MCID: IGG007
MIFTS: 50

Igg4-Related Disease

Categories: Bone diseases, Endocrine diseases, Genetic diseases, Immune diseases, Rare diseases
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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:

Orphanet: 58  
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 11 DOID:0080356
ICD10 31 M35.5
UMLS via Orphanet 72 C3203653
ICD11 33 99883782
UMLS 71 C0494949

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...

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:



Diseases related to Igg4-Related Disease

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

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
1
Betamethasone Approved, Vet_approved Phase 4 378-44-9 9782
2
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
3
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
4
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
5
Prednisolone Approved, Vet_approved Phase 4 50-24-8 4894 5755
6
Belimumab Approved Phase 4 356547-88-1
7
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5 1875
8
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 4159 6741
9
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7 4897
10 Betamethasone-17,21-dipropionate Phase 4
11
Betamethasone Valerate Phase 4 2152-44-5
12
Betamethasone sodium phosphate Phase 4
13 Betamethasone dipropionate, betamethasone sodium phosphate drug combination Phase 4
14
Betamethasone benzoate Phase 4
15 Liver Extracts Phase 4
16 glucocorticoids Phase 4
17 Immunosuppressive Agents Phase 4
18 Immunologic Factors Phase 4
19 Antineoplastic Agents, Hormonal Phase 4
20 Hormones Phase 4
21 Hormone Antagonists Phase 4
22 Anti-Inflammatory Agents Phase 4
23
Methylprednisolone Acetate Phase 4 584547
24 Immunoglobulins Phase 3
25 Antibodies, Monoclonal Phase 3
26 Antibodies Phase 3
27
Mycophenolic acid Approved, Investigational Phase 2 24280-93-1 446541
28
Rituximab Approved Phase 1, Phase 2 174722-31-7
29
Abatacept Approved Phase 2 332348-12-6
30
Zanubrutinib Approved, Investigational Phase 2 1691249-45-2 135565884
31
Acetaminophen Approved Phase 2 103-90-2 1983
32
Famotidine Approved Phase 2 76824-35-6 3325 5702160
33
Histamine Approved, Investigational Phase 2 51-45-6 774
34
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
35
Elotuzumab Approved Phase 2 915296-00-3
36
Promethazine Approved, Investigational Phase 2 60-87-7 4927
37 Anti-Infective Agents Phase 2
38 Anti-Bacterial Agents Phase 2
39 Antitubercular Agents Phase 2
40 Antibiotics, Antitubercular Phase 2
41 Antirheumatic Agents Phase 2
42 Antineoplastic Agents, Immunological Phase 1, Phase 2
43 Immune Checkpoint Inhibitors Phase 2
44 Protein Kinase Inhibitors Phase 2
45 Immunoglobulins, Intravenous Phase 2
46 Anti-Ulcer Agents Phase 2
47 Neurotransmitter Agents Phase 2
48 Antipyretics Phase 2
49 Neuroprotective Agents Phase 2
50 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

Genetic Tests for Igg4-Related Disease

Anatomical Context for Igg4-Related Disease

Organs/tissues related to Igg4-Related Disease:

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

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
36045066 2023
2
Pulmonary IgG4-related disease with favourable response to rituximab: A case report. 62
36330374 2022
3
Paravertebral involvement in IgG4-related disease. 62
36469346 2022
4
Pulmonary and coronary arterial abnormalities in patients with IgG4-related disease. 62
36299866 2022
5
IgG4-related Disease Mimicking a Paratesticular Tumor and Pelvic Lymph Node Metastasis. 62
35705247 2022
6
High-fat diet aggravates experimental autoimmune pancreatitis through the activation of type I interferon signaling pathways. 62
36403482 2022
7
Otological IgG4-Related Disease With Inner Ear Involvement: A Case Report and Review of Literature. 62
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
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
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
36401985 2022
11
IgG4-related sialadenitis - a rare bilateral submandibular gland enlargement: case report. 62
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
34937495 2022
13
Immunoglobulin G4-related disease with Marked Eosinophilia: A Case Report and Literature Review. 62
36351576 2022
14
Progressive dysphagia caused by IgG4-related sclerosing disease involving the esophagogastric junction. 62
35917876 2022
15
IgG4 paratesticular fibrous pseudotumor: Case presentation and literature review. 62
36164384 2022
16
Current research topics in FAPI theranostics: a bibliometric analysis. 62
36437424 2022
17
Ultrasonic Wave Pericardiectomy for IgG4-Related Constrictive Pericarditis. 62
35085526 2022
18
Marked Enlargement of Bilateral Infraorbital Nerve in a Known Case of IgG4-related Disease. 62
35657676 2022
19
Dr. Sugawara et al reply. 62
36319010 2022
20
Is all that fibrosis idiopathic? Usual interstitial pneumonia in IgG4 related disease. 62
36403359 2022
21
Spectrum of Large and Medium Vessel Vasculitis in Adults: Primary Vasculitides, Arthritides, Connective Tissue, and Fibroinflammatory Diseases. 62
36166150 2022
22
Otologic manifestations of IgG4-related disease. 62
36427792 2022
23
Orbital inflammatory pseudotumor revealing multi-organ immunoglobulin G4-related disease. 62
36445834 2022
24
Multimodality Imaging Evaluation of Coronary IgG4-Related Disease: A "Tumor-Like" Cardiac Lesion. 62
36428873 2022
25
Bilateral lacrimal gland disease: clinical features and outcomes. 62
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
35902401 2022
27
Xanthomatous cutaneous Rosai-Dorfman disease with overlapping features of IgG4-related disease. 62
36262360 2022
28
The role of serological biomarkers in the diagnosis and management of autoimmune pancreatitis. 62
36125384 2022
29
Utility of renal biopsy in differentiating idiopathic multicentric Castleman disease from IgG4-related disease. 62
36414812 2022
30
Severe triple vessel disease secondary to IgG4-related coronary periarteritis. 62
36378869 2022
31
IgG4-Related Disease with Testicular Involvement: Association or Coincidence? 62
36318998 2022
32
Perspectives on current and emerging therapies for IgG4-related disease. 62
36408992 2022
33
Cutaneous IgG4-related disease. 62
36379910 2022
34
A rare case report on hypertrophic pachymeningitis: Serum IgG4-related disease. 62
36188084 2022
35
Bilateral dacryoadenitis associated with IgG4-related disease presenting as orbital pseudo-inflammatory tumor. 62
35933087 2022
36
Thoracic manifestations of IgG4-related disease. 62
36437514 2022
37
IgG4-Related Disease of the Pharynx with Spontaneous Regression. 62
35817139 2022
38
IgG4-related disease presenting as a solitary epidural pseudotumor. 62
36052648 2022
39
Isolated IgG4-related disease of the left maxillary antrum. 62
36328041 2022
40
IgG4-Related Disease of the Central Nervous System; A Case Series. 62
36366774 2022
41
The Positive Predictive Value of a Very High Serum IgG4 Concentration for the Diagnosis of IgG4-Related Disease. 62
36319016 2022
42
Roles of IgG4 and IgG4/IgG ratio to IgG4-related disease in patients with elevated serum IgG4 level. 62
36305979 2022
43
Clues to mortality trends and their related factors in IgG4-related disease: A Japanese single-center retrospective study. 62
36300954 2022
44
Igg4 Related Disease of Skull Base-A Diagnostic Challenge. 62
36452566 2022
45
IgG4-positive plasma cells in nonspecific sialadenitis and sialolithiasis. 62
35606412 2022
46
Isolated IgG4-associated autoimmune hepatitis or the first manifestation of IgG4-related disease? 62
36273965 2022
47
IgG4-Related Disease: sight-threatening orbital disease, spectacular improvement after Rituximab therapy. 62
36269307 2022
48
Reduced COVID-19 vaccine response in patient treated with Dupilumab for IgG4 related disease. 62
36313899 2022
49
Miliary Pulmonary Cryptococcosis in IgG4-related Disease. 62
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
36158321 2022

Variations for Igg4-Related Disease

Expression for Igg4-Related Disease

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

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
Show member pathways
13.69 CCR6 CD19 CD1A CD27 CD4 CRP
2
Show member pathways
13.49 TNFSF13B IL4 IL13 IL10 CXCR5 CD4
3
Show member pathways
13.3 TNFSF13B IL4 IL13 IL10 CXCR5 CD4
4
Show member pathways
13.19 TNFSF13B SDC1 PRTN3 IL4 IL13 IL10
5
Show member pathways
13.15 TNFSF13B IL4 IL13 IL10 CXCR5 CD4
6 12.38 TNFSF13B MPO IL4 IL10 CXCR5 CD38
7
Show member pathways
11.73 IL4 CXCR5 CD4
8 11.73 CXCR5 IL10 IL13 IL4
9 11.65 PRTN3 MPO CD4
10 11.64 IL10 CD4 CD38 CD1A CD19
11 11.56 SDC1 MPO IL4 IL10 CD4 CD38
12 11.54 IL4 IL13 IL10 CXCR5 CD4 CCR6
13
Show member pathways
11.5 IL4 IL13 IL10
14 11.4 IL4 IL13 IL10 CD4
15 11.17 IL4 IL13 IL10 CD4
16 11.14 IL10 IL13 IL4
17 11.12 IL4 IL13 IL10
18 11.05 SDC1 PRTN3 CD4
19 11.02 SLAMF7 IL4 IL10 ICOSLG CXCR5 CRP
20 10.66 IL4 IL13

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

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

# Name GO ID Score Top Affiliating Genes
1 exogenous lipid antigen binding GO:0030884 9.26 CD1E CD1A
2 endogenous lipid antigen binding GO:0030883 8.92 CD1E CD1A

Sources for Igg4-Related Disease

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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