General Information of Disease (ID: DIS50C5O)

Disease Name Dermatomyositis
Synonyms Amyopathic dermatomyositis; adult dermatomyositis; dermatopolymyositis; DM; polymyositis with skin involvement; dermatomyositis
Disease Class 4A41: Idiopathic inflammatory myopathy
Definition Dermatomyositis (DM) is a type of idiopathic inflammatory myopathy characterized by evocative skin lesions and symmetrical proximal muscle weakness.
Disease Hierarchy
DIS5DHFP: Polymyositis
DISWD40R: Disease
DIS77ACK: Rheumatic disorder
DISY5SZC: Dermatitis
DIS50C5O: Dermatomyositis
ICD Code
ICD-11
ICD-11: 4A41.0
Disease Identifiers
MONDO ID
MONDO_0016367
MESH ID
D003882
UMLS CUI
C0011633
MedGen ID
8331
Orphanet ID
221
SNOMED CT ID
396230008

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 1 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Benzoic Acid DMKB9FI Approved Small molecular drug [1]
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This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Daxdilimab DM5BV5H Phase 2 Monoclonal antibody [2]
KZR-616 DM6VD4L Phase 2 Small molecule [3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 20 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
BLK TTNDSC3 Limited Genetic Variation [4]
CCL21 TTLZK1U Limited Genetic Variation [5]
CLEC4C TT7YT06 Limited Biomarker [6]
DCPS TTLSW9V Limited Biomarker [7]
DYSF TTA7MXQ Limited Altered Expression [8]
IL31 TT1RJXK Limited Biomarker [9]
TRIM24 TT9Q7AE Limited Altered Expression [10]
CASP6 TTKW4ML Strong Altered Expression [11]
CD83 TTT9MRQ Strong Altered Expression [12]
CPN1 TT8V2KD Strong Genetic Variation [13]
DDX58 TTVB0O3 Strong Biomarker [14]
HLA-B TTGS10J Strong Genetic Variation [15]
IFNB1 TT4TZ8J Strong Biomarker [16]
IL1A TTPM6HI Strong Genetic Variation [17]
IL31RA TT9HPX0 Strong Biomarker [18]
MCHR2 TTS17MG Strong Altered Expression [11]
MYH2 TTBIL13 Strong Biomarker [19]
PMS1 TTX1ISF Strong Biomarker [20]
SRGN TTCHB06 Strong Biomarker [21]
TRIM28 TTQ2BKV Strong Biomarker [22]
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⏷ Show the Full List of 20 DTT(s)
This Disease Is Related to 1 DTP Molecule(s)
Gene Name DTP ID Evidence Level Mode of Inheritance REF
SLC2A10 DT3BI6S Strong Altered Expression [23]
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This Disease Is Related to 1 DME Molecule(s)
Gene Name DME ID Evidence Level Mode of Inheritance REF
AOC2 DE8DP90 Strong Biomarker [24]
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This Disease Is Related to 51 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
CABIN1 OT4G5CIK Limited Biomarker [25]
CACYBP OTJMZD2T Limited Biomarker [26]
FAM167A OT9JF3JQ Limited Genetic Variation [4]
KANK2 OT3SZIWM Limited Biomarker [26]
NT5C1A OTS7UF36 Limited Biomarker [27]
PUF60 OTG90DYF Limited Biomarker [28]
RBM14 OTO9GMBD Limited Biomarker [26]
SLURP1 OT89YD2E Limited Biomarker [7]
TP53INP1 OT2363Z9 Limited Biomarker [26]
TSACC OT3QW6PH Limited Biomarker [26]
MPC1 OT6DYFUO moderate Altered Expression [29]
ACTN3 OT9DZ7JQ Strong Biomarker [30]
ANGPTL2 OTB6JG41 Strong Biomarker [31]
ANKRD55 OT62KZII Strong Altered Expression [32]
ATP5F1E OTMPLAIS Strong Biomarker [33]
ATP5IF1 OTQFD73H Strong Biomarker [33]
BANK1 OTXAAA11 Strong Genetic Variation [34]
C2 OTHMF4YM Strong Biomarker [35]
C9 OT7I5FDX Strong Biomarker [36]
CD207 OTI9RUDN Strong Altered Expression [37]
CKLF OTHLPHA0 Strong Altered Expression [38]
COL4A5 OTHG60RE Strong Altered Expression [23]
DHX9 OT5AAOQI Strong Biomarker [39]
DNASE1L3 OTEUIMC2 Strong Altered Expression [40]
EIF2B5 OTV3R4RB Strong Genetic Variation [41]
GARS1 OT5B6R9Y Strong Biomarker [42]
HERC5 OTZ5PR39 Strong Altered Expression [43]
HLA-DPA1 OT7OG7Y2 Strong Genetic Variation [13]
HLA-DPB1 OTW8JHU2 Strong Genetic Variation [44]
IFI6 OTWOOAM4 Strong Altered Expression [45]
IFIH1 OTZA2AHA Strong Biomarker [46]
IFNA1 OTPMKY0L Strong Altered Expression [47]
IL1RAPL2 OTP7IOJO Strong Altered Expression [48]
KARS1 OT0EU4SV Strong Biomarker [49]
KDELR1 OT8YBS5G Strong Biomarker [19]
MB OTYWYL2D Strong Altered Expression [50]
MORC3 OT81NKVT Strong Biomarker [51]
MX1 OT6X8G5T Strong Altered Expression [52]
NECAP2 OTLH23GP Strong Biomarker [53]
NFAT5 OTKIE59S Strong Altered Expression [54]
PIP OTH719AH Strong Genetic Variation [13]
PLAAT4 OTI66SAJ Strong Altered Expression [55]
PLCL1 OTJL2C79 Strong Genetic Variation [56]
RIT2 OTSNYG0D Strong Biomarker [32]
RMI2 OTA0HP4S Strong Biomarker [57]
RNPC3 OTW5MKC1 Strong Biomarker [58]
ROBO3 OTPVG40S Strong Biomarker [14]
RTRAF OTJ6NVMW Strong Genetic Variation [41]
SFPQ OTLCIAPJ Strong Biomarker [59]
SYNM OTOI8TRJ Strong Biomarker [60]
TRIM21 OTA4UJCF Strong Biomarker [61]
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⏷ Show the Full List of 51 DOT(s)

References

1 Benzoic acid production via cascade biotransformation and coupled fermentation-biotransformation. Biotechnol Bioeng. 2020 Aug;117(8):2340-2350.
2 ClinicalTrials.gov (NCT05669014) A Phase 2, Randomized, Double-blind, Placebo-controlled, Efficacy and Safety Study of Daxdilimab Subcutaneous Injection in Adult Participants With Inadequately Controlled Dermatomyositis or Anti-synthetase Inflammatory Myositis. U.S.National Institutes of Health.
3 ClinicalTrials.gov (NCT04628936) An Open-label Extension to the Phase 2 Randomized, Double-blind, Placebo-controlled, Crossover Multicenter Study to Evaluate the Safety and Efficacy of KZR-616 in the Treatment of Patients With Active Polymyositis or Dermatomyositis. U.S.National Institutes of Health.
4 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.
5 A single-nucleotide polymorphism of CCL21 rs951005 T>C is associated with susceptibility of polymyositis and such patients with interstitial lung disease in a Chinese Han population.Clin Exp Rheumatol. 2015 Sep-Oct;33(5):639-46. Epub 2015 Aug 31.
6 Role of the chemokine receptors CXCR3, CXCR4 and CCR7 in the intramuscular recruitment of plasmacytoid dendritic cells in dermatomyositis.J Neuroimmunol. 2018 Jun 15;319:142-148. doi: 10.1016/j.jneuroim.2018.01.008. Epub 2018 Jan 9.
7 Current understanding and recent advances in myositis-specific and -associated autoantibodies detected in patients with dermatomyositis.Expert Rev Clin Immunol. 2020 Jan;16(1):79-89. doi: 10.1080/1744666X.2019.1699059. Epub 2019 Dec 8.
8 Global analysis of protein expression in muscle tissues of dermatomyositis/polymyosisits patients demonstrated an association between dysferlin and human leucocyte antigen A.Rheumatology (Oxford). 2019 Mar 25:kez085. doi: 10.1093/rheumatology/kez085. Online ahead of print.
9 Role of the Pruritic Cytokine IL-31 in Autoimmune Skin Diseases.Front Immunol. 2019 Jun 21;10:1383. doi: 10.3389/fimmu.2019.01383. eCollection 2019.
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 MHC class I and II expression in juvenile dermatomyositis skeletal muscle.Clin Exp Rheumatol. 2009 May-Jun;27(3):519-26.
12 Plasmacytoid dendritic cells in inflamed muscle of patients with juvenile dermatomyositis.Arthritis Rheum. 2007 May;56(5):1658-68. doi: 10.1002/art.22558.
13 Variation at HLA-DPB1 is associated with dermatomyositis in Chinese population.J Dermatol. 2016 Nov;43(11):1307-1313. doi: 10.1111/1346-8138.13397. Epub 2016 May 6.
14 The RIG-I pathway is involved in peripheral T cell lymphopenia in patients with dermatomyositis.Arthritis Res Ther. 2019 May 29;21(1):131. doi: 10.1186/s13075-019-1905-z.
15 Malignancy as a comorbidity in rheumatic diseases: a retrospective hospital-based study.Clin Rheumatol. 2018 Jan;37(1):81-85. doi: 10.1007/s10067-017-3676-8. Epub 2017 May 16.
16 Measurement of cytokines and chemokines and association with clinical severity of dermatomyositis and clinically amyopathic dermatomyositis.Br J Dermatol. 2018 Dec;179(6):1334-1341. doi: 10.1111/bjd.17079. Epub 2018 Oct 7.
17 Cytokine gene polymorphisms as risk and severity factors for juvenile dermatomyositis.Arthritis Rheum. 2008 Dec;58(12):3941-50. doi: 10.1002/art.24039.
18 Itch in dermatomyositis: the role of increased skin interleukin-31.Br J Dermatol. 2018 Sep;179(3):669-678. doi: 10.1111/bjd.16498. Epub 2018 Jun 21.
19 A three-way interplay of DR4, autoantibodies and synovitis in biopsy-proven idiopathic inflammatory myositis.Rheumatol Int. 2012 Mar;32(3):611-9. doi: 10.1007/s00296-010-1637-5. Epub 2010 Dec 2.
20 Immune responses to DNA mismatch repair enzymes hMSH2 and hPMS1 in patients with pancreatic cancer, dermatomyositis and polymyositis.Int J Cancer. 2005 Oct 10;116(6):925-33. doi: 10.1002/ijc.21118.
21 Identification of specific chondroitin sulfate species in cutaneous autoimmune disease.J Histochem Cytochem. 2011 Aug;59(8):780-90. doi: 10.1369/0022155411411304.
22 Autoantibody to transcriptional intermediary factor-1 as a myositis-specific antibody: clinical correlation with clinically amyopathic dermatomyositis or dermatomyositis with mild myopathy.Br J Dermatol. 2019 Apr;180(4):881-887. doi: 10.1111/bjd.17098. Epub 2018 Oct 10.
23 Private specificities can dominate the humoral response to self-antigens in patients with cryptogenic fibrosing alveolitis.Respir Res. 2001;2(2):119-24. doi: 10.1186/rr46. Epub 2001 Feb 20.
24 Overexpression of semicarbazide-sensitive amine oxidase in human myopathies.Muscle Nerve. 2004 Feb;29(2):261-6. doi: 10.1002/mus.10550.
25 Successful treatment of plasma exchange for rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis: A case report.Medicine (Baltimore). 2018 Apr;97(15):e0436. doi: 10.1097/MD.0000000000010436.
26 Efficacy and safety of oral high-trough level tacrolimus in acute/subacute interstitial pneumonia with dermatomyositis.Int J Rheum Dis. 2019 Feb;22(2):303-313. doi: 10.1111/1756-185X.13414. Epub 2018 Nov 5.
27 Autoantibodies to Cytosolic 5'-Nucleotidase 1A in Primary Sjgren's Syndrome and Systemic Lupus Erythematosus.Front Immunol. 2018 Jun 5;9:1200. doi: 10.3389/fimmu.2018.01200. eCollection 2018.
28 Anti-FIRexon2, a splicing variant form of PUF60, autoantibody is detected in the sera of esophageal squamous cell carcinoma.Cancer Sci. 2019 Jun;110(6):2004-2013. doi: 10.1111/cas.14024. Epub 2019 May 20.
29 Detection of serum MCP-1 and TGF-1 in polymyositis/dermatomyositis patients and its significance.Eur J Med Res. 2019 Feb 14;24(1):12. doi: 10.1186/s40001-019-0368-7.
30 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.
31 The role of angiopoietin-like protein 2 in pathogenesis of dermatomyositis.Biochem Biophys Res Commun. 2012 Feb 17;418(3):494-9. doi: 10.1016/j.bbrc.2012.01.052. Epub 2012 Jan 18.
32 T-cell transcriptomics from peripheral blood highlights differences between polymyositis and dermatomyositis patients.Arthritis Res Ther. 2018 Aug 29;20(1):188. doi: 10.1186/s13075-018-1688-7.
33 Pyruvate kinase M2 and the mitochondrial ATPase Inhibitory Factor 1 provide novel biomarkers of dermatomyositis: a metabolic link to oncogenesis.J Transl Med. 2017 Feb 10;15(1):29. doi: 10.1186/s12967-017-1136-5.
34 Association between the BANK1 rs3733197 polymorphism and polymyositis/dermatomyositis in a Chinese Han population.Clin Rheumatol. 2019 Feb;38(2):431-436. doi: 10.1007/s10067-018-4257-1. Epub 2018 Aug 25.
35 Penicillamine induced polymyositis and dermatomyositis.J Rheumatol. 1987 Oct;14(5):995-1001.
36 Hereditary complement (C9) deficiency associated with dermatomyositis.Br J Dermatol. 2001 May;144(5):1080-3. doi: 10.1046/j.1365-2133.2001.04204.x.
37 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.
38 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.
39 Autoantibodies to RNA helicase A: a new serologic marker of early lupus.Arthritis Rheum. 2007 Feb;56(2):596-604. doi: 10.1002/art.22329.
40 Serum level of DNase1l3 in patients with dermatomyositis/polymyositis, systemic lupus erythematosus and rheumatoid arthritis, and its association with disease activity.Clin Exp Med. 2017 Nov;17(4):459-465. doi: 10.1007/s10238-016-0448-8. Epub 2016 Dec 30.
41 Prevalence of Pruritus in Cutaneous Lupus Erythematosus: Brief Report of a Multicenter, Multinational Cross-Sectional Study.Biomed Res Int. 2018 Jul 25;2018:3491798. doi: 10.1155/2018/3491798. eCollection 2018.
42 Primary structure and functional expression of human Glycyl-tRNA synthetase, an autoantigen in myositis.J Biol Chem. 1994 Nov 18;269(46):28790-7.
43 Interferon-stimulated gene 15 (ISG15) conjugates proteins in dermatomyositis muscle with perifascicular atrophy.Ann Neurol. 2010 Jan;67(1):53-63. doi: 10.1002/ana.21805.
44 Novel susceptibility alleles in HLA region for myositis and myositis specific autoantibodies in Korean patients.Semin Arthritis Rheum. 2019 Oct;49(2):283-287. doi: 10.1016/j.semarthrit.2019.03.005. Epub 2019 Mar 9.
45 Discovery of Key Genes in Dermatomyositis Based on the Gene Expression Omnibus Database.DNA Cell Biol. 2018 Oct 31. doi: 10.1089/dna.2018.4256. Online ahead of print.
46 Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High-Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti-Melanoma Differentiation-Associated Gene 5-Positive Dermatomyositis.Arthritis Rheumatol. 2020 Mar;72(3):488-498. doi: 10.1002/art.41105. Epub 2020 Jan 27.
47 Aberrant activation of the type I interferon system may contribute to the pathogenesis of anti-melanoma differentiation-associated gene 5 dermatomyositis.Br J Dermatol. 2019 May;180(5):1090-1098. doi: 10.1111/bjd.16917. Epub 2018 Sep 26.
48 Immunolocalization of interleukin-1 receptors in the sarcolemma and nuclei of skeletal muscle in patients with idiopathic inflammatory myopathies.Arthritis Rheum. 2007 Feb;56(2):674-87. doi: 10.1002/art.22388.
49 Lysyl-Transfer RNA Synthetase Induces the Maturation of Dendritic Cells through MAPK and NF-B Pathways, Strongly Contributing to Enhanced Th1 Cell Responses.J Immunol. 2018 Nov 1;201(9):2832-2841. doi: 10.4049/jimmunol.1800386. Epub 2018 Oct 1.
50 Serum levels of B-cell activating factor of the TNF family (BAFF) correlate with anti-Jo-1 autoantibodies levels and disease activity in patients with anti-Jo-1positive polymyositis and dermatomyositis.Arthritis Res Ther. 2018 Jul 27;20(1):158. doi: 10.1186/s13075-018-1650-8.
51 Perimysial microarteriopathy in dermatomyositis with anti-nuclear matrix protein-2 antibodies.Eur J Neurol. 2020 Mar;27(3):514-521. doi: 10.1111/ene.14097. Epub 2019 Oct 25.
52 Glomerular expression of myxovirus resistance protein 1 in human mesangial cells: possible activation of innate immunity in the pathogenesis of lupus nephritis.Nephrology (Carlton). 2013 Dec;18(12):833-7. doi: 10.1111/nep.12155.
53 Integrated comparison of the miRNAome and mRNAome in muscles of dermatomyositis and polymyositis reveals common and specific miRNA-mRNAs.Epigenomics. 2019 Jan;11(1):23-33. doi: 10.2217/epi-2018-0064. Epub 2018 Dec 7.
54 Localization and Expression of Nuclear Factor of Activated T-Cells 5 in Myoblasts Exposed to Pro-inflammatory Cytokines or Hyperosmolar Stress and in Biopsies from Myositis Patients.Front Physiol. 2018 Feb 21;9:126. doi: 10.3389/fphys.2018.00126. eCollection 2018.
55 Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis.Neuropathol Appl Neurobiol. 2019 Aug;45(5):513-522. doi: 10.1111/nan.12519. Epub 2018 Nov 22.
56 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.
57 The major dermatomyositis-specific Mi-2 autoantigen is a presumed helicase involved in transcriptional activation.Arthritis Rheum. 1995 Oct;38(10):1389-99. doi: 10.1002/art.1780381006.
58 Facts and controversies in mixed connective tissue disease.Med Clin (Barc). 2018 Jan 12;150(1):26-32. doi: 10.1016/j.medcli.2017.06.066. Epub 2017 Aug 31.
59 Splicing factor proline/glutamine-rich is a novel autoantigen of dermatomyositis and associated with anti-melanoma differentiation-associated gene 5 antibody.J Autoimmun. 2017 Feb;77:116-122. doi: 10.1016/j.jaut.2016.11.006. Epub 2016 Dec 3.
60 Expression of the intermediate filament protein synemin in myofibrillar myopathies and other muscle diseases.Acta Neuropathol. 2003 Jul;106(1):1-7. doi: 10.1007/s00401-003-0695-0. Epub 2003 Apr 1.
61 Cluster Analysis Using Anti-Aminoacyl-tRNA Synthetases and SS-A/Ro52 antibodies in Patients With Polymyositis/Dermatomyositis.J Clin Rheumatol. 2019 Sep;25(6):246-251. doi: 10.1097/RHU.0000000000000836.