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 12 77 54 15
Multifocal Fibrosclerosis 54 74
Igg4-Positive Multiorgan Lymphoproliferative Syndrome 54
Immunoglobulin G4-Related Sclerosing Disease 54
Systemic Igg4-Related Plasmacytic Syndrome 54
Systemic Igg4-Related Sclerosing Syndrome 54
Igg4-Related Systemic Sclerosing Disease 54
Multifocal Idiopathic Fibrosclerosis 54
Retroperitoneal Fibrosis, Familial 54
Igg4-Related Autoimmune Disease 54
Igg4-Related Sclerosing Disease 54
Mediastinal Fibrosis, Familial 54
Igg4-Related Systemic Disease 54
Igg4-Associated Disease 54
Hyper-Igg4 Disease 54
Igg4-Syndrome 54

Classifications:



External Ids:

Disease Ontology 12 DOID:0080356
ICD10 34 M35.5
UMLS 74 C0494949

Summaries for Igg4-Related Disease

NIH Rare Diseases : 54 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 interstitial nephritis and systemic lupus erythematosus. An important gene associated with Igg4-Related Disease is TNFSF13B (TNF Superfamily Member 13b), and among its related pathways/superpathways are Innate Immune System and Apoptosis and survival Anti-apoptotic TNFs/NF-kB/Bcl-2 pathway. The drugs Methylprednisolone hemisuccinate and Methylprednisolone have been mentioned in the context of this disorder. Affiliated tissues include salivary gland, skin and t cells.

Disease Ontology : 12 An autoimmune hypersensitivity 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 : 77 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 217)
# Related Disease Score Top Affiliating Genes
1 interstitial nephritis 29.9 CRP PDCD1
2 systemic lupus erythematosus 28.7 CRP PDCD1 TNFSF13 TNFSF13B
3 idiopathic hypertrophic pachymeningitis 11.4
4 mikulicz disease 11.3
5 pancreatitis 10.3
6 autoimmune pancreatitis 10.3
7 retroperitoneal fibrosis 10.3
8 lymphoma 10.3
9 radin blood group antigen 10.3
10 membranous nephropathy 10.2
11 sjogren syndrome 10.2
12 diabetes insipidus 10.2
13 rosai-dorfman disease 10.2
14 asthma 10.1
15 opsoclonus-myoclonus syndrome 10.1 TNFSF13 TNFSF13B
16 granulomatosis with polyangiitis 10.1
17 castleman disease 10.1
18 aortitis 10.1
19 muscle hypertrophy 10.1
20 hepatitis 10.1
21 vasculitis 10.1
22 pituitary hormone deficiency, combined, 2 10.0
23 portal hypertension 10.0
24 histiocytosis 10.0
25 exophthalmos 10.0
26 fibrosclerosis, multifocal 10.0
27 diffuse large b-cell lymphoma 10.0
28 sialadenitis 10.0
29 scleritis 10.0
30 purpura 10.0
31 aortic aneurysm 10.0
32 thyroiditis 10.0
33 polyarteritis nodosa 10.0
34 takayasu arteritis 10.0
35 aortic aneurysm, familial abdominal, 1 10.0
36 cardiac conduction defect 10.0
37 lung cancer 10.0
38 nodal marginal zone lymphoma 10.0
39 kidney disease 10.0
40 mediastinitis 10.0
41 lung disease 10.0
42 cholangitis 10.0
43 dacryoadenitis 10.0
44 hypereosinophilic syndrome 10.0
45 fibrosing mediastinitis 10.0
46 autoimmune disease of musculoskeletal system 10.0 CRP TNFSF13B
47 pilomatrixoma 9.9
48 oral squamous cell carcinoma 9.9
49 sarcoma 9.9
50 phaeohyphomycosis 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

Drugs & Therapeutics for Igg4-Related Disease

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

(show all 45)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone hemisuccinate Approved Phase 4,Phase 2 2921-57-5
2
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2 83-43-2 6741
3
Prednisolone Approved, Vet_approved Phase 4,Phase 2 50-24-8 5755
4
Prednisone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 53-03-2 5865
5
Prednisolone phosphate Approved, Vet_approved Phase 4,Phase 2 302-25-0
6
Leflunomide Approved, Investigational Phase 4,Not Applicable 75706-12-6 3899
7
Betamethasone Approved, Vet_approved Phase 4 378-44-9 9782
8
Prednisolone hemisuccinate Experimental Phase 4,Phase 2 2920-86-7
9 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
10 Immunoglobulin G Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1,Not Applicable
11 Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
12 Methylprednisolone Acetate Phase 4,Phase 2
13 Prednisolone acetate Phase 4,Phase 2
14 Antibodies Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1,Not Applicable
15 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Not Applicable
16 Hormones Phase 4,Phase 2,Phase 3,Not Applicable
17 Immunologic Factors Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1,Not Applicable
18 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
19 Immunosuppressive Agents Phase 4,Phase 2,Not Applicable
20 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
21 Immunoglobulins Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1,Not Applicable
22 Liver Extracts Phase 4
23 Betamethasone Valerate Phase 4 2152-44-5
24 Betamethasone-17,21-dipropionate Phase 4
25 Betamethasone benzoate Phase 4
26 Betamethasone sodium phosphate Phase 4
27 Betamethasone dipropionate, betamethasone sodium phosphate drug combination Phase 4
28
Mycophenolic acid Approved Phase 2 24280-93-1 446541
29
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
30
Abatacept Approved Phase 2 332348-12-6 10237
31 Antitubercular Agents Phase 2
32 Anti-Infective Agents Phase 2
33 Antibiotics, Antitubercular Phase 2
34 Anti-Bacterial Agents Phase 2
35 Antineoplastic Agents, Immunological Phase 1, Phase 2
36 Antirheumatic Agents Phase 1, Phase 2,Phase 2
37
Lenalidomide Approved Phase 1 191732-72-6 216326
38 Angiogenesis Inhibitors Phase 1
39 Angiogenesis Modulating Agents Phase 1
40 Fluorides Early Phase 1
41 Deoxyglucose Early Phase 1
42 Radiopharmaceuticals Early Phase 1
43 Fluorodeoxyglucose F18 Early Phase 1
44 Vaccines
45 Autoantibodies

Interventional clinical trials:

(show all 22)
# Name Status NCT ID Phase Drugs
1 Leflunomide for Maintenance of Remission in IgG4 Related Disease Completed NCT02703194 Phase 4 Prednisone;Leflunomide
2 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
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 Active, not recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
9 18F-FDG PET/CT for IgG4-Related Disease Unknown status NCT01665196 Early Phase 1 18F-FDG
10 Follicular Helper T Cells: Biological Marker and Involvement in the Physiopathology of the IgG4-related Disease Completed NCT02899039 Not Applicable
11 Retrospective Study for Diagnosis of Undetected IgG4 Related Diseases in Patients With High Serum IgG4 Levels. Completed NCT02229448
12 Infraorbital Nerve Involvement on Magnetic Resonance Imaging in IgG4-related Ophthalmic Disease Completed NCT03690908
13 National Registry of IgG4-RD in China Recruiting NCT03023371
14 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695
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 Meir Medical Center Rheumatologic Biobank Recruiting NCT03473912
18 Comparative Autoantibody and Immunologic Cell Marker Study Enrolling by invitation NCT02422875
19 Plasmablast Detection From IgG4-Related Disease Patients Not yet recruiting NCT03466970
20 Characterization of Tissular T Follicular Helper Cells in IgG4-RD Not yet recruiting NCT02872441 Not Applicable
21 A Laboratory Scan of Patients With Aortic Aneurysms to IgG4 Levels in the Blood Not yet recruiting NCT03267875 Not Applicable
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:

42
Salivary Gland, Skin, T Cells, Lung, Thyroid, Lymph Node, B Cells

Publications for Igg4-Related Disease

Articles related to Igg4-Related Disease:

(show top 50) (show all 851)
# Title Authors Year
1
Parathyroid adenoma with prominent lymphocytic infiltrate having histological features highly suggestive of IgG4-related disease: a case report and literature review. ( 30726786 )
2019
2
Abdominal aortic aneurysm as an IgG4-related disease. ( 31032886 )
2019
3
IgG4-related Disease Presenting as Cardiac Arrest. ( 29551702 )
2019
4
Differences and similarities between IgG4-related disease with and without dacryoadenitis and sialoadenitis: clinical manifestations and treatment efficacy. ( 30709411 )
2019
5
A Rare Case of IgG4-Related Disease Presenting as a Unilateral Severe Dacryoadenitis Complicated by Hypophysitis and Hypertrophic Pachymeningitis. ( 30724757 )
2019
6
Diffuse large B cell lymphoma with chromosomal translocation t(14;19)(q32;q13) occurring in IgG4-related disease. ( 31111176 )
2019
7
IgG4-Related Disease and Eosinophilic Granulomatosis with Polyangiitis: Similarity or Coexistence? ( 30772965 )
2019
8
Large dural-based mass with bony hyperostosis in a 16-year-old male: IgG4-related disease mimicking lymphoplasmacyte-rich meningioma. ( 31073682 )
2019
9
Effects of glucocorticoids on B-cell subpopulations in patients with IgG4-related disease. ( 30652677 )
2019
10
IL-10+ T follicular regulatory cells are associated with the pathogenesis of IgG4-related disease. ( 30658078 )
2019
11
IgG4-Related Disease: Is Rituximab the Best Therapeutic Strategy for Cases Refractory to Conventional Therapy? Results of a Systematic Review. ( 30665856 )
2019
12
Analysis of allergic reaction in IgG4-related disease. ( 30676808 )
2019
13
IgG4-related disease - A new disease entity developed in the twenty-first century. ( 30676824 )
2019
14
Major salivary gland enlargement in IgG4-related disease is associated with multiorgan involvement and higher basal disease activity. ( 30676828 )
2019
15
Up-regulation of activation-induced cytidine deaminase and its strong expression in extra-germinal centres in IgG4-related disease. ( 30679751 )
2019
16
IgG4-related disease in endocrine practice. ( 30697253 )
2019
17
IgG4-related disease: what a hematologist needs to know. ( 30705099 )
2019
18
IgG4-related disease can present as recurrent spontaneous hemothorax: a case report. ( 30709387 )
2019
19
Pulmonary vascular involvement of IgG4-related disease: Case series with a PRISMA-compliant systemic review. ( 30732204 )
2019
20
Temporal bone involvement of IgG4-related disease: a rare condition misleading to petrous apicitis causing lateral rectus palsy. ( 30814106 )
2019
21
Neurologic IgG4-Related Disease. ( 30915193 )
2019
22
A case with good response to belimumab for lupus nephritis complicated by IgG4-related disease. ( 30917769 )
2019
23
Clinicopathological features of cutaneous Rosai-Dorfman disease and its relationship to IgG4-related disease:a retrospective study. ( 30920643 )
2019
24
IgG4-related disease in patients with newly diagnosed idiopathic retroperitoneal fibrosis: a population-based Danish study. ( 30931680 )
2019
25
Thoracic Hyper-IgG4-Related Disease Mimicking Malignant Pleural Mesothelioma. ( 30941506 )
2019
26
IgG4-related disease in a patient with HIV infection. ( 30988103 )
2019
27
IgG4-related disease: Association with a rare gene variant expressed in cytotoxic T cells. ( 30993913 )
2019
28
IgG4-Related Disease Presenting as Cervical Lymphadenopathy. ( 31031535 )
2019
29
IgG4-Related Disease with Emphasis on Its Gastrointestinal Manifestation. ( 31046976 )
2019
30
The IFN-α-IL-33 Axis as Possible Biomarkers in IgG4-Related Disease. ( 31058651 )
2019
31
IgG4 related disease: A single centre experience from South Australia. ( 31059167 )
2019
32
Phospholipase A2 receptor-associated membranous nephropathy in a patient with IgG4-related disease: A case report. ( 31096469 )
2019
33
Genetic analysis of IgG4-related disease. ( 31104539 )
2019
34
Identification of galectin-3 as an autoantigen in patients with IgG4-related disease. ( 29852256 )
2019
35
IgG4-Related Disease with Lung Involvement. ( 29935778 )
2019
36
Pathogenesis of IgG4-related disease: a critical review. ( 30019169 )
2019
37
Radiotherapy for parotid IgG4-related disease. ( 30198210 )
2019
38
IgG4-related disease: a new challenging diagnosis mimicking lung cancer. ( 30295799 )
2019
39
Retroperitoneal Fibrosis Diagnosed as IgG4-related Disease after 35 Years. ( 30333398 )
2019
40
Clinical Questions and an Inquiring Mind for Patients with IgG4-related Disease. ( 30333424 )
2019
41
Cytokines produced by innate immune cells in IgG4-related disease. ( 30334477 )
2019
42
Immunophenotype involved in IgG4-related disease. ( 30334637 )
2019
43
IgG4-related disease of the paratestis with the scrotal fluid: A case report. ( 30375182 )
2019
44
IgG4-related disease in patients with idiopathic orbital inflammation syndrome: data from the French SIOI prospective cohort. ( 30421562 )
2019
45
New insights into IgG4-related disease: emerging new CD4+ T-cell subsets. ( 30422824 )
2019
46
Morphologic Overlap Between Inflammatory Myofibroblastic Tumor and IgG4-related Disease: Lessons From Next-generation Sequencing. ( 30451733 )
2019
47
Does IgG4 level at the time of diagnosis correlate with disease outcome in IgG4-Related disease? ( 30509569 )
2019
48
Pulmonary inflammatory myofibroblastic tumour and IgG4-related disease presenting with lower limb paralysis. ( 30580286 )
2019
49
Retroperitoneal fibrosis. Steroid treatment response seems to depend on its association to IgG4 related disease. ( 30593393 )
2019
50
Clinical Manifestations and Long-term Outcomes of IgG4-Related Kidney and Retroperitoneal Involvement in a United Kingdom IgG4-Related Disease Cohort. ( 30596168 )
2019

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
12.87 CRP DEFA3 DEFA4 IL33 MS4A3 PDCD1
2
Show member pathways
11.02 TNFSF13 TNFSF13B
3 10.43 TNFSF13 TNFSF13B

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 extracellular space GO:0005615 9.43 CRP DEFA3 DEFA4 IL33 TNFSF13 TNFSF13B
2 extracellular region GO:0005576 9.17 CRP DEFA3 DEFA4 IL33 PLA2R1 TNFSF13

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 9.48 TNFSF13 TNFSF13B
2 antimicrobial humoral immune response mediated by antimicrobial peptide GO:0061844 9.46 DEFA3 DEFA4
3 antimicrobial humoral response GO:0019730 9.43 DEFA3 DEFA4
4 T cell costimulation GO:0031295 9.4 PDCD1 TNFSF13B
5 killing of cells of other organism GO:0031640 9.37 DEFA3 DEFA4
6 antibacterial humoral response GO:0019731 9.32 DEFA3 DEFA4
7 defense response to fungus GO:0050832 9.26 DEFA3 DEFA4
8 innate immune response in mucosa GO:0002227 9.16 DEFA3 DEFA4
9 defense response to Gram-positive bacterium GO:0050830 9.13 CRP DEFA3 DEFA4
10 membrane disruption in other organism GO:0051673 8.62 DEFA3 DEFA4

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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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