Details of Disease

General Information of Disease (ID: DIS1U0UF)

Disease Name	IgG4 related disease
Synonyms	multifocal idiopathic fibrosclerosis; systemic IgG4-related plasmacytic syndrome; IgG4-related systemic sclerosing disease; IgG4-positive multiorgan lymphoproliferative syndrome; hyper-IgG4 disease; IgG4-syndrome; IgG4-associated disease; systemic IgG4-related sclerosing syndrome; IgG4-related autoimmune disease; multifocal fibrosclerosis; IgG4-RD; IgG4-related systemic disease; IgG4-related sclerosing disease; Immunoglobulin G4-related sclerosing disease
Disease Class	4A43: Sjogren syndrome
Definition	A recently described mass-forming lesion that occurs in the pancreas, submandibular glands, lacrimal glands, lymph nodes, and hepatobiliary tract. It is characterized by the presence of marked tissue sclerosis and infiltration by numerous plasma cells. The plasma cells show immunohistochemical staining for IgG4 and the serum IgG4 levels are often increased.
Disease Hierarchy	DISORMTM: Autoimmune disease DIS1U0UF: IgG4 related disease
ICD Code	ICD-11 ICD-11: 4A43.0 ICD-10 ICD-10: M35, M35.8 Expand ICD-11 '4A43.0 Expand ICD-10 'M35; 'M35.8
Disease Identifiers	MONDO ID MONDO_0017287 MESH ID D000077733 UMLS CUI C3203653 MedGen ID 473761 Orphanet ID 284264

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Obexelimab	DMVIC4J	Phase 3	Antibody	[1]
Xmab 5871	DMLIPY1	Phase 2	NA	[2]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 2 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CCR8	TTE836A	Strong	Biomarker	[3]
SLAMF7	TT7ILZ1	Strong	Biomarker	[4]
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This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
ANXA11	DTGQ2CF	Strong	Biomarker	[5]
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This Disease Is Related to 2 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CCL18	OT7JYSK9	moderate	Biomarker	[3]
MARCO	OT0NW2Q0	Strong	Biomarker	[6]
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References

1	ClinicalTrials.gov (NCT05662241) A Phase 3, Multicenter, Randomized, Double-blind, Placebo-controlled Study to Evaluate the Efficacy and Safety of Obexelimab in Patients With IgG4-Related Disease (INDIGO). U.S.National Institutes of Health.
2	Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
3	Upregulation and pathogenic roles of CCL18-CCR8 axis in IgG4-related disease.Mod Rheumatol. 2020 Jul;30(4):729-737. doi: 10.1080/14397595.2019.1632061. Epub 2019 Aug 8.
4	A CD8- Subset of CD4+SLAMF7+ Cytotoxic T Cells Is Expanded in Patients With IgG4-Related Disease and Decreases Following Glucocorticoid Treatment.Arthritis Rheumatol. 2018 Jul;70(7):1133-1143. doi: 10.1002/art.40469. Epub 2018 May 20.
5	Annexin A11 is targeted by IgG4 and IgG1 autoantibodies in IgG4-related disease.Gut. 2018 Apr;67(4):728-735. doi: 10.1136/gutjnl-2017-314548. Epub 2017 Aug 1.
6	DNA Microarray Analysis of Submandibular Glands in IgG4-Related Disease Indicates a Role for MARCO and Other Innate Immune-Related Proteins.Medicine (Baltimore). 2016 Feb;95(7):e2853. doi: 10.1097/MD.0000000000002853.