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Polymyositis

MedGen UID:
39086
Concept ID:
C0085655
Disease or Syndrome
Synonyms: Multiple Myositis; Myositides, Multiple; Myositis, Multiple; Polymyositides
SNOMED CT: Neuromyositis (31384009); PM - Polymyositis (31384009); Polymyositis (31384009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019127
Orphanet: ORPHA732

Definition

A rare idiopathic inflammatory myopathy (IIM) historically characterized by symmetric proximal muscle weakness, elevated muscle enzymes (creatine kinase), myopathic findings on electromyography, and muscle biopsy showing endomyial infiltration composed mainly of macrophages and lymphocytes. The features are non-specific, thus the disease should be distinguished from similar entities with specific clinical, immunological, histological features, notably dermatomyositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, inclusion body myositis, and myositis associated with other connective tissue disorder. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPolymyositis
Follow this link to review classifications for Polymyositis in Orphanet.

Professional guidelines

PubMed

Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Dankó K, Dimachkie MM, Feldman BM, Torre IG, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinska M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Song YW, Vencovsky J, Ytterberg SR, Miller FW, Rider LG; International Myositis Classification Criteria Project consortium, The Euromyositis register and The Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland)
Ann Rheum Dis 2017 Dec;76(12):1955-1964. Epub 2017 Oct 27 doi: 10.1136/annrheumdis-2017-211468. PMID: 29079590Free PMC Article

Recent clinical studies

Etiology

Palmucci S, Di Mari A, Cancemi G, Pennisi I, Mauro LA, Sambataro G, Sambataro D, Galioto F, Fazio G, Ferlito A, Pino F, Basile A, Vancheri C
Medicina (Kaunas) 2022 Nov 30;58(12) doi: 10.3390/medicina58121757. PMID: 36556960Free PMC Article
Silva AMS, Campos ED, Zanoteli E
Arq Neuropsiquiatr 2022 May;80(5 Suppl 1):238-248. doi: 10.1590/0004-282X-ANP-2022-S131. PMID: 35976321Free PMC Article
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Atluri RB
Mo Med 2016 Mar-Apr;113(2):127-30. PMID: 27311223Free PMC Article
Dalakas MC, Hohlfeld R
Lancet 2003 Sep 20;362(9388):971-82. doi: 10.1016/S0140-6736(03)14368-1. PMID: 14511932

Diagnosis

Palmucci S, Di Mari A, Cancemi G, Pennisi I, Mauro LA, Sambataro G, Sambataro D, Galioto F, Fazio G, Ferlito A, Pino F, Basile A, Vancheri C
Medicina (Kaunas) 2022 Nov 30;58(12) doi: 10.3390/medicina58121757. PMID: 36556960Free PMC Article
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317
Dalakas MC, Hohlfeld R
Lancet 2003 Sep 20;362(9388):971-82. doi: 10.1016/S0140-6736(03)14368-1. PMID: 14511932

Therapy

Voet NB, van der Kooi EL, van Engelen BG, Geurts AC
Cochrane Database Syst Rev 2019 Dec 6;12(12):CD003907. doi: 10.1002/14651858.CD003907.pub5. PMID: 31808555Free PMC Article
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation
Ann Rheum Dis 2017 May;76(5):792-801. doi: 10.1136/annrheumdis-2017-211400. PMID: 28385805Free PMC Article
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317
Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, Barohn RJ, Feldman BM, Harris-Love MO, Koontz DC, Fertig N, Kelley SS, Pryber SL, Miller FW, Rockette HE; RIM Study Group
Arthritis Rheum 2013 Feb;65(2):314-24. doi: 10.1002/art.37754. PMID: 23124935Free PMC Article

Prognosis

Xu S, Hu X, Wang J, Xu Q, Han Z, Zhou H, Gao M
Clin Chim Acta 2023 Jul 1;547:117443. Epub 2023 Jun 15 doi: 10.1016/j.cca.2023.117443. PMID: 37329941
Fujisawa T
Medicina (Kaunas) 2021 Apr 3;57(4) doi: 10.3390/medicina57040347. PMID: 33916864Free PMC Article
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317
Melli G, Chaudhry V, Cornblath DR
Medicine (Baltimore) 2005 Nov;84(6):377-385. doi: 10.1097/01.md.0000188565.48918.41. PMID: 16267412
Dalakas MC, Hohlfeld R
Lancet 2003 Sep 20;362(9388):971-82. doi: 10.1016/S0140-6736(03)14368-1. PMID: 14511932

Clinical prediction guides

Xu S, Hu X, Wang J, Xu Q, Han Z, Zhou H, Gao M
Clin Chim Acta 2023 Jul 1;547:117443. Epub 2023 Jun 15 doi: 10.1016/j.cca.2023.117443. PMID: 37329941
Palmucci S, Di Mari A, Cancemi G, Pennisi I, Mauro LA, Sambataro G, Sambataro D, Galioto F, Fazio G, Ferlito A, Pino F, Basile A, Vancheri C
Medicina (Kaunas) 2022 Nov 30;58(12) doi: 10.3390/medicina58121757. PMID: 36556960Free PMC Article
Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation
Ann Rheum Dis 2017 May;76(5):792-801. doi: 10.1136/annrheumdis-2017-211400. PMID: 28385805Free PMC Article
Tieu J, Lundberg IE, Limaye V
Best Pract Res Clin Rheumatol 2016 Feb;30(1):149-68. Epub 2016 May 26 doi: 10.1016/j.berh.2016.04.007. PMID: 27421222
Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, Herbelin L, Barohn R, Isenberg D, Miller FW
Arthritis Care Res (Hoboken) 2011 Nov;63 Suppl 11(0 11):S118-57. doi: 10.1002/acr.20532. PMID: 22588740Free PMC Article

Recent systematic reviews

Zhen C, Hou Y, Zhao B, Ma X, Dai T, Yan C
Front Immunol 2022;13:1051609. Epub 2022 Dec 12 doi: 10.3389/fimmu.2022.1051609. PMID: 36578492Free PMC Article
Cheng CY, Baritussio A, Giordani AS, Iliceto S, Marcolongo R, Caforio ALP
Autoimmun Rev 2022 Apr;21(4):103037. Epub 2022 Jan 5 doi: 10.1016/j.autrev.2022.103037. PMID: 34995763
Turk MA, Hayworth JL, Nevskaya T, Pope JE
J Rheumatol 2021 Jan 1;48(1):25-34. Epub 2020 May 1 doi: 10.3899/jrheum.190768. PMID: 32358156
Voet NB, van der Kooi EL, van Engelen BG, Geurts AC
Cochrane Database Syst Rev 2019 Dec 6;12(12):CD003907. doi: 10.1002/14651858.CD003907.pub5. PMID: 31808555Free PMC Article
Barnes H, Holland AE, Westall GP, Goh NS, Glaspole IN
Cochrane Database Syst Rev 2018 Jan 3;1(1):CD010908. doi: 10.1002/14651858.CD010908.pub2. PMID: 29297205Free PMC Article

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