U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Links from PMC

Immune-mediated necrotizing myopathy

MedGen UID:
899492
•Concept ID:
C3267047
•
Disease or Syndrome
Synonyms: Anti-HMG-CoA myopathy; anti-HMG-CoA myopathy; Anti-SRP myopathy; anti-SRP myopathy; Autoimmune necrotizing myopathy; Autoimmune necrotizing myositis; autoimmune necrotizing myositis; Immune mediated necrotizing myopathy; Immune myopathy with myocyte necrosis; immune myopathy with myocyte necrosis; immune-mediated necrotizing myopathy; IMNM; NAM; necrotizing autoimmune myopathy
SNOMED CT: Autoimmune necrotizing myopathy (715863001); Immune mediated necrotizing myopathy (715863001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016098
Orphanet: ORPHA206569

Definition

A rare form of idiopathic inflammatory myopathy with clinical manifestation of acute or subacute proximal muscle weakness and histopathological features of myocyte necrosis and regeneration without significant inflammation. The main presenting feature is subacute severe symmetrical proximal myopathy with a markedly elevated creatine kinase level. The course is often severe but may be self-limiting and recovery may occur within weeks to months of discontinuing the causative agent, if identified. The disease is thought to be related to an immune response possibly triggered by drug therapy, connective tissue diseases, or cancer. The exact mechanism underling the disorder is not known but some autoantibodies appear to be a likely cause. Malignancy may be involved. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVImmune-mediated necrotizing myopathy

Professional guidelines

PubMed

Pathophysiological Mechanisms and Treatment of Dermatomyositis and Immune Mediated Necrotizing Myopathies: A Focused Review.
Kamperman RG, van der Kooi AJ, de Visser M, Aronica E, Raaphorst J
Int J Mol Sci 2022 Apr 13;23(8) doi: 10.3390/ijms23084301. PMID: 35457124Free PMC Article
Current Classification and Management of Inflammatory Myopathies.
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Current diagnosis and treatment of polymyositis and dermatomyositis.
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460

Recent clinical studies

Etiology

Clinical pearls and promising therapies in myositis.
Connolly CM, Paik JJ
Expert Rev Clin Immunol 2023 Jul-Dec;19(7):797-811. Epub 2023 May 18 doi: 10.1080/1744666X.2023.2212162. PMID: 37158055Free PMC Article
Statins Neuromuscular Adverse Effects.
Attardo S, Musumeci O, Velardo D, Toscano A
Int J Mol Sci 2022 Jul 28;23(15) doi: 10.3390/ijms23158364. PMID: 35955495Free PMC Article
Idiopathic inflammatory myopathies.
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Immune-mediated necrotizing myopathy: clinical features and pathogenesis.
Allenbach Y, Benveniste O, Stenzel W, Boyer O
Nat Rev Rheumatol 2020 Dec;16(12):689-701. Epub 2020 Oct 22 doi: 10.1038/s41584-020-00515-9. PMID: 33093664
Anti-HMGCR Myopathy.
Mohassel P, Mammen AL
J Neuromuscul Dis 2018;5(1):11-20. doi: 10.3233/JND-170282. PMID: 29480216Free PMC Article

Diagnosis

Immune-mediated necrotizing myopathy (IMNM): A myopathological challenge.
Merlonghi G, Antonini G, Garibaldi M
Autoimmun Rev 2022 Feb;21(2):102993. Epub 2021 Nov 16 doi: 10.1016/j.autrev.2021.102993. PMID: 34798316
Idiopathic inflammatory myopathies.
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Immune-mediated necrotizing myopathy: clinical features and pathogenesis.
Allenbach Y, Benveniste O, Stenzel W, Boyer O
Nat Rev Rheumatol 2020 Dec;16(12):689-701. Epub 2020 Oct 22 doi: 10.1038/s41584-020-00515-9. PMID: 33093664
Current Classification and Management of Inflammatory Myopathies.
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Current diagnosis and treatment of polymyositis and dermatomyositis.
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460

Therapy

Statins Neuromuscular Adverse Effects.
Attardo S, Musumeci O, Velardo D, Toscano A
Int J Mol Sci 2022 Jul 28;23(15) doi: 10.3390/ijms23158364. PMID: 35955495Free PMC Article
Immune-mediated necrotizing myopathy: clinical features and pathogenesis.
Allenbach Y, Benveniste O, Stenzel W, Boyer O
Nat Rev Rheumatol 2020 Dec;16(12):689-701. Epub 2020 Oct 22 doi: 10.1038/s41584-020-00515-9. PMID: 33093664
Current diagnosis and treatment of polymyositis and dermatomyositis.
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Anti-HMGCR Myopathy.
Mohassel P, Mammen AL
J Neuromuscul Dis 2018;5(1):11-20. doi: 10.3233/JND-170282. PMID: 29480216Free PMC Article
Immune-mediated necrotizing myopathy.
Bergua C, Chiavelli H, Simon JP, Boyer O, Jouen F, Stenzel W, Martinet J
Z Rheumatol 2016 Mar;75(2):151-6. doi: 10.1007/s00393-015-0029-3. PMID: 26783154

Prognosis

Anti-SRP immune-mediated necrotizing myopathy: A critical review of current concepts.
Ma X, Bu BT
Front Immunol 2022;13:1019972. Epub 2022 Oct 13 doi: 10.3389/fimmu.2022.1019972. PMID: 36311711Free PMC Article
Statins Neuromuscular Adverse Effects.
Attardo S, Musumeci O, Velardo D, Toscano A
Int J Mol Sci 2022 Jul 28;23(15) doi: 10.3390/ijms23158364. PMID: 35955495Free PMC Article
Idiopathic inflammatory myopathies.
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Immune-mediated necrotizing myopathy: clinical features and pathogenesis.
Allenbach Y, Benveniste O, Stenzel W, Boyer O
Nat Rev Rheumatol 2020 Dec;16(12):689-701. Epub 2020 Oct 22 doi: 10.1038/s41584-020-00515-9. PMID: 33093664
Current Classification and Management of Inflammatory Myopathies.
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article

Clinical prediction guides

Clinical pearls and promising therapies in myositis.
Connolly CM, Paik JJ
Expert Rev Clin Immunol 2023 Jul-Dec;19(7):797-811. Epub 2023 May 18 doi: 10.1080/1744666X.2023.2212162. PMID: 37158055Free PMC Article
Statins Neuromuscular Adverse Effects.
Attardo S, Musumeci O, Velardo D, Toscano A
Int J Mol Sci 2022 Jul 28;23(15) doi: 10.3390/ijms23158364. PMID: 35955495Free PMC Article
Immune-mediated necrotizing myopathy (IMNM): A myopathological challenge.
Merlonghi G, Antonini G, Garibaldi M
Autoimmun Rev 2022 Feb;21(2):102993. Epub 2021 Nov 16 doi: 10.1016/j.autrev.2021.102993. PMID: 34798316
Anti-HMGCR myopathy: clinical and histopathological features, and prognosis.
Kurashige T
Curr Opin Rheumatol 2021 Nov 1;33(6):554-562. doi: 10.1097/BOR.0000000000000832. PMID: 34456255
Development of a New Classification System for Idiopathic Inflammatory Myopathies Based on Clinical Manifestations and Myositis-Specific Autoantibodies.
Mariampillai K, Granger B, Amelin D, Guiguet M, Hachulla E, Maurier F, Meyer A, Tohmé A, Charuel JL, Musset L, Allenbach Y, Benveniste O
JAMA Neurol 2018 Dec 1;75(12):1528-1537. doi: 10.1001/jamaneurol.2018.2598. PMID: 30208379Free PMC Article

Recent systematic reviews

Inflammatory myopathy following coronavirus disease 2019 vaccination: A systematic review.
Ding Y, Ge Y
Front Public Health 2022;10:1007637. Epub 2022 Oct 21 doi: 10.3389/fpubh.2022.1007637. PMID: 36339243Free PMC Article
Physical therapy in adult inflammatory myopathy patients: a systematic review.
Van Thillo A, Vulsteke JB, Van Assche D, Verschueren P, De Langhe E
Clin Rheumatol 2019 Aug;38(8):2039-2051. Epub 2019 May 21 doi: 10.1007/s10067-019-04571-9. PMID: 31115788
Statin-Associated Autoimmune Myopathy: A Systematic Review of 100 Cases.
Nazir S, Lohani S, Tachamo N, Poudel D, Donato A
J Clin Rheumatol 2017 Apr;23(3):149-154. doi: 10.1097/RHU.0000000000000497. PMID: 28277343

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.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...