Details of Disease

General Information of Disease (ID: DISLUF66)

• Disease Name: Rheumatic fever
• Synonyms: inflammatory rheumatism; RHF - rheumatic fever; acute rheumatic fever; ARF
• Disease Class: 1B40: Rheumatic fever
• Definition: A post-bacterial multisystem inflammatory disease occurring as a post-infectious, nonsuppurative sequela of untreated streptococcus pyogenes (Group A streptococcus [GAS]) pharyngitis, and mainly occurs in individuals aged 5 to 15 years. The most common presenting signs are fever, migratory polyarthritis and carditis.
• Disease Hierarchy:
  DIS5JY9R: Post-bacterial disorder
  DIS77ACK: Rheumatic disorder
  DISLUF66: Rheumatic fever
• ICD Code:
  ICD-11: ICD-11: 1B40-1B42
  ICD-10: ICD-10: R50
  Expand ICD-11: MG26
  Expand ICD-10: R50
• Disease Identifiers:
  MONDO ID: MONDO_0017767
  MESH ID: D012213
  UMLS CUI: C0035436
  MedGen ID: 48448
  Orphanet ID: 3099
  SNOMED CT ID: 195528001

Drug-Interaction Atlas (DIA) of This Disease

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

Drug Name	Drug ID	Highest Status	Drug Type	REF
Sulfadiazine	DMTW3R8	Approved	Small molecular drug	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 5 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
GALNS	TTT9YPO	Limited	Biomarker	[2]
GAST	TT4LRVO	Limited	Biomarker	[2]
NPM1	TTHBS98	Disputed	Biomarker	[3]
CBX7	TTBN3HC	moderate	Altered Expression	[4]
RHD	TTLCKI8	Strong	Biomarker	[5]

This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC25A19	DTT82QK	Strong	Biomarker	[6]

This Disease Is Related to 19 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
DMP1	OTBWBWW7	Limited	Altered Expression	[7]
DMTF1	OTDKO9OO	Limited	Altered Expression	[8]
HUWE1	OTFH6BJS	Limited	Biomarker	[9]
IL2RG	OTRZ3OMY	Limited	Biomarker	[10]
MYOM2	OTD2UOXW	Limited	Biomarker	[11]
PAGR1	OTXR5PQ8	Limited	Biomarker	[2]
PIK3C3	OTLUM9L7	Limited	Altered Expression	[12]
PRC1	OTHD0XS0	Limited	Biomarker	[13]
RAB11A	OTC4FW0J	Limited	Biomarker	[14]
RBM45	OTWTHD77	Limited	Genetic Variation	[15]
TBX2	OTTOT7A9	Limited	Altered Expression	[16]
CDK5RAP3	OTC0Q2QS	Strong	Biomarker	[17]
FCN1	OTK6ZHXH	Strong	Altered Expression	[18]
FCN2	OTTHJBKZ	Strong	Genetic Variation	[19]
HARS2	OTC8X3H9	Strong	Biomarker	[20]
HLA-DQA2	OT1DH0N9	Strong	Genetic Variation	[21]
MNT	OTPC4ANL	Strong	Genetic Variation	[22]
RUNX1T1	OT30DED5	Strong	Biomarker	[23]
TBX3	OTM64N7K	Strong	Altered Expression	[24]

References

• [1] Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
• [2] The Limitations of the Rheumatogenic Concept for Group A Streptococcus: Systematic Review and Genetic Analysis.Clin Infect Dis. 2020 Mar 17;70(7):1453-1460. doi: 10.1093/cid/ciz425.
• [3] Nucleophosmin protein expression level, but not threonine 198 phosphorylation, is essential in growth and proliferation.Oncogene. 2009 Sep 10;28(36):3209-20. doi: 10.1038/onc.2009.178. Epub 2009 Jun 29.
• [4] Oncogenic role of the chromobox protein CBX7 in gastric cancer.J Exp Clin Cancer Res. 2010 Aug 19;29(1):114. doi: 10.1186/1756-9966-29-114.
• [5] HLA, blood groups and secretor status in patients with established rheumatic fever and rheumatic heart disease.Tissue Antigens. 1986 Mar;27(3):172-8. doi: 10.1111/j.1399-0039.1986.tb01517.x.
• [6] Tuftsin phosphorylcholine-a novel compound harnessing helminths to fight autoimmunity.Immunol Res. 2018 Dec;66(6):637-641. doi: 10.1007/s12026-018-9051-2.
• [7] Disruption of the ARF transcriptional activator DMP1 facilitates cell immortalization, Ras transformation, and tumorigenesis.Genes Dev. 2000 Jul 15;14(14):1797-809.
• [8] Human DMTF1 antagonizes DMTF1 regulation of the p14(ARF) tumor suppressor and promotes cellular proliferation.Biochim Biophys Acta. 2015 Sep;1849(9):1198-208. doi: 10.1016/j.bbagrm.2015.07.009. Epub 2015 Jul 15.
• [9] ARF-BP1/Mule is a critical mediator of the ARF tumor suppressor.Cell. 2005 Jul 1;121(7):1071-83. doi: 10.1016/j.cell.2005.03.037.
• [10] Unique risk factors for insertional mutagenesis in a mouse model of XSCID gene therapy.Proc Natl Acad Sci U S A. 2006 Aug 1;103(31):11730-5. doi: 10.1073/pnas.0603635103. Epub 2006 Jul 24.
• [11] Systems immunology reveals a linked IgG3-C4 response in patients with acute rheumatic fever.Immunol Cell Biol. 2020 Jan;98(1):12-21. doi: 10.1111/imcb.12298. Epub 2019 Nov 18.
• [12] Nuclear trafficking of EGFR by Vps34 represses Arf expression to promote lung tumor cell survival.Oncogene. 2016 Jul 28;35(30):3986-94. doi: 10.1038/onc.2015.480. Epub 2015 Dec 21.
• [13] Role of the chromobox protein CBX7 in lymphomagenesis.Proc Natl Acad Sci U S A. 2007 Mar 27;104(13):5389-94. doi: 10.1073/pnas.0608721104. Epub 2007 Mar 20.
• [14] FIP4/Arfophilin-2 plays overlapping but distinct roles from FIP3/Arfophilin-1 in neuronal migration during cortical layer formation.Eur J Neurosci. 2018 Nov;48(9):3082-3096. doi: 10.1111/ejn.14199. Epub 2018 Nov 2.
• [15] HLA class II DR and DQ genotypes and haplotypes associated with rheumatic fever among a clinically homogeneous patient population of Latvian children.Arthritis Res Ther. 2007;9(3):R58. doi: 10.1186/ar2216.
• [16] The role of Tbx2 and Tbx3 in mammary development and tumorigenesis.J Mammary Gland Biol Neoplasia. 2004 Apr;9(2):109-18. doi: 10.1023/B:JOMG.0000037156.64331.3f.
• [17] CDK5RAP3 is a novel repressor of p14ARF in hepatocellular carcinoma cells.PLoS One. 2012;7(7):e42210. doi: 10.1371/journal.pone.0042210. Epub 2012 Jul 31.
• [18] Sickening or Healing the Heart? The Association of Ficolin-1 and Rheumatic Fever.Front Immunol. 2018 Dec 18;9:3009. doi: 10.3389/fimmu.2018.03009. eCollection 2018.
• [19] MBL2 and FCN2 gene polymorphisms in a cohort of Italian children with rheumatic fever: A case-control study.Semin Arthritis Rheum. 2017 Oct;47(2):264-268. doi: 10.1016/j.semarthrit.2017.04.006. Epub 2017 Apr 27.
• [20] Histidyl-tRNA synthetase.Biol Chem. 1999 Jun;380(6):623-46. doi: 10.1515/BC.1999.079.
• [21] DRB1, DQA1, DQB1 genes in Turkish children with rheumatic fever.Clin Exp Rheumatol. 2005 Jan-Feb;23(1):117-20.
• [22] The families of patients with acute rheumatic fever or glomerulonephritis in Trinidad.Am J Epidemiol. 1977 Aug;106(2):130-8. doi: 10.1093/oxfordjournals.aje.a112442.
• [23] The t(8;21) fusion protein contacts co-repressors and histone deacetylases to repress the transcription of the p14ARF tumor suppressor.Blood Cells Mol Dis. 2003 Mar-Apr;30(2):177-83. doi: 10.1016/s1079-9796(03)00021-4.
• [24] TBX3 is overexpressed in breast cancer and represses p14 ARF by interacting with histone deacetylases.Cancer Res. 2008 Feb 1;68(3):693-9. doi: 10.1158/0008-5472.CAN-07-5012.