MCID: IGG007
MIFTS: 43

Igg4-Related Disease

Categories: Rare diseases, Immune diseases, Genetic diseases

Aliases & Classifications for Igg4-Related Disease

MalaCards integrated aliases for Igg4-Related Disease:

Name: Igg4-Related Disease 53
Multifocal Fibrosclerosis 53 73
Igg4-Positive Multiorgan Lymphoproliferative Syndrome 53
Immunoglobulin G4-Related Sclerosing Disease 53
Systemic Igg4-Related Plasmacytic Syndrome 53
Systemic Igg4-Related Sclerosing Syndrome 53
Igg4-Related Systemic Sclerosing Disease 53
Multifocal Idiopathic Fibrosclerosis 53
Retroperitoneal Fibrosis, Familial 53
Igg4-Related Autoimmune Disease 53
Igg4-Related Sclerosing Disease 53
Mediastinal Fibrosis, Familial 53
Igg4-Related Systemic Disease 53
Igg4-Associated Disease 53
Hyper-Igg4 Disease 53
Igg4-Syndrome 53

Classifications:



External Ids:

ICD10 33 M35.5
UMLS 73 C0494949

Summaries for Igg4-Related Disease

NIH Rare Diseases : 53 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. Some researchers believe that it may be an autoimmune or allergic disorder. IgG4-related disease is usually treated with systemic steroids like prednisone. Immunosuppressive medications (azathioprine or mycophenolate mofetil) and biologics (rituximab or bortezomib) may be used in those who don't respond to steroids or for longer-term management. In severe cases, organ replacement therapy may be needed. 

MalaCards based summary : Igg4-Related Disease, also known as multifocal fibrosclerosis, is related to autoimmune disease and idiopathic hypertrophic pachymeningitis. An important gene associated with Igg4-Related Disease is PDCD1 (Programmed Cell Death 1), and among its related pathways/superpathways are TRAF Pathway and Spinal Cord Injury. The drugs Prednisone and Prednisolone have been mentioned in the context of this disorder. Affiliated tissues include salivary gland, skin and lymph node, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Wikipedia : 76 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 104)
# Related Disease Score Top Affiliating Genes
1 autoimmune disease 27.4 PDCD1 TNFSF13 TNFSF13B
2 idiopathic hypertrophic pachymeningitis 11.3
3 igg4-related dacryoadenitis and sialadenitis 11.1
4 retroperitoneal fibrosis 10.1
5 pancreatitis 10.1
6 autoimmune pancreatitis 10.0
7 lymphoma 10.0
8 asthma 10.0
9 rosai-dorfman disease 10.0
10 castleman disease 9.9
11 prostatitis 9.9
12 aortitis 9.9
13 mediastinitis 9.9
14 wegener granulomatosis 9.8
15 membranous nephropathy 9.8
16 hepatitis 9.8
17 sialadenitis 9.8
18 interstitial nephritis 9.8
19 scleritis 9.8
20 purpura 9.8
21 thyroiditis 9.8
22 fibrosing mediastinitis 9.8
23 fibrosclerosis, multifocal 9.7
24 glomerulonephritis 9.7
25 multicentric castleman disease 9.7
26 sclerosing cholangitis 9.7
27 pericarditis 9.7
28 erdheim-chester disease 9.7
29 conjunctivitis 9.7
30 vasculitis 9.7
31 cholangitis 9.7
32 lipogranulomatosis 9.7
33 aneurysm 9.7
34 biliary cirrhosis, primary, 1 9.6
35 burkitt lymphoma 9.6
36 pulmonary hypertension, primary, 1 9.6
37 thrombocytopenic purpura, autoimmune 9.6
38 lung cancer 9.6
39 enterocolitis 9.6
40 autoimmune lymphoproliferative syndrome 9.6
41 aortic aneurysm, familial thoracic 1 9.6
42 autoimmune disease 1 9.6
43 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.6
44 cholangitis, primary sclerosing 9.6
45 psoriasis 14, pustular 9.6
46 cholangiocarcinoma 9.6
47 alopecia 9.6
48 autoimmune hepatitis 9.6
49 b-cell lymphomas 9.6
50 crohn's disease 9.6

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



Diseases related to Igg4-Related Disease

Symptoms & Phenotypes for Igg4-Related Disease

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-115 9.28 PHB
2 Increased shRNA abundance (Z-score > 2) GR00366-A-199 9.28 PHB
3 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.28 PHB
4 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.28 PDCD1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-36 9.28 PDCD1 PHB
6 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.28 PDCD1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-57 9.28 PHB
8 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.28 PDCD1

Drugs & Therapeutics for Igg4-Related Disease

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

(show all 43)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3 53-03-2 5865
2
Prednisolone Approved, Vet_approved Phase 4,Phase 2 50-24-8 5755
3
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
4
Betamethasone Approved, Vet_approved Phase 4 378-44-9 9782
5
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2 83-43-2 6741
6 glucocorticoids Phase 4,Phase 2,Phase 3
7 Hormone Antagonists Phase 4,Phase 2,Phase 3
8 Hormones Phase 4,Phase 2,Phase 3
9 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3
10 Immunoglobulin G Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
11 Antibodies Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
12 Immunoglobulins Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
13 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3
14 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3
15 Antirheumatic Agents Phase 4,Phase 1,Phase 2
16 Prednisolone acetate Phase 4,Phase 2
17 Betamethasone Valerate Phase 4 2152-44-5
18 Methylprednisolone acetate Phase 4,Phase 2
19 Methylprednisolone Hemisuccinate Phase 4,Phase 2
20 Immunosuppressive Agents Phase 4,Phase 2,Phase 1
21 Prednisolone hemisuccinate Phase 4,Phase 2
22 Prednisolone phosphate Phase 4,Phase 2
23 Liver Extracts Phase 4
24 Betamethasone benzoate Phase 4
25 Betamethasone dipropionate, betamethasone sodium phosphate drug combination Phase 4
26 Betamethasone sodium phosphate Phase 4
27 Betamethasone-17,21-dipropionate Phase 4
28
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
29
Mycophenolic acid Approved Phase 2 24280-93-1 446541
30
Mycophenolate mofetil Approved, Investigational Phase 2 128794-94-5 5281078
31 Anti-Bacterial Agents Phase 2,Phase 1
32 Antibiotics, Antitubercular Phase 2
33 Anti-Infective Agents Phase 2,Phase 1
34 Antitubercular Agents Phase 2
35
Lenalidomide Approved Phase 1 191732-72-6 216326
36 Angiogenesis Inhibitors Phase 1
37 Angiogenesis Modulating Agents Phase 1
38 Fluorides Early Phase 1
39 Fluorodeoxyglucose F18 Early Phase 1
40 Deoxyglucose Early Phase 1
41 Radiopharmaceuticals Early Phase 1
42 Vaccines
43 Autoantibodies

Interventional clinical trials:

(show all 19)
# 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 Recruiting NCT03368274 Phase 4 T 614
2 Leflunomide for Maintenance of Remission in IgG4 Related Disease Recruiting NCT02703194 Phase 4 Prednisone;Leflunomide
3 Glucocorticoids in Patients With IgG4-RD Unknown status NCT01758393 Phase 2, Phase 3 Prednisone
4 Study to Evaluate the Effect of XmAb®5871 on Disease Activity in Patients With IgG4-Related Disease (RD) Completed NCT02725476 Phase 2
5 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
6 Study of Treatment Response on IgG4 Related Disease (IgG4RD) Recruiting NCT02458196 Phase 2 Prednisone;Prednisone and Mycophenolate mofetil
7 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
8 Follicular Helper T Cells: Biological Marker and Involvement in the Physiopathology of the IgG4-related Disease Completed NCT02899039 Not Applicable
9 Retrospective Study for Diagnosis of Undetected IgG4 Related Diseases in Patients With High Serum IgG4 Levels. Completed NCT02229448
10 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695
11 18F-FDG PET/CT for IgG4-Related Disease Recruiting NCT01665196 Early Phase 1 18F-FDG
12 National Registry of IgG4-RD in China Recruiting NCT03023371
13 Bile Usefulness for Detecting IgG4-related Sclerosing Cholangitis Recruiting NCT02616705
14 Comparative Autoantibody and Immunologic Cell Marker Study Enrolling by invitation NCT02422875
15 Plasmablast Detection From IgG4-Related Disease Patients Not yet recruiting NCT03466970
16 A Laboratory Scan of Patients With Aortic Aneurysms to IgG4 Levels in the Blood Not yet recruiting NCT03267875 Not Applicable
17 Meir Medical Center Rheumatologic Biobank Not yet recruiting NCT03473912
18 Characterization of Tissular T Follicular Helper Cells in IgG4-RD Not yet recruiting NCT02872441 Not Applicable
19 Evaluation Of Pleural Effusion At Assiut University Hospital Not yet recruiting NCT03260088

Search NIH Clinical Center for Igg4-Related Disease

Genetic Tests for Igg4-Related Disease

Anatomical Context for Igg4-Related Disease

MalaCards organs/tissues related to Igg4-Related Disease:

41
Salivary Gland, Skin, Lymph Node, Thyroid, T Cells, Lung, Liver

Publications for Igg4-Related Disease

Articles related to Igg4-Related Disease:

(show top 50) (show all 271)
# Title Authors Year
1
Treatment and outcomes in patients with IgG4-related disease using the IgG4 responder index. ( 29452298 )
2018
2
Interleukin-4 contributes to the shift of balance of IgG subclasses toward IgG4 in IgG4-related disease. ( 29861381 )
2018
3
Paratesticular Fibrous Pseudotumor: A New Entity of IgG4-Related Disease? ( 29970445 )
2018
4
IgG4-related disease presenting with scarring alopecia of the scalp. ( 29892674 )
2018
5
Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male. ( 29366363 )
2018
6
IgG4-Related Disease Mimicking Crohn's Disease: A Case Report and Review of Literature. ( 29417330 )
2018
7
IgG4-Related Disease of the Thyroid Gland Requiring Emergent Total Thyroidectomy: A Case Report. ( 29855801 )
2018
8
IgG4-related disease and intracranial hypertenstion: Case report of a novel mechanistic association. ( 29406918 )
2018
9
Risk factors of relapse following glucocorticoid tapering in IgG4-related disease. ( 29846165 )
2018
10
Glucocorticoid receptor expression in resident and hematopoietic cells in IgG4-related disease. ( 29434340 )
2018
11
IgG4-related disease: features and treatment response in a multi-ethnic cohort in Singapore. ( 29846168 )
2018
12
The Clinicopathologic Spectrum of IgG4-Related Disease. ( 29866638 )
2018
13
IgG4-Related Disease with Lung Involvement. ( 29935778 )
2018
14
Two cases of generalized pustular psoriasis complicated by IgG4-related disease. ( 29709076 )
2018
15
Clivus lesion revealed inflammatory pseudotumor associated with IgG4-related disease: a case report. ( 29966794 )
2018
16
IgG4-related disease with skin, submaxillary and pulmonary involvement. ( 29938895 )
2018
17
Sustained clinical response after single course of rituximab as first-line monotherapy in adult-onset asthma and periocular xanthogranulomas syndrome associated with IgG4-related disease: A case report. ( 29952960 )
2018
18
Comment on the article entitled "Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome" (Autoimmunity Reviews 16 (2017) 1036-1043). ( 29427824 )
2018
19
The Utility of FDG PET/CT in IgG4-Related Disease with a Focus on Coronary Artery Involvement. ( 29391913 )
2018
20
Idiopathic thrombocytopenic purpura in a patient with IgG4-related disease. ( 29888414 )
2018
21
IgG4-related disease: review of the histopathologic features, differential diagnosis, and therapeutic approach. ( 29924455 )
2018
22
Combined membranous nephropathy and tubulointerstitial nephritis as a rare renal manifestation of IgG4-related disease: a case-based literature review. ( 29388171 )
2018
23
Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease: a multicentre study. ( 29980706 )
2018
24
Retroperitoneal fibrosis associated with IgG4-related disease diagnosed by prostate biopsy developed with acute post-renal renal failure: A case report. ( 29034178 )
2018
25
Hepatobiliary and Pancreatic: Burkitt lymphoma mimicking IgG4-related disease. ( 29808497 )
2018
26
IgG4-related disease of the central and peripheral nervous systems. ( 29413316 )
2018
27
Rituximab for retroperitoneal fibrosis due toA IgG4-related disease: A case report and literature review. ( 29845002 )
2018
28
No specific imaging pattern can help differentiate IgG4-related disease from idiopathic retroperitoneal fibrosis: 18 histologically proven cases. ( 29465374 )
2018
29
LatY136F knock-in mouse model for human IgG4-related disease. ( 29902238 )
2018
30
Hepatitis B virus Reactivation During Steroid Treatment of IgG4-Related Disease. ( 29311390 )
2018
31
IgG4-related disease of the mitral valve demonstrated by immunohistochemistry. ( 29437558 )
2018
32
IgG4-related disease: association between chronic rhino-sinusitis and systemic symptoms. ( 29948263 )
2018
33
The tarsal plate manifestation of IgG4-related disease. ( 29926366 )
2018
34
IgG4-related disease. ( 28871762 )
2017
35
IgG4-related disease in autoimmune lymphoproliferative syndrome. ( 28478106 )
2017
36
IgG4-related disease with possible myocardial involvement. ( 29277579 )
2017
37
Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome. ( 28780079 )
2017
38
Lymphomas in IgG4-related disease: clinicopathologic features in a Western population. ( 29285637 )
2017
39
Unexpected Fibrosing Mediastinitis Shown on FDG PET/CT in a Patient With IgG4-Related Disease. ( 28806261 )
2017
40
IgG4-related disease as the differential diagnosis of hypophysitis. ( 28898148 )
2017
41
Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. ( 28070877 )
2017
42
IL-1 family cytokines and receptors in IgG4-related disease. ( 28823915 )
2017
43
IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis. ( 29196804 )
2017
44
IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis. ( 28552902 )
2017
45
IgG4-related disease and IgG4-mediated neurological autoimmune disorders: One and the same? ( 28778436 )
2017
46
IgG4-related disease of the lung: a rare differential diagnosis to lung cancer after positive positron emission tomography and biopsy. ( 28950319 )
2017
47
A Challenging Diagnosis of IgG4-Related Disease When Understanding Limitations of Laboratory Testing Was Pivotal. ( 28828007 )
2017
48
IgG4-related Disease - A Patient with Multiple Organ Involvement. ( 28922106 )
2017
49
IgG4-related disease presenting as posterior scleritis and vitritis, progressing to multifocal orbital involvement. ( 28438752 )
2017
50
IgG4-Related Disease With Orbital Pseudotumors Treated With Rituximab Combined With Palpebral Surgery. ( 28055067 )
2017

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.85 TNFSF13 TNFSF13B
2 11.34 TNFSF13 TNFSF13B
3 11.11 TNFSF13 TNFSF13B
4
Show member pathways
10.82 TNFSF13 TNFSF13B
5 10.1 TNFSF13 TNFSF13B

GO Terms for Igg4-Related Disease

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

# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 9.26 PDCD1 PHB TNFSF13 TNFSF13B
2 tumor necrosis factor-mediated signaling pathway GO:0033209 9.16 TNFSF13 TNFSF13B
3 T cell costimulation GO:0031295 8.62 PDCD1 TNFSF13B

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

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 8.96 TNFSF13 TNFSF13B
2 tumor necrosis factor receptor binding GO:0005164 8.62 TNFSF13 TNFSF13B

Sources for Igg4-Related Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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