U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

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
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Stübgen JP
J Neurol Sci 2017 Jun 15;377:19-24. Epub 2017 Mar 27 doi: 10.1016/j.jns.2017.03.033. PMID: 28477693
Dalakas MC, Hohlfeld R
Lancet 2003 Sep 20;362(9388):971-82. doi: 10.1016/S0140-6736(03)14368-1. PMID: 14511932

Diagnosis

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
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

Saygin D, Werth V, Paik JJ, Park JK, Needham M, Lundberg IE, Christopher-Stine L
Ann Rheum Dis 2024 Jun 12;83(7):826-829. doi: 10.1136/ard-2023-224652. PMID: 38216318
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
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
Goel KM, King M
Scott Med J 1986 Jan;31(1):15-9. doi: 10.1177/003693308603100104. PMID: 3515551

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
Huber AM
Pediatr Clin North Am 2018 Aug;65(4):739-756. doi: 10.1016/j.pcl.2018.04.006. PMID: 30031496
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317
Ioannou Y, Sultan S, Isenberg DA
Curr Opin Rheumatol 1999 Nov;11(6):468-74. PMID: 10551670

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
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
Ghirardello A, Zampieri S, Iaccarino L, Tarricone E, Bendo R, Gambari PF, Doria A
Autoimmunity 2005 Feb;38(1):79-83. doi: 10.1080/08916930400022681. PMID: 15966133

Recent systematic reviews

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
Brunasso AM, Aberer W, Massone C
ScientificWorldJournal 2014;2014:179180. Epub 2014 Jan 29 doi: 10.1155/2014/179180. PMID: 24600322Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...