish
Résumé
Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thymectomy and immunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.
Identifiants
pubmed: 31048702
doi: 10.1038/s41572-019-0079-y
pii: 10.1038/s41572-019-0079-y
doi:
Substances chimiques
Adrenal Cortex Hormones
0
Agrin
0
Anti-Inflammatory Agents, Non-Steroidal
0
Autoantibodies
0
Biomarkers
0
Cortactin
0
KCNA4 protein, human
0
Kv1.4 Potassium Channel
0
LDL-Receptor Related Proteins
0
LRP4 protein, human
0
Muscle Proteins
0
Receptors, Cholinergic
0
Receptors, Nicotinic
0
Ryanodine Receptor Calcium Release Channel
0
Collagen
9007-34-5
MUSK protein, human
EC 2.7.10.1
Receptor Protein-Tyrosine Kinases
EC 2.7.10.1
Acetylcholinesterase
EC 3.1.1.7
COLQ protein, human
EC 3.1.1.7
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM