MCID: IGG007
MIFTS: 47

Igg4-Related Disease

Categories: Endocrine diseases, Genetic diseases, Immune diseases, Rare 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 IL33 (Interleukin 33), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Prednisolone and Prednisone 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 190)
# Related Disease Score Top Affiliating Genes
1 autoimmune disease 29.1 PDCD1 TNFSF13 TNFSF13B
2 idiopathic hypertrophic pachymeningitis 11.4
3 mikulicz disease 11.2
4 pancreatitis 10.3
5 autoimmune pancreatitis 10.3
6 retroperitoneal fibrosis 10.3
7 lymphoma 10.2
8 diabetes insipidus 10.2
9 membranous nephropathy 10.2
10 asthma 10.1
11 rosai-dorfman disease 10.1
12 granulomatosis with polyangiitis 10.0
13 castleman disease 10.0
14 aortitis 10.0
15 vasculitis 10.0
16 portal hypertension 10.0
17 histiocytosis 10.0
18 exophthalmos 10.0
19 hepatitis 10.0
20 scleritis 10.0
21 purpura 10.0
22 thyroiditis 10.0
23 polyarteritis nodosa 10.0
24 takayasu arteritis 9.9
25 cardiac conduction defect 9.9
26 lung cancer 9.9
27 sjogren syndrome 9.9
28 b-cell lymphomas 9.9
29 diffuse large b-cell lymphoma 9.9
30 nodal marginal zone lymphoma 9.9
31 sialadenitis 9.9
32 interstitial nephritis 9.9
33 aortic aneurysm 9.9
34 mediastinitis 9.9
35 lung disease 9.9
36 cholangitis 9.9
37 fibrosing mediastinitis 9.9
38 pilomatrixoma 9.9
39 oral squamous cell carcinoma 9.9
40 sarcoma 9.9
41 phaeohyphomycosis 9.9
42 squamous cell carcinoma 9.9
43 papillary carcinoma 9.9
44 clear cell sarcoma 9.9
45 composite lymphoma 9.9
46 paraplegia 9.9
47 cutaneous pseudolymphoma 9.9
48 nonspecific interstitial pneumonia 9.9
49 aortic aneurysm, familial abdominal, 1 9.9
50 pulmonary hypertension, primary, 1 9.9

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

MGI Mouse Phenotypes related to Igg4-Related Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.35 IL33 MARCO PDCD1 TNFSF13 TNFSF13B
2 immune system MP:0005387 9.02 IL33 MARCO PDCD1 TNFSF13 TNFSF13B

Drugs & Therapeutics for Igg4-Related Disease

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

(show all 44)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone Approved, Vet_approved Phase 4,Phase 2 50-24-8 5755
2
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 53-03-2 5865
3
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 4,Phase 2 2921-57-5
5
Prednisolone phosphate Approved, Vet_approved Phase 4,Phase 2 302-25-0
6
Betamethasone Approved, Vet_approved Phase 4 378-44-9 9782
7
Leflunomide Approved, Investigational Phase 4,Not Applicable 75706-12-6 3899
8
Prednisolone hemisuccinate Experimental Phase 4,Phase 2 2920-86-7
9 Antibodies Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
10 Immunologic Factors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
11 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Not Applicable
12 Immunoglobulin G Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
13 Immunoglobulins Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
14 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
15 Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
16 Hormones Phase 4,Phase 2,Phase 3,Not Applicable
17 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
18 Methylprednisolone acetate Phase 4,Phase 2
19 Immunosuppressive Agents Phase 4,Phase 2,Phase 1,Not Applicable
20 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
21 Prednisolone acetate Phase 4,Phase 2
22 Antirheumatic Agents Phase 4,Phase 1,Phase 2,Not Applicable
23 Betamethasone sodium phosphate Phase 4
24 Betamethasone benzoate Phase 4
25 Betamethasone Valerate Phase 4 2152-44-5
26 Betamethasone dipropionate, betamethasone sodium phosphate drug combination Phase 4
27 Betamethasone-17,21-dipropionate Phase 4
28 Liver Extracts Phase 4
29
Mycophenolic acid Approved Phase 2 24280-93-1 446541
30
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
31
Abatacept Approved Phase 2 332348-12-6 10237
32 Anti-Infective Agents Phase 2,Phase 1
33 Antibiotics, Antitubercular Phase 2
34 Anti-Bacterial Agents Phase 2,Phase 1
35 Antitubercular Agents Phase 2
36
Lenalidomide Approved Phase 1 191732-72-6 216326
37 Angiogenesis Modulating Agents Phase 1
38 Angiogenesis Inhibitors Phase 1
39 Deoxyglucose Early Phase 1
40 Fluorides Early Phase 1
41 Fluorodeoxyglucose F18 Early Phase 1
42 Radiopharmaceuticals Early Phase 1
43 Autoantibodies
44 Vaccines

Interventional clinical trials:

(show all 22)
# 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 of Treatment Response on IgG4 Related Disease (IgG4RD) Unknown status NCT02458196 Phase 2 Prednisone;Prednisone and Mycophenolate mofetil
5 Study to Evaluate the Effect of XmAb®5871 on Disease Activity in Patients With IgG4-Related Disease (RD) Completed NCT02725476 Phase 2
6 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
7 Safety and Efficacy of Abatacept in IgG4-Related Disease Recruiting NCT03669861 Phase 2 Abatacept
8 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
9 Follicular Helper T Cells: Biological Marker and Involvement in the Physiopathology of the IgG4-related Disease Completed NCT02899039 Not Applicable
10 Retrospective Study for Diagnosis of Undetected IgG4 Related Diseases in Patients With High Serum IgG4 Levels. Completed NCT02229448
11 Infraorbital Nerve Involvement on Magnetic Resonance Imaging in IgG4-related Ophthalmic Disease Completed NCT03690908
12 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695
13 National Registry of IgG4-RD in China Recruiting NCT03023371
14 18F-FDG PET/CT for IgG4-Related Disease Recruiting NCT01665196 Early Phase 1 18F-FDG
15 Leflunomide Treatment for IgG4-RD Recruiting NCT03715699 Not Applicable Prednisone;Prednisone and Leflunomide
16 Bile Usefulness for Detecting IgG4-related Sclerosing Cholangitis Recruiting NCT02616705
17 Comparative Autoantibody and Immunologic Cell Marker Study Enrolling by invitation NCT02422875
18 Plasmablast Detection From IgG4-Related Disease Patients Not yet recruiting NCT03466970
19 Characterization of Tissular T Follicular Helper Cells in IgG4-RD Not yet recruiting NCT02872441 Not Applicable
20 A Laboratory Scan of Patients With Aortic Aneurysms to IgG4 Levels in the Blood Not yet recruiting NCT03267875 Not Applicable
21 Meir Medical Center Rheumatologic Biobank Not yet recruiting NCT03473912
22 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, Lung, T Cells, Thyroid, B Cells

Publications for Igg4-Related Disease

Articles related to Igg4-Related Disease:

(show top 50) (show all 803)
# Title Authors Year
1
New insights into IgG4-related disease: emerging new CD4+ T-cell subsets. ( 30422824 )
2019
2
Treatment and outcomes in patients with IgG4-related disease using the IgG4 responder index. ( 29452298 )
2018
3
Interleukin-4 contributes to the shift of balance of IgG subclasses toward IgG4 in IgG4-related disease. ( 29861381 )
2018
4
Paratesticular Fibrous Pseudotumor: A New Entity of IgG4-Related Disease? ( 29970445 )
2018
5
IgG4-related disease presenting with scarring alopecia of the scalp. ( 29892674 )
2018
6
Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male. ( 29366363 )
2018
7
IgG4-Related Disease Mimicking Crohn's Disease: A Case Report and Review of Literature. ( 29417330 )
2018
8
IgG4-Related Disease of the Thyroid Gland Requiring Emergent Total Thyroidectomy: A Case Report. ( 29855801 )
2018
9
IgG4-related disease and intracranial hypertenstion: Case report of a novel mechanistic association. ( 29406918 )
2018
10
Risk factors of relapse following glucocorticoid tapering in IgG4-related disease. ( 29846165 )
2018
11
Glucocorticoid receptor expression in resident and hematopoietic cells in IgG4-related disease. ( 29434340 )
2018
12
IgG4-related disease: features and treatment response in a multi-ethnic cohort in Singapore. ( 29846168 )
2018
13
The Clinicopathologic Spectrum of IgG4-Related Disease. ( 29866638 )
2018
14
IgG4-Related Disease with Lung Involvement. ( 29935778 )
2018
15
Two cases of generalized pustular psoriasis complicated by IgG4-related disease. ( 29709076 )
2018
16
Clivus lesion revealed inflammatory pseudotumor associated with IgG4-related disease: a case report. ( 29966794 )
2018
17
IgG4-related disease with skin, submaxillary and pulmonary involvement. ( 29938895 )
2018
18
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
19
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
20
The Utility of FDG PET/CT in IgG4-Related Disease with a Focus on Coronary Artery Involvement. ( 29391913 )
2018
21
Idiopathic thrombocytopenic purpura in a patient with IgG4-related disease. ( 29888414 )
2018
22
IgG4-related disease: review of the histopathologic features, differential diagnosis, and therapeutic approach. ( 29924455 )
2018
23
Combined membranous nephropathy and tubulointerstitial nephritis as a rare renal manifestation of IgG4-related disease: a case-based literature review. ( 29388171 )
2018
24
Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease: a multicentre study. ( 29980706 )
2018
25
Retroperitoneal fibrosis associated with IgG4-related disease diagnosed by prostate biopsy developed with acute post-renal renal failure: A case report. ( 29034178 )
2018
26
Hepatobiliary and Pancreatic: Burkitt lymphoma mimicking IgG4-related disease. ( 29808497 )
2018
27
IgG4-related disease of the central and peripheral nervous systems. ( 29413316 )
2018
28
Rituximab for retroperitoneal fibrosis due toA IgG4-related disease: A case report and literature review. ( 29845002 )
2018
29
No specific imaging pattern can help differentiate IgG4-related disease from idiopathic retroperitoneal fibrosis: 18 histologically proven cases. ( 29465374 )
2018
30
LatY136F knock-in mouse model for human IgG4-related disease. ( 29902238 )
2018
31
Hepatitis B virus Reactivation During Steroid Treatment of IgG4-Related Disease. ( 29311390 )
2018
32
IgG4-related disease of the mitral valve demonstrated by immunohistochemistry. ( 29437558 )
2018
33
IgG4-related disease: association between chronic rhino-sinusitis and systemic symptoms. ( 29948263 )
2018
34
Aphthous Stomatitis and Laryngitis, Another Form of Presentation of an IgG4-Related Disease? ( 30297197 )
2018
35
Current Concept of Autoimmune Pancreatitis and IgG4-related Disease. ( 30002467 )
2018
36
Mechanistic Insights into Autoimmune Pancreatitis and IgG4-Related Disease. ( 30401468 )
2018
37
Current perspectives on autoimmune pancreatitis and IgG4-related disease. ( 30541967 )
2018
38
IgG4-related Disease Presenting as Cardiac Arrest. ( 29551702 )
2018
39
IgG4-related Disease in the Stomach which Was Confused with Gastrointestinal Stromal Tumor (GIST): Two Case Reports and Review of the Literature. ( 29629225 )
2018
40
No overlap between IgG4-related disease and microscopic polyangiitis and granulomatosis with polyangiitis despite elevated serum IgG4 at diagnosis: a retrospective monocentric study. ( 30552533 )
2018
41
Epstein-Barr Virus Lytic Reactivation Induces IgG4 Production by Host B Lymphocytes in Graves' Disease Patients and Controls: A Subset of Graves' Disease Is an IgG4-Related Disease-Like Condition. ( 30222515 )
2018
42
A case of hypertrophic herpes simplex virus affecting the eyelid and cornea masquerading as IgG4-related disease. ( 29468223 )
2018
43
CC-chemokine ligand 18 is a useful biomarker associated with disease activity in IgG4-related disease. ( 29030359 )
2018
44
Multi-organ IgG4-related disease: Demystifying the diagnostic enigma. ( 29067928 )
2018
45
Implications of elevated C-reactive protein and serum amyloid A levels in IgG4-related disease: comment on the article by Perugino et al. ( 29088583 )
2018
46
Deciphering the biology of IgG4-related disease: specific antigens and disease? ( 29101259 )
2018
47
IgG4-related disease. ( 29110571 )
2018
48
IgG4-related disease presenting with raised serum IgG2-real timeline of IgG4-RD? ( 29155962 )
2018
49
Lymphadenopathy associated with IgG4-related disease: Diagnosis & differential diagnosis. ( 29157939 )
2018
50
IgG4-Related Disease of the Appendix. ( 29188490 )
2018

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

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 tumor necrosis factor-mediated signaling pathway GO:0033209 9.16 TNFSF13 TNFSF13B
2 regulation of signaling receptor activity GO:0010469 9.13 IL33 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 tumor necrosis factor receptor binding GO:0005164 8.96 TNFSF13 TNFSF13B
2 cytokine activity GO:0005125 8.8 IL33 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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