PMID- 26376969 OWN - NLM STAT- MEDLINE DCOM- 20151222 LR - 20220410 IS - 1474-4465 (Electronic) IS - 1474-4422 (Linking) VI - 14 IP - 10 DP - 2015 Oct TI - Myasthenia gravis: subgroup classification and therapeutic strategies. PG - 1023-36 LID - S1474-4422(15)00145-3 [pii] LID - 10.1016/S1474-4422(15)00145-3 [doi] AB - Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated myasthenia gravis might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. Pyridostigmine is the preferred symptomatic treatment, and for patients who do not adequately respond to symptomatic therapy, corticosteroids, azathioprine, and thymectomy are first-line immunosuppressive treatments. Additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies. Long-term drug treatment is essential for most patients and must be tailored to the particular form of myasthenia gravis. CI - Copyright (c) 2015 Elsevier Ltd. All rights reserved. FAU - Gilhus, Nils Erik AU - Gilhus NE AD - Department of Clinical Medicine, University of Bergen, Bergen, Norway; Department of Neurology, Haukeland University Hospital, Bergen, Norway. Electronic address: nils.gilhus@helse-bergen.no. FAU - Verschuuren, Jan J AU - Verschuuren JJ AD - Department of Neurology, Leiden University Medical Center, Leiden, Netherlands. LA - eng PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - Lancet Neurol JT - The Lancet. Neurology JID - 101139309 SB - IM CIN - Lancet Neurol. 2016 Apr;15(4):355-6. PMID: 26971654 CIN - Lancet Neurol. 2016 Apr;15(4):356-7. PMID: 26971655 CIN - Lancet Neurol. 2016 Apr;15(4):357-8. PMID: 26971656 MH - Humans MH - *Myasthenia Gravis/classification/drug therapy/immunology EDAT- 2015/09/18 06:00 MHDA- 2015/12/23 06:00 CRDT- 2015/09/18 06:00 PHST- 2014/07/30 00:00 [received] PHST- 2015/06/02 00:00 [revised] PHST- 2015/06/19 00:00 [accepted] PHST- 2015/09/18 06:00 [entrez] PHST- 2015/09/18 06:00 [pubmed] PHST- 2015/12/23 06:00 [medline] AID - S1474-4422(15)00145-3 [pii] AID - 10.1016/S1474-4422(15)00145-3 [doi] PST - ppublish SO - Lancet Neurol. 2015 Oct;14(10):1023-36. doi: 10.1016/S1474-4422(15)00145-3.