Details of Disease

General Information of Disease (ID: DISGB1BZ)

• Disease Name: Idiopathic inflammatory myopathy
• Synonyms: idiopathic myositis
• Disease Class: 4A41: Idiopathic inflammatory myopathy
• Definition: Idiopathic form of inflammatory myopathy.
• Disease Hierarchy:
  DISCIXF0: Myositis disease
  DISGB1BZ: Idiopathic inflammatory myopathy
• ICD Code: ICD-11: ICD-11: 4A41
• Disease Identifiers:
  MONDO ID: MONDO_0600023
  MESH ID: D009220
  UMLS CUI: C0751356
  MedGen ID: 148290
  SNOMED CT ID: 702380008

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Nipocalimab?	DM0DYAX	Phase 3	Monoclonal antibody	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 9 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
HLA-DQA1	TTU2I3J	Limited	Biomarker	[2]
BLK	TTNDSC3	Strong	Genetic Variation	[3]
CA14	TTEYTKG	Strong	Genetic Variation	[4]
F13A1	TTXI2RA	Strong	Biomarker	[5]
F13B	TTAXGIP	Strong	Biomarker	[5]
TGM1	TT7A949	Strong	Biomarker	[5]
TNNI3	TTNLDK6	Strong	Biomarker	[6]
TPO	TT52XDZ	Strong	Biomarker	[7]
IL1RN	TT6GSR3	Definitive	Biomarker	[8]

This Disease Is Related to 28 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
RBM45	OTWTHD77	Limited	Biomarker	[9]
TRIM33	OT0KS4J7	Limited	Altered Expression	[10]
AARS1	OTW8D813	Strong	Biomarker	[11]
ACTN3	OT9DZ7JQ	Strong	Genetic Variation	[12]
CD207	OTI9RUDN	Strong	Altered Expression	[13]
CHD3	OTDBU4F3	Strong	Biomarker	[14]
CHD4	OTBDEHDP	Strong	Biomarker	[14]
CKLF	OTHLPHA0	Strong	Altered Expression	[15]
CYCS	OTBFALJD	Strong	Biomarker	[16]
DEFB103B	OT8RWY64	Strong	Altered Expression	[17]
EXOSC10	OTH8Z0AL	Strong	Biomarker	[18]
EXT1	OTRPALJK	Strong	Biomarker	[19]
FAM167A	OT9JF3JQ	Strong	Genetic Variation	[3]
HARS1	OTHOEOTS	Strong	Biomarker	[20]
HARS2	OTC8X3H9	Strong	Biomarker	[20]
HLA-C	OTV38BUJ	Strong	Altered Expression	[21]
IFIH1	OTZA2AHA	Strong	Biomarker	[22]
MORC3	OT81NKVT	Strong	Biomarker	[23]
NFKBIL1	OTYFTPS0	Strong	Biomarker	[24]
PLCL1	OTJL2C79	Strong	Genetic Variation	[25]
PUF60	OTG90DYF	Strong	Biomarker	[26]
RNPC3	OTW5MKC1	Strong	Biomarker	[27]
RO60	OTLGM5A8	Strong	Altered Expression	[28]
TARS2	OTXQY23P	Strong	Biomarker	[29]
TARS3	OTPMDV66	Strong	Biomarker	[29]
TRIM21	OTA4UJCF	Strong	Altered Expression	[30]
SAE1	OT18HFX5	Definitive	Biomarker	[31]
TARS1	OT0PWST5	Definitive	Biomarker	[32]

References

• [1] ClinicalTrials.gov (NCT05379634) A Phase 2, Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study to Evaluate the Efficacy and Safety of Nipocalimab in Participants With Active Idiopathic Inflammatory Myopathies. U.S.National Institutes of Health.
• [2] HLA-DPB1 associations differ between DRB1*03 positive anti-Jo-1 and anti-PM-Scl antibody positive idiopathic inflammatory myopathy.Rheumatology (Oxford). 2009 Oct;48(10):1213-7. doi: 10.1093/rheumatology/kep248. Epub 2009 Aug 18.
• [3] Single nucleotide polymorphisms in the FAM167A-BLK gene are associated with polymyositis/dermatomyositis in the Han Chinese population.Immunol Res. 2015 Jun;62(2):153-62. doi: 10.1007/s12026-015-8646-0.
• [4] Interferon-gamma and interleukin-4 gene polymorphisms in Caucasian idiopathic inflammatory myopathy patients in UK.Ann Rheum Dis. 2007 Jul;66(7):970-3. doi: 10.1136/ard.2006.068858. Epub 2007 Apr 3.
• [5] Increase in transglutaminase 2 in idiopathic inflammatory myopathies.Eur Neurol. 2004;51(1):10-4. doi: 10.1159/000074911. Epub 2003 Nov 18.
• [6] Diagnostic Approach to Cardiac Involvement in Idiopathic Inflammatory Myopathies.Int Heart J. 2018 Mar 30;59(2):256-262. doi: 10.1536/ihj.17-204. Epub 2018 Mar 20.
• [7] Clinical contribution of myositis-related antibodies detected by immunoblot to idiopathic inflammatory myositis: A one-year retrospective study.Autoimmunity. 2018 Mar;51(2):89-95. doi: 10.1080/08916934.2018.1441830. Epub 2018 Feb 20.
• [8] Polymorphisms in the IL-1 receptor antagonist gene VNTR are possible risk factors for juvenile idiopathic inflammatory myopathies.Clin Exp Immunol. 2000 Jul;121(1):47-52. doi: 10.1046/j.1365-2249.2000.01266.x.
• [9] Human leukocyte antigen in Japanese patients with idiopathic inflammatory myopathy.Mod Rheumatol. 2020 Jul;30(4):696-702. doi: 10.1080/14397595.2019.1637593. Epub 2019 Jul 18.
• [10] Anti-TIF1 antibody and the expression of TIF1 in idiopathic inflammatory myopathies.Int J Rheum Dis. 2019 Feb;22(2):314-320. doi: 10.1111/1756-185X.13424. Epub 2018 Nov 5.
• [11] Comprehensive insight into human aminoacyl-tRNA synthetases as autoantigens in idiopathic inflammatory myopathies.Crit Rev Immunol. 2007;27(6):559-72. doi: 10.1615/critrevimmunol.v27.i6.60.
• [12] The ACTN3 R577X polymorphism is associated with inflammatory myopathies in a Mexican population.Scand J Rheumatol. 2012 Oct;41(5):396-400. doi: 10.3109/03009742.2012.669495. Epub 2012 May 29.
• [13] Fascin-expressing dendritic cells dominate in polymyositis and dermatomyositis.J Rheumatol. 2013 Feb;40(2):186-91. doi: 10.3899/jrheum.120590. Epub 2012 Nov 1.
• [14] Autoantibodies to Mi-2 alpha and Mi-2 beta in patients with idiopathic inflammatory myopathy.Rheumatology (Oxford). 2019 Sep 1;58(9):1655-1661. doi: 10.1093/rheumatology/kez092.
• [15] Chemokine-like factor expression in the idiopathic inflammatory myopathies.Acta Neurol Scand. 2008 Aug;118(2):106-14. doi: 10.1111/j.1600-0404.2007.00990.x. Epub 2008 Feb 19.
• [16] Oral cyclophosphamide in treatment of patients with refractory idiopathic inflammatory myopathies: a retrospective observational study.Clin Rheumatol. 2018 Aug;37(8):2113-2123. doi: 10.1007/s10067-018-4174-3. Epub 2018 Jul 3.
• [17] Human -defensin-3 correlates with muscle fibre degeneration in idiopathic inflammatory myopathies.Innate Immun. 2014 Jan;20(1):49-60. doi: 10.1177/1753425913481820. Epub 2013 Apr 22.
• [18] An update on the immunogenetics of idiopathic inflammatory myopathies: major histocompatibility complex and beyond.Curr Opin Rheumatol. 2009 Nov;21(6):588-93. doi: 10.1097/BOR.0b013e3283315a22.
• [19] Detection of TT virus in patients with idiopathic inflammatory myopathies.Ann N Y Acad Sci. 2005 Jun;1050:304-13. doi: 10.1196/annals.1313.032.
• [20] Proinflammatory Histidyl-Transfer RNA Synthetase-Specific CD4+ T Cells in the Blood and Lungs of Patients With Idiopathic Inflammatory Myopathies.Arthritis Rheumatol. 2020 Jan;72(1):179-191. doi: 10.1002/art.41075. Epub 2019 Nov 26.
• [21] The immunoproteasomes are key to regulate myokines and MHC class I expression in idiopathic inflammatory myopathies.J Autoimmun. 2016 Dec;75:118-129. doi: 10.1016/j.jaut.2016.08.004. Epub 2016 Aug 10.
• [22] Frequency, mutual exclusivity and clinical associations of myositis autoantibodies in a combined European cohort of idiopathic inflammatory myopathy patients.J Autoimmun. 2019 Jul;101:48-55. doi: 10.1016/j.jaut.2019.04.001. Epub 2019 Apr 13.
• [23] Association of anti-nuclear matrix protein 2 antibody with complications in patients with idiopathic inflammatory myopathies: A meta-analysis of 20 cohorts.Clin Immunol. 2019 Jan;198:11-18. doi: 10.1016/j.clim.2018.11.008. Epub 2018 Nov 13.
• [24] Genetic association study of NF-B genes in UK Caucasian adult and juvenile onset idiopathic inflammatory myopathy.Rheumatology (Oxford). 2012 May;51(5):794-9. doi: 10.1093/rheumatology/ker379. Epub 2011 Dec 30.
• [25] Positive association of genetic variations in the phospholipase C-like 1 gene with dermatomyositis in Chinese Han.Immunol Res. 2016 Feb;64(1):204-12. doi: 10.1007/s12026-015-8738-x.
• [26] The prevalence and clinical significance of anti-PUF60 antibodies in patients with idiopathic inflammatory myopathy.Clin Rheumatol. 2018 Jun;37(6):1573-1580. doi: 10.1007/s10067-018-4031-4. Epub 2018 Mar 15.
• [27] Immunoglobulin gene polymorphisms are susceptibility factors in clinical and autoantibody subgroups of the idiopathic inflammatory myopathies.Arthritis Rheum. 2008 Oct;58(10):3239-46. doi: 10.1002/art.23899.
• [28] Anti-Ro52 antibodies frequently co-occur with anti-Jo-1 antibodies in sera from patients with idiopathic inflammatory myopathy.Clin Exp Immunol. 1997 Jul;109(1):32-40. doi: 10.1046/j.1365-2249.1997.4081308.x.
• [29] Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles distinguish European American patients with different myositis autoantibodies.Medicine (Baltimore). 2006 Mar;85(2):111-127. doi: 10.1097/01.md.0000217525.82287.eb.
• [30] Ro52/TRIM21-deficient expression and function in different subsets of peripheral blood mononuclear cells is associated with a proinflammatory cytokine response in patients with idiopathic inflammatory myopathies.Clin Exp Immunol. 2017 Apr;188(1):154-162. doi: 10.1111/cei.12914. Epub 2017 Jan 22.
• [31] Identification of multiple cancer-associated myositis-specific autoantibodies in idiopathic inflammatory myopathies: a large longitudinal cohort study.Arthritis Res Ther. 2017 Nov 25;19(1):259. doi: 10.1186/s13075-017-1469-8.
• [32] Secreted Threonyl-tRNA synthetase stimulates endothelial cell migration and angiogenesis.Sci Rep. 2013;3:1317. doi: 10.1038/srep01317.