Igg4-Related Disease disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: IGG007 MIFTS: 50	Igg4-Related Disease Categories: Bone diseases, Endocrine diseases, Genetic diseases, Immune diseases, Rare diseases	Data Licensing For inquiries, contact: [email protected]
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Aliases & Classifications for Igg4-Related Disease

MalaCards integrated aliases for Igg4-Related Disease:

Name: Igg4-Related Disease ¹¹ ¹⁹ ⁵⁸ ⁵⁸ ⁷⁵ ¹⁴

Multifocal Fibrosclerosis ¹⁹ ⁷¹ ³¹

Immunoglobulin G4-Related Sclerosing Disease ¹⁹ ⁵⁸

Igg4-Related Sclerosing Disease ¹⁹ ⁵⁸

Igg4-Related Systemic Disease ¹⁹ ⁵⁸

Igg4-Positive Multiorgan Lymphoproliferative Syndrome ¹⁹

Systemic Igg4-Related Plasmacytic Syndrome ¹⁹

Systemic Igg4-Related Sclerosing Syndrome ¹⁹

Igg4-Related Systemic Sclerosing Disease ¹⁹

Multifocal Idiopathic Fibrosclerosis ¹⁹

Retroperitoneal Fibrosis, Familial ¹⁹

Igg4-Related Autoimmune Disease ¹⁹

Mediastinal Fibrosis, Familial ¹⁹

Igg4-Associated Disease ¹⁹

Hyper-Igg4 Disease ¹⁹

Igg4-Syndrome ¹⁹

Igg4-Rd ⁵⁸

Characteristics:

Prevelance:

Igg4-Related Systemic Disease: 1-9/100000 (Japan, Japan) 1-9/1000000 (Denmark, Denmark) ⁵⁸

Age Of Onset:

Igg4-Related Disease: Adult ⁵⁸

Igg4-Related Systemic Disease: Adolescent,Adult,Childhood,Elderly ⁵⁸

Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases
Anatomical: Immune diseases Bone diseases Endocrine diseases

See all MalaCards categories (disease lists)

ICD10: ³¹

Diseases of the musculoskeletal system and connective tissue

Systemic connective tissue disorders

Other systemic involvement of connective tissue

Multifocal fibrosclerosis

ICD11: ³³

Diseases of the immune system

Nonorgan specific systemic autoimmune disorders

Overlap or undifferentiated nonorgan specific systemic autoimmune disease

IgG4 related disease

Orphanet: ⁵⁸

Rare systemic and rhumatological diseases

External Ids:

Disease Ontology ¹¹ DOID:0080356

ICD10 ³¹ M35.5

UMLS via Orphanet ⁷² C3203653

Orphanet ⁵⁸ ORPHA284264 ORPHA596448

ICD11 ³³ 99883782

UMLS ⁷¹ C0494949

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Summaries for Igg4-Related Disease

GARD: ¹⁹ IgG4-related disease is an immune-mediated condition that can affect multiple organ systems. Common features include IgG4-related autoimmune pancreatitis, swelling of or within an organ system (an inflammatory pseudotumor), salivary gland disease (which can lead to enlargement of the salivary glands), swollen lymph nodes (lymphadenopathy), skin manifestations, and symptoms consistent with allergies or asthma. The cause of IgG4-related disease is unknown.

MalaCards based summary: Igg4-Related Disease, also known as multifocal fibrosclerosis, is related to sialadenitis and polyarteritis nodosa. An important gene associated with Igg4-Related Disease is CCR6 (C-C Motif Chemokine Receptor 6), and among its related pathways/superpathways are Innate Immune System and MIF Mediated Glucocorticoid Regulation. The drugs Betamethasone and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include salivary gland, bone and skin, and related phenotypes are immune system and hematopoietic system

Disease Ontology: ¹¹ An autoimmune disease that is characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis, autoimmune pancreatitis, and sometimes elevated serum IgG4 levels affecting various organs.

Wikipedia: ⁷⁵ IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic... more...

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Related Diseases for Igg4-Related Disease

Diseases related to Igg4-Related Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 1040)

#	Related Disease	Score	Top Affiliating Genes
1	sialadenitis	31.1	TNFSF13B IL13 ICOSLG CD4 CCR6
2	polyarteritis nodosa	31.1	PRTN3 MPO CRP
3	sclerosing cholangitis	31.1	IL10 ICOSLG CRP CD4 CCR6
4	castleman disease	31.1	SDC1 IL10 CRP CD4 CCR6
5	thyroiditis	31.1	IL4 IL10 CRP
6	hypereosinophilic syndrome	30.9	MPO IL4 IL13 IL10
7	mikulicz disease	30.9	TNFSF13B IL4 IL13 IL10 ICOSLG CRP
8	hashimoto thyroiditis	30.9	IL4 ICOSLG CD4 CCR6
9	aortitis	30.8	PRTN3 MPO CRP CD4 CCR6
10	autoimmune pancreatitis	30.8	SDC1 IL10 ICOSLG CRP CD4 CCR6
11	vasculitis	30.8	TNFSF13B PRTN3 MPO IL10 CRP
12	cavernous sinus thrombosis	30.7	CRP CD4
13	interstitial nephritis	30.7	PRTN3 PLA2R1 ICOSLG CRP CD4 CCR6
14	churg-strauss syndrome	30.7	PRTN3 MPO IL10
15	ige responsiveness, atopic	30.7	IL4 IL13 IL10
16	pleural disease	30.6	CRP CD4 CCR6
17	histiocytosis	30.6	IL4 CD1A CCR6
18	purpura	30.5	TNFSF13B IL4 IL10 CRP
19	cellulitis	30.5	CRP CD4 CCR6
20	orbital plasma cell granuloma	30.5	PRTN3 CRP
21	chronic orbital inflammation	30.5	PRTN3 CRP
22	scleritis	30.5	PRTN3 MPO IL4 CRP CCR6
23	sjogren syndrome	30.5	TNFSF13B PRTN3 IL10 ICOSLG CCR6 CA2
24	cholangitis	30.5	PRTN3 MPO IL10 CRP CA2
25	mediastinitis	30.5	MPO CRP CD4
26	thrombocytopenic purpura, autoimmune	30.5	ICOSLG CD4 CCR6
27	rhinitis	30.5	IL4 IL13 IL10 ICOSLG
28	panniculitis	30.5	CRP CD4 CCR6
29	lung disease	30.4	PRTN3 MPO IL13 IL10 CCR6
30	autoimmune disease	30.4	TNFSF13B PRTN3 IL4 IL13 IL10 ICOSLG
31	vitiligo-associated multiple autoimmune disease susceptibility 1	30.4	ICOSLG CD4 CCR6
32	dacryoadenitis	30.4	TNFSF13B IL4 IL13 IL10 ICOSLG CD4
33	esophagitis	30.4	IL4 IL13 IL10
34	marginal zone b-cell lymphoma	30.4	SDC1 ICOSLG CD38 CD19 CCR6
35	posterior scleritis	30.4	PRTN3 MPO CRP
36	microscopic polyangiitis	30.4	PRTN3 MPO
37	burkitt lymphoma	30.4	TNFSF13B IL4 IL10 CXCR5 CD19
38	interstitial lung disease	30.4	IL4 IL13 IL10 ICOSLG CRP CCR6
39	syphilis	30.4	PRTN3 CRP CD4 CCR6
40	cholangitis, primary sclerosing	30.4	PRTN3 MPO IL4 IL10 ICOSLG CRP
41	anterior scleritis	30.3	PRTN3 CRP
42	spondylitis	30.3	IL10 CRP CCR6
43	anterior uveitis	30.3	IL10 ICOSLG CRP CD4 CCR6
44	localized scleroderma	30.3	IL4 IL13 CD4
45	lymphoma, hodgkin, classic	30.3	TNFSF13B IL13 ICOSLG CCR6
46	posterior uveitis	30.3	IL13 ICOSLG CD4 CCR6
47	allergic disease	30.3	IL4 IL13 IL10 ICOSLG CD4 CCR6
48	lymphoma, mucosa-associated lymphoid type	30.3	TNFSF13B SDC1 ICOSLG CXCR5 CD4 CD19
49	lipoid nephrosis	30.3	PLA2R1 IL13 IL10
50	kimura disease	30.3	PLA2R1 IL4 CD1E CD1A

Graphical network of the top 20 diseases related to Igg4-Related Disease:

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Symptoms & Phenotypes for Igg4-Related Disease

MGI Mouse Phenotypes related to Igg4-Related Disease:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	immune system	MP:0005387	9.91	CA2 CCR6 CD19 CD27 CD38 CD4
2	hematopoietic system	MP:0005397	9.5	CCR6 CD19 CD27 CD38 CD4 CXCR5

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Drugs & Therapeutics for Igg4-Related Disease

Drugs for Igg4-Related Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 83)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Betamethasone

Approved, Vet_approved

Phase 4

378-44-9

9782

Prednisone

Approved, Vet_approved

Phase 4

53-03-2

5865

Prednisolone phosphate

Approved, Vet_approved

Phase 4

302-25-0

Prednisolone acetate

Approved, Vet_approved

Phase 4

52-21-1

Prednisolone

Approved, Vet_approved

Phase 4

50-24-8

4894 5755

Belimumab

Approved

Phase 4

356547-88-1

Methylprednisolone hemisuccinate

Approved

Phase 4

2921-57-5

1875

Methylprednisolone

Approved, Vet_approved

Phase 4

83-43-2

4159 6741

Prednisolone hemisuccinate

Experimental

Phase 4

2920-86-7

4897

Betamethasone-17,21-dipropionate

Phase 4

Betamethasone Valerate

Phase 4

2152-44-5

Betamethasone sodium phosphate

Phase 4

Betamethasone dipropionate, betamethasone sodium phosphate drug combination

Phase 4

Betamethasone benzoate

Phase 4

Liver Extracts

Phase 4

glucocorticoids

Phase 4

Immunosuppressive Agents

Phase 4

Immunologic Factors

Phase 4

Antineoplastic Agents, Hormonal

Phase 4

Hormones

Phase 4

Hormone Antagonists

Phase 4

Anti-Inflammatory Agents

Phase 4

Methylprednisolone Acetate

Phase 4

584547

Immunoglobulins

Phase 3

Antibodies, Monoclonal

Phase 3

Antibodies

Phase 3

Mycophenolic acid

Approved, Investigational

Phase 2

24280-93-1

446541

Rituximab

Approved

Phase 1, Phase 2

174722-31-7

Abatacept

Approved

Phase 2

332348-12-6

Zanubrutinib

Approved, Investigational

Phase 2

1691249-45-2

135565884

Acetaminophen

Approved

Phase 2

103-90-2

1983

Famotidine

Approved

Phase 2

76824-35-6

3325 5702160

Histamine

Approved, Investigational

Phase 2

51-45-6

774

Diphenhydramine

Approved, Investigational

Phase 2

147-24-0, 58-73-1

3100

Elotuzumab

Approved

Phase 2

915296-00-3

Promethazine

Approved, Investigational

Phase 2

60-87-7

4927

Anti-Infective Agents

Phase 2

Anti-Bacterial Agents

Phase 2

Antitubercular Agents

Phase 2

Antibiotics, Antitubercular

Phase 2

Antirheumatic Agents

Phase 2

Antineoplastic Agents, Immunological

Phase 1, Phase 2

Immune Checkpoint Inhibitors

Phase 2

Protein Kinase Inhibitors

Phase 2

Immunoglobulins, Intravenous

Phase 2

Anti-Ulcer Agents

Phase 2

Neurotransmitter Agents

Phase 2

Antipyretics

Phase 2

Neuroprotective Agents

Phase 2

Immunoglobulin G

Phase 2

Interventional clinical trials:

(show all 30)

#	Name	Status	NCT ID	Phase	Drugs
1	To Evaluate the Clinical Efficacy of Iguratimod in the Treatment of IgG4 Related Disease (IgG4-RD) With Mild Symptom	Unknown status	NCT03368274	Phase 4	T 614
2	A Study of Safety and Efficacy of Leflunomide for Maintenance of Remission in IgG4 Related Disease	Completed	NCT02703194	Phase 4	Prednisone;Leflunomide
3	Belimumab Treatment for IgG4-related Disease, a Prospective, Open-label Clinical Trial	Recruiting	NCT04660565	Phase 4	Prednisone and Belimumab;Prednisone
4	A Randomized Trial of Glucocorticoids in Patients With IgG4-Related Disease	Unknown status	NCT01758393	Phase 2, Phase 3	Prednisone
5	A Phase 3, Randomized, Double-blind, Multicenter, Placebo Controlled Study of Inebilizumab Efficacy and Safety in IgG4-Related Disease	Recruiting	NCT04540497	Phase 3	Inebilizumab
6	A Randomized Trial of Treatment in Patients With IgG4-Related Disease	Unknown status	NCT02458196	Phase 2	Prednisone;Prednisone and Mycophenolate mofetil
7	A Prospective, Open-label, Single Center Abatacept in IgG4-Related Disease 10-patient Proof-of-concept Study	Completed	NCT03669861	Phase 2	Abatacept
8	An Open-label, Single-arm, Pilot Study to Evaluate the Effect of XmAb®5871 on Disease Activity in Patients With IgG4-Related Disease	Completed	NCT02725476	Phase 2
9	Rituximab (RTX) for IgG4-related Disease (IgG4-RD): a Prospective,Open-label Trial	Completed	NCT01584388	Phase 1, Phase 2	Rituximab
10	A Phase II, Single-Site, Open-Label Study of Zanubrutinib in Patients With IgG4-Related Disease	Recruiting	NCT04602598	Phase 2	Zanubrutinib 80 MG
11	Elotuzumab in Immunoglobulin G4-Related Disease (IgG4-RD)	Recruiting	NCT04918147	Phase 2	elotuzumab;placebo for elotuzumab;methylprednisolone;diphenhydramine;acetaminophen;famotidine;prednisone
12	An Open Label, Two-Arm Study to Evaluate the Effect of Rilzabrutinib (PRN1008/SAR444671) on Safety and Disease Activity in Patients With IgG4-Related Disease	Recruiting	NCT04520451	Phase 2	rilzabrutinib;Glucocorticoids
13	Treatment of IgG4-Related Disease With Revlimid and Rituximab: The TIGR2 Trial	Completed	NCT02705638	Phase 1	Rituximab;Lenalidomide
14	A Laboratory Scan of Patients With Aortic Aneurysms to IgG4 Levels in the Blood	Unknown status	NCT03267875
15	Plasmablast Detection From IgG4-Related Disease Patients	Unknown status	NCT03466970
16	Evaluation of 18F-FDG PET/CT in Diagnosis and Response Assessment of Patients With IgG4-Related Disease	Unknown status	NCT01665196	Early Phase 1	18F-FDG
17	Detection of Circulating Plasmablast in Diagnosis and Relapse Prediction of IgG4 Related Diseases	Unknown status	NCT04539197
18	Leflunomide Treatment for IgG4-RD	Unknown status	NCT03715699		Prednisone;Prednisone and Leflunomide
19	Characterization of Tissular T Follicular Helper Cells in IgG4-RD	Unknown status	NCT02872441
20	Infraorbital Nerve Involvement on Magnetic Resonance Imaging in European Patients With IgG4-related Ophthalmic Disease	Completed	NCT03690908
21	Bile Usefulness for Detecting IgG4-related Sclerosing Cholangitis	Completed	NCT02616705
22	Follicular Helper T Cells: Biological Marker and Involvement in the Physiopathology of the IgG4-related Disease	Completed	NCT02899039
23	A Survey of Patients With Elevated Serum IGG4 With Unknown IGG4-related Disease to Whom Biopsy and Performance of Subsequent Specific Staining for Clinical, Laboratory and Pathologic Correlation.	Completed	NCT02229448
24	Characterizing IgG4-related Disease With 68Ga-FAPI PET/CT	Recruiting	NCT04125511	Early Phase 1	68Ga-FAPI
25	Clinical Study on the Efficacy and Safety of Tofatib and Cyclophosphamide in the Treatment of Active IgG4 Related Diseases	Recruiting	NCT05625581		tofacitinib
26	National Registry of IgG4-RD in China	Recruiting	NCT03023371
27	Cohort Study of Patients With IgG4-Related Disease	Recruiting	NCT01670695
28	Rheumatologic Biobank for Clinical and Basic Research: Isolation, Extraction and Storage of Protein, DNA and RNA Extracted From Tissues of Patients With Rheumatoid Diseases	Recruiting	NCT03473912
29	Evaluation and Prediction of Relapse Risk After Glucocorticoid and Immunosuppressant Withdrawal in Patients With Stable IgG4 Related Disease: An Open-labeled Multi-centric Randomized Controlled Study From China	Recruiting	NCT04124861		Drug free, IS monotherapy and GC combined with IS
30	Impact of COVID-19 on the Clinical Outcomes and Management of IgG4 Related Disease Patients With Pancreatobiliary Involvement: a Multicenter Retrospective Study	Active, not recruiting	NCT04817553

Search NIH Clinical Center for Igg4-Related Disease

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Genetic Tests for Igg4-Related Disease

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Anatomical Context for Igg4-Related Disease

Organs/tissues related to Igg4-Related Disease:

MalaCards : Salivary Gland, Bone, Skin, T Cells, Lung, Liver, Kidney

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Publications for Igg4-Related Disease

Articles related to Igg4-Related Disease:

(show top 50) (show all 3317)

#	Title	Authors	PMID	Year
1	Elevated Pleural Adenosine Deaminase Levels in IgG4-related Disease With Pleural Effusion: A Case Series. ⁶²	Chae S...Oh EJ	36045066	2023
2	Pulmonary IgG4-related disease with favourable response to rituximab: A case report. ⁶²	Muller R...Schleinitz N	36330374	2022
3	Paravertebral involvement in IgG4-related disease. ⁶²	Akao S...Komori K	36469346	2022
4	Pulmonary and coronary arterial abnormalities in patients with IgG4-related disease. ⁶²	Cochran RL...Little BP	36299866	2022
5	IgG4-related Disease Mimicking a Paratesticular Tumor and Pelvic Lymph Node Metastasis. ⁶²	Sugawara E...Katsumata K	35705247	2022
6	High-fat diet aggravates experimental autoimmune pancreatitis through the activation of type I interferon signaling pathways. ⁶²	Sekai I...Watanabe T	36403482	2022
7	Otological IgG4-Related Disease With Inner Ear Involvement: A Case Report and Review of Literature. ⁶²	Ren Q...Ding X	33325728	2022
8	Central Diabetes Insipidus Due to IgG4-related Hypophysitis That Required over One Year to Reach the Final Diagnosis Due to Symptoms Being Masked by Sialadenitis. ⁶²	Iwamoto Y...Kaneto H	35569983	2022
9	18 F-FDG and 68 Ga-FAPI-04 PET/CT Imaging of a Case With Immunoglobulin G4-Related Disease Presenting as Hepatic Masses. ⁶²	Zhang Z...Zuo C	36083160	2022
10	The landscape of T and B lymphocytes interaction and synergistic effects of Th1 and Th2 type response in the involved tissue of IgG4-RD revealed by single cell transcriptome analysis. ⁶²	Cai S...Dong L	36401985	2022
11	IgG4-related sialadenitis - a rare bilateral submandibular gland enlargement: case report. ⁶²	Mahmoud R...Peleg O	35028774	2022
12	Dupilumab as an adjunct treatment for a patient with steroid-dependent immunoglobulin G4-related disease complicated by asthma: a case report. ⁶²	Otani T...Hattori N	34937495	2022
13	Immunoglobulin G4-related disease with Marked Eosinophilia: A Case Report and Literature Review. ⁶²	Origuchi T...Kawakami A	36351576	2022
14	Progressive dysphagia caused by IgG4-related sclerosing disease involving the esophagogastric junction. ⁶²	Wang YK...Wu IC	35917876	2022
15	IgG4 paratesticular fibrous pseudotumor: Case presentation and literature review. ⁶²	Zeitouni L...Hammadeh MY	36164384	2022
16	Current research topics in FAPI theranostics: a bibliometric analysis. ⁶²	van den Hoven AF...Lavalaye J	36437424	2022
17	Ultrasonic Wave Pericardiectomy for IgG4-Related Constrictive Pericarditis. ⁶²	Matsumoto Y...Kasashima F	35085526	2022
18	Marked Enlargement of Bilateral Infraorbital Nerve in a Known Case of IgG4-related Disease. ⁶²	Rajabi MT...Mohammadi A	35657676	2022
19	Dr. Sugawara et al reply. ⁶²	Sugawara E...Katsumata K	36319010	2022
20	Is all that fibrosis idiopathic? Usual interstitial pneumonia in IgG4 related disease. ⁶²	Khoury J...Adir Y	36403359	2022
21	Spectrum of Large and Medium Vessel Vasculitis in Adults: Primary Vasculitides, Arthritides, Connective Tissue, and Fibroinflammatory Diseases. ⁶²	Seitz L...Lotscher F	36166150	2022
22	Otologic manifestations of IgG4-related disease. ⁶²	Baldizan Velasco L...Angulo CM	36427792	2022
23	Orbital inflammatory pseudotumor revealing multi-organ immunoglobulin G4-related disease. ⁶²	Ben Salah R...Bahloul Z	36445834	2022
24	Multimodality Imaging Evaluation of Coronary IgG4-Related Disease: A "Tumor-Like" Cardiac Lesion. ⁶²	Lanzafame LRM...D'Angelo T	36428873	2022
25	Bilateral lacrimal gland disease: clinical features and outcomes. ⁶²	Huang S...Selva D	34725471	2022
26	The disturbance of the distribution of T helper cell subsets in the mantle area surrounding germinal centers in immunoglobulin G4-related sclerosing sialadenitis. ⁶²	Kasashima S...Harada K	35902401	2022
27	Xanthomatous cutaneous Rosai-Dorfman disease with overlapping features of IgG4-related disease. ⁶²	Gitin A...Motaparthi K	36262360	2022
28	The role of serological biomarkers in the diagnosis and management of autoimmune pancreatitis. ⁶²	de Pretis N...Frulloni L	36125384	2022
29	Utility of renal biopsy in differentiating idiopathic multicentric Castleman disease from IgG4-related disease. ⁶²	Kawanishi M...Ito T	36414812	2022
30	Severe triple vessel disease secondary to IgG4-related coronary periarteritis. ⁶²	Goudot G...Gerhard Herman MD	36378869	2022
31	IgG4-Related Disease with Testicular Involvement: Association or Coincidence? ⁶²	Wang G...Wen Z	36318998	2022
32	Perspectives on current and emerging therapies for IgG4-related disease. ⁶²	Tanaka Y...Stone JH	36408992	2022
33	Cutaneous IgG4-related disease. ⁶²	Mishlab S...Hersh EA	36379910	2022
34	A rare case report on hypertrophic pachymeningitis: Serum IgG4-related disease. ⁶²	Rayamajhi S...Kharel G	36188084	2022
35	Bilateral dacryoadenitis associated with IgG4-related disease presenting as orbital pseudo-inflammatory tumor. ⁶²	Ogul H...Nisa Unlu E	35933087	2022
36	Thoracic manifestations of IgG4-related disease. ⁶²	Muller R...Schleinitz N	36437514	2022
37	IgG4-Related Disease of the Pharynx with Spontaneous Regression. ⁶²	Lee SH...Yusof Z	35817139	2022
38	IgG4-related disease presenting as a solitary epidural pseudotumor. ⁶²	Cao C...Shao Q	36052648	2022
39	Isolated IgG4-related disease of the left maxillary antrum. ⁶²	Ramani P...Krishnan RP	36328041	2022
40	IgG4-Related Disease of the Central Nervous System; A Case Series. ⁶²	Abbas G...Durcan L	36366774	2022
41	The Positive Predictive Value of a Very High Serum IgG4 Concentration for the Diagnosis of IgG4-Related Disease. ⁶²	Baker MC...Wallace ZS	36319016	2022
42	Roles of IgG4 and IgG4/IgG ratio to IgG4-related disease in patients with elevated serum IgG4 level. ⁶²	Wang H...Li L	36305979	2022
43	Clues to mortality trends and their related factors in IgG4-related disease: A Japanese single-center retrospective study. ⁶²	Kawahara H...Kawano M	36300954	2022
44	Igg4 Related Disease of Skull Base-A Diagnostic Challenge. ⁶²	Ramanathan K...Shivakumar S	36452566	2022
45	IgG4-positive plasma cells in nonspecific sialadenitis and sialolithiasis. ⁶²	Peuraharju E...Atula T	35606412	2022
46	Isolated IgG4-associated autoimmune hepatitis or the first manifestation of IgG4-related disease? ⁶²	Cosentino C...Kemp W	36273965	2022
47	IgG4-Related Disease: sight-threatening orbital disease, spectacular improvement after Rituximab therapy. ⁶²	Pelewicz-Sowa M...Miskiewicz P	36269307	2022
48	Reduced COVID-19 vaccine response in patient treated with Dupilumab for IgG4 related disease. ⁶²	Abadeh A...Lee JK	36313899	2022
49	Miliary Pulmonary Cryptococcosis in IgG4-related Disease. ⁶²	Ohkubo N	36288984	2022
50	Role of apoptosis inhibitor of macrophages in patients with IgG4‑related disease/autoimmune pancreatitis and the clinical characteristics associated with this condition. ⁶²	Tanoue S...Ido A	36158321	2022

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Genes for Igg4-Related Disease

Genes related to Igg4-Related Disease (0 elite genes):

(show all 21)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence
1	CCR6	C-C Motif Chemokine Receptor 6	Protein Coding	9.97	DISEASES inferred ¹⁴ ¹⁴
2	ICOSLG	Inducible T Cell Costimulator Ligand	Protein Coding	7.82	DISEASES inferred ¹⁴ ¹⁴
3	CRP	C-Reactive Protein	Protein Coding	6.73	DISEASES inferred ¹⁴
4	SDC1	Syndecan 1	Protein Coding	6.62	DISEASES inferred ¹⁴
5	PLA2R1	Phospholipase A2 Receptor 1	Protein Coding	6.55	DISEASES inferred ¹⁴
6	PRTN3	Proteinase 3	Protein Coding	6.51	DISEASES inferred ¹⁴
7	CD4	CD4 Molecule	Protein Coding	6.46	DISEASES inferred ¹⁴
8	SLAMF7	SLAM Family Member 7	Protein Coding	6.16	DISEASES inferred ¹⁴
9	CXCR5	C-X-C Motif Chemokine Receptor 5	Protein Coding	6.15	DISEASES inferred ¹⁴
10	IL4	Interleukin 4	Protein Coding	6.15	DISEASES inferred ¹⁴
11	IL13	Interleukin 13	Protein Coding	6.12	DISEASES inferred ¹⁴
12	CD38	CD38 Molecule	Protein Coding	6.1	DISEASES inferred ¹⁴
13	TNFSF13B	TNF Superfamily Member 13b	Protein Coding	6.06	DISEASES inferred ¹⁴
14	IL10	Interleukin 10	Protein Coding	6.04	DISEASES inferred ¹⁴
15	CD27	CD27 Molecule	Protein Coding	6	DISEASES inferred ¹⁴
16	CD19	CD19 Molecule	Protein Coding	5.99	DISEASES inferred ¹⁴
17	CD1E	CD1e Molecule	Protein Coding	5.92	DISEASES inferred ¹⁴
18	MPO	Myeloperoxidase	Protein Coding	5.91	DISEASES inferred ¹⁴
19	CD1A	CD1a Molecule	Protein Coding	5.91	DISEASES inferred ¹⁴
20	CA2	Carbonic Anhydrase 2	Protein Coding	5.88	DISEASES inferred ¹⁴
21	IL5	Interleukin 5	Protein Coding	5.88	DISEASES inferred ¹⁴

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Variations for Igg4-Related Disease

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Expression for Igg4-Related Disease

Search GEO for disease gene expression data for Igg4-Related Disease.

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Pathways for Igg4-Related Disease

Pathways related to Igg4-Related Disease according to GeneCards Suite gene sharing:

(show all 20)

#	Super pathways	Score	Top Affiliating Genes
1	Innate Immune System ⁶⁶ Show member pathways Immune System ⁶⁶ Neutrophil degranulation ⁶⁶	13.69	CCR6 CD19 CD1A CD27 CD4 CRP
2	MIF Mediated Glucocorticoid Regulation ⁶⁴ Show member pathways all-trans-Retinoic Acid Signaling in Brain ⁶⁴ Endothelin-1 Signaling Pathway ⁶⁴ Glucocorticoid Receptor Signaling ⁶⁴ IL-6 Pathway ⁶⁴ MIF Regulation of Innate Immune Cells ⁶⁴ NFAT Signaling and Lymphocyte Interactions ⁶⁴ Overview of proinflammatory and profibrotic mediators ⁷⁴ PEDF Induced Signaling ⁶⁴ PGC1Alpha Pathway ⁶⁴ RAR-Gamma-RXR-Alpha Degradation ⁶⁴ STAT3 Pathway ⁶⁴	13.49	TNFSF13B IL4 IL13 IL10 CXCR5 CD4
3	Akt Signaling ⁶⁴ Show member pathways p38 Signaling ⁶⁴ Tec Kinases Signaling ⁶⁴	13.3	TNFSF13B IL4 IL13 IL10 CXCR5 CD4
4	Cytokine Signaling in Immune system ⁶⁶ Show member pathways Signaling by Interleukins ⁶⁶	13.19	TNFSF13B SDC1 PRTN3 IL4 IL13 IL10
5	PAK Pathway ⁶⁴ Show member pathways Antioxidant Action of Vitamin-C ⁶⁴ Epithelial Adherens Junctions ⁶⁴	13.15	TNFSF13B IL4 IL13 IL10 CXCR5 CD4
6	NF-kappaB Signaling ³	12.38	TNFSF13B MPO IL4 IL10 CXCR5 CD38
7	Th17 Differentiation ⁶⁵ Show member pathways IL-21 Signaling and its Primary Biological Effects in Different Immune Cell Types ⁶⁵	11.73	IL4 CXCR5 CD4
8	Allograft rejection ⁷⁴	11.73	CXCR5 IL10 IL13 IL4
9	C-MYB transcription factor network ⁶¹	11.65	PRTN3 MPO CD4
10	Dendritic Cells Developmental Lineage Pathway ⁶⁵	11.64	IL10 CD4 CD38 CD1A CD19
11	Hematopoietic Stem Cells and Lineage-specific Markers ⁶⁵	11.56	SDC1 MPO IL4 IL10 CD4 CD38
12	Innate Lymphoid Cells Differentiation ⁶⁵	11.54	IL4 IL13 IL10 CXCR5 CD4 CCR6
13	Inflammatory bowel disease signaling ⁷⁴ Show member pathways Ulcerative colitis signaling ⁷⁴	11.5	IL4 IL13 IL10
14	Th2 Differentiation ⁶⁵	11.4	IL4 IL13 IL10 CD4
15	Cytokines and inflammatory response ⁷⁴	11.17	IL4 IL13 IL10 CD4
16	Immune infiltration in pancreatic cancer ⁷⁴	11.14	IL10 IL13 IL4
17	OX40 Pathway ⁶⁴	11.12	IL4 IL13 IL10
18	Other interleukin signaling ⁶⁶	11.05	SDC1 PRTN3 CD4
19	Extrafollicular B cell activation by SARS-CoV-2 ⁷⁴	11.02	SLAMF7 IL4 IL10 ICOSLG CXCR5 CRP
20	IL-9 Signaling and its Primary Biological Effects in Different Immune Cell Types ⁶⁵	10.66	IL4 IL13

Sources

GO Terms for Igg4-Related Disease

Cellular components related to Igg4-Related Disease according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	membrane	GO:0016020	10.32	TNFSF13B SLAMF7 SDC1 PLA2R1 ICOSLG CXCR5
2	membrane	GO:0016021	10.32	TNFSF13B SLAMF7 SDC1 PLA2R1 ICOSLG CXCR5
3	plasma membrane	GO:0005887	10.02	CCR6 CD19 CD1A CD1E CD27 CD4
4	plasma membrane	GO:0005886	10.02	CCR6 CD19 CD1A CD1E CD27 CD4
5	external side of plasma membrane	GO:0009897	9.66	SLAMF7 SDC1 IL13 ICOSLG CXCR5 CD4

Biological processes related to Igg4-Related Disease according to GeneCards Suite gene sharing:

(show all 12)

#	Name	GO ID	Score	Top Affiliating Genes
1	adaptive immune response	GO:0002250	10.22	SLAMF7 ICOSLG CD4 CD1E CD1A CD19
2	B cell proliferation	GO:0042100	9.99	TNFSF13B IL10 CD38
3	B cell activation	GO:0042113	9.85	CXCR5 ICOSLG IL4
4	negative regulation of endothelial cell apoptotic process	GO:2000352	9.85	IL4 IL13 IL10
5	antigen processing and presentation, endogenous lipid antigen via MHC class Ib	GO:0048006	9.8	CD1E CD1A
6	immune system process	GO:0002376	9.8	TNFSF13B SLAMF7 ICOSLG CD4 CD1E CD1A
7	T cell activation	GO:0042110	9.76	SLAMF7 IL4 ICOSLG CD4
8	regulation of isotype switching	GO:0045191	9.73	IL4 IL10
9	B cell costimulation	GO:0031296	9.71	TNFSF13B IL4
10	negative regulation of complement-dependent cytotoxicity	GO:1903660	9.62	IL13 IL4
11	positive regulation of B cell proliferation	GO:0030890	9.56	CD38 IL13 IL4 TNFSF13B
12	immune response	GO:0006955	9.53	TNFSF13B IL4 IL13 IL10 CXCR5 CD4