MCID: IGG014
MIFTS: 28

Igg4-Related Sclerosing Cholangitis

Categories: Bone diseases, Liver diseases, Rare diseases

Aliases & Classifications for Igg4-Related Sclerosing Cholangitis

MalaCards integrated aliases for Igg4-Related Sclerosing Cholangitis:

Name: Igg4-Related Sclerosing Cholangitis 59

Characteristics:

Orphanet epidemiological data:

59
igg4-related sclerosing cholangitis
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 59 ORPHA447764
ICD10 via Orphanet 34 K83.0

Summaries for Igg4-Related Sclerosing Cholangitis

MalaCards based summary : Igg4-Related Sclerosing Cholangitis is related to sclerosing cholangitis and cholangitis. An important gene associated with Igg4-Related Sclerosing Cholangitis is CCL1 (C-C Motif Chemokine Ligand 1), and among its related pathways/superpathways are PAK Pathway and CCR5 Pathway in Macrophages. The drugs Prednisolone and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and t cells.

Related Diseases for Igg4-Related Sclerosing Cholangitis

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

(show all 17)
# Related Disease Score Top Affiliating Genes
1 sclerosing cholangitis 11.0
2 cholangitis 11.0
3 cholangiocarcinoma 10.6
4 pancreatitis 10.5
5 autoimmune pancreatitis 10.5
6 cholangitis, primary sclerosing 10.4
7 biliary cirrhosis, primary, 1 10.2
8 anemia, autoimmune hemolytic 10.2
9 hemolytic anemia 10.2
10 primary biliary cirrhosis 10.2
11 primary biliary cholangitis 10.2
12 autoimmune hepatitis 10.1
13 hepatitis 10.1
14 liver disease 10.1
15 sialadenitis 10.1
16 obstructive jaundice 10.1
17 bile duct cancer 10.1

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



Diseases related to Igg4-Related Sclerosing Cholangitis

Symptoms & Phenotypes for Igg4-Related Sclerosing Cholangitis

Drugs & Therapeutics for Igg4-Related Sclerosing Cholangitis

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

(show all 28)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
2
Prednisone Approved, Vet_approved Phase 4,Not Applicable 53-03-2 5865
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
6
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
7
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
8 Immunoglobulins Phase 4,Phase 1,Phase 2,Not Applicable
9 Immunologic Factors Phase 4,Phase 1,Phase 2
10 Immunoglobulin G Phase 4,Phase 1,Phase 2,Not Applicable
11 Antibodies Phase 4,Phase 1,Phase 2,Not Applicable
12 Antirheumatic Agents Phase 4,Phase 1,Phase 2
13 Methylprednisolone acetate Phase 4
14 Immunosuppressive Agents Phase 4,Phase 1
15 Antineoplastic Agents, Hormonal Phase 4
16 Hormone Antagonists Phase 4
17 Prednisolone acetate Phase 4
18 Hormones Phase 4
19 Anti-Inflammatory Agents Phase 4
20 glucocorticoids Phase 4
21 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
22 Liver Extracts Phase 4
23
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
24
Lenalidomide Approved Phase 1 191732-72-6 216326
25 Anti-Infective Agents Phase 1
26 Angiogenesis Modulating Agents Phase 1
27 Angiogenesis Inhibitors Phase 1
28 Anti-Bacterial Agents Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Leflunomide for Maintenance of Remission in IgG4 Related Disease Recruiting NCT02703194 Phase 4 Prednisone;Leflunomide
2 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
3 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
4 Bile Usefulness for Detecting IgG4-related Sclerosing Cholangitis Recruiting NCT02616705
5 Treatment of Obstructive Jaundice in Autoimmune Pancreatitis and/or Immunoglobulin G4-related Sclerosing Cholangitis by Corticosteroids Active, not recruiting NCT02797665 Not Applicable corticosteroids

Search NIH Clinical Center for Igg4-Related Sclerosing Cholangitis

Genetic Tests for Igg4-Related Sclerosing Cholangitis

Anatomical Context for Igg4-Related Sclerosing Cholangitis

MalaCards organs/tissues related to Igg4-Related Sclerosing Cholangitis:

41
Liver, Bone, T Cells

Publications for Igg4-Related Sclerosing Cholangitis

Articles related to Igg4-Related Sclerosing Cholangitis:

(show top 50) (show all 62)
# Title Authors Year
1
Differential diagnosis of cholangiocarcinoma and IgG4-related sclerosing cholangitis by fluorescence inA situ hybridization using transpapillary forceps biopsy specimens. ( 29532638 )
2018
2
Localized intrahepatic IgG4-related sclerosing cholangitis (IgG4-SC) as an additional type of IgG4-SC: a systematic analysis of 12 cases. ( 29313399 )
2018
3
The Immunobiology of RANKL and Myeloid Derived Suppressor Cell Activation in IgG4-related Sclerosing Cholangitis. ( 29774578 )
2018
4
IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed with Liver Biopsy. ( 30305974 )
2018
5
A good response to steroid therapy in IgG4-related sclerosing cholangitis: a case report. ( 30324147 )
2018
6
Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis. ( 30186095 )
2018
7
IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis. ( 30205418 )
2018
8
Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report. ( 28202062 )
2017
9
Case report: A female case of isolated IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma. ( 28422840 )
2017
10
IgG4-related sclerosing cholangitis and chronic sclerosing sialadenitis mimicking cholangiocarcinoma and neck malignancy. ( 28823378 )
2017
11
Clinical Features, Response to Treatment, and Outcomes ofA IgG4-Related Sclerosing Cholangitis. ( 28111336 )
2017
12
IgG4-related sclerosing cholangitis overlapping with autoimmune hepatitis: Report of a case. ( 28238541 )
2017
13
Autoimmune Hemolytic Anemia Obscured by the Obstructive Jaundice Associated with IgG4-related Sclerosing Cholangitis in a Patient with Type 1 Autoimmune Pancreatitis: A Case Report and Review of the Literature. ( 29269682 )
2017
14
Poor response of initial steroid therapy for IgG4-related sclerosing cholangitis with multiple organs affected. ( 28328835 )
2017
15
IgG4-related Sclerosing Cholangitis with No Biliary Stricture but Severe Thickening of the Bile Duct Wall. ( 27301508 )
2016
16
IgG4-related sclerosing cholangitis: all we need to know. ( 26817943 )
2016
17
Comparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma. ( 26703117 )
2016
18
Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis. ( 27470434 )
2016
19
IgG4-Related Sclerosing Cholangitis. ( 27466792 )
2016
20
HIDA Detects High Grade Biliary Obstruction Secondary to IgG4 IgG4-Related Sclerosing Cholangitis. ( 25857413 )
2015
21
Extensive Metastatic Cholangiocarcinoma Associated With IgG4-Related Sclerosing Cholangitis Misdiagnosed as Isolated IgG4-Related Sclerosing Cholangitis: A Case Report and Literature Review. ( 26559312 )
2015
22
IgG4-related sclerosing cholangitis in the absence of autoimmune pancreatitis mimicking extrahepatic cholangiocarcinoma. ( 25635498 )
2015
23
Isolated intrapancreatic IgG4-related sclerosing cholangitis. ( 25632210 )
2015
24
Comparison of intraductal ultrasonography findings between primary sclerosing cholangitis and IgG4-related sclerosing cholangitis. ( 25594435 )
2015
25
Assessment in steroid trial for IgG4-related sclerosing cholangitis. ( 25863870 )
2015
26
Pericardiobiliary Fistulation: A Rare Complication of Therapeutic ERCP in a Patient With IgG4-Related Sclerosing Cholangitis. ( 26203452 )
2015
27
Isolated IgG4-related sclerosing cholangitis: a report of 9 cases. ( 24890945 )
2014
28
Nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan. ( 24353071 )
2014
29
IgG4-related sclerosing cholangitis without obvious pancreatic lesion: difficulty in differential diagnosis. ( 24690458 )
2014
30
Type 1 Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis Is Associated With Extrapancreatic Organ Failure, Malignancy, and Mortality in a Prospective UK Cohort. ( 25155229 )
2014
31
First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia. ( 25024635 )
2014
32
Overlap of IgG4-related sclerosing cholangitis and primary biliary cirrhosis. ( 24990335 )
2014
33
Clinical observations in hepatology: IgG4 related sclerosing cholangitis. ( 25179687 )
2014
34
Intrapancreatic biliary stricture in autoimmune pancreatitis should not be included in IgG4-related sclerosing cholangitis. ( 25207660 )
2014
35
Reply to intrapancreatic biliary stricture in autoimmune pancreatitis should not be included in IgG4-related sclerosing cholangitis. ( 25207661 )
2014
36
Difference from bile duct cancer and relationship between bile duct wall thickness and serum IgG/IgG4 levels in IgG4-related sclerosing cholangitis. ( 25713877 )
2014
37
Relapse of IgG4-related sclerosing cholangitis after steroid therapy: image findings and risk factors. ( 24573568 )
2014
38
Establishment of a serum IgG4 cut-off value for the differential diagnosis of IgG4-related sclerosing cholangitis: a Japanese cohort. ( 23621484 )
2013
39
Diagnosis of IgG4-related sclerosing cholangitis. ( 24282356 )
2013
40
IgG4-related sclerosing cholangitis: the cholangiocarcinoma mimic. ( 24164754 )
2013
41
The role of peroral video cholangioscopy in patients with IgG4-related sclerosing cholangitis. ( 22948487 )
2013
42
Possible involvement of CCL1-CCR8 interaction in lymphocytic recruitment in IgG4-related sclerosing cholangitis. ( 23811304 )
2013
43
Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. ( 22717980 )
2012
44
Case of IgG4-related sclerosing cholangitis with a normal serum IgG4 level: report of a case. ( 23123394 )
2012
45
IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma. ( 22965631 )
2012
46
Usefulness of steroid administration for diagnosis of IgG4-related sclerosing cholangitis. ( 23343338 )
2012
47
A case of IgG4-related sclerosing cholangitis overlapped with primary biliary cirrhosis. ( 22790128 )
2012
48
Usefulness of Intraductal Ultrasonography in the Diagnosis of Cholangiocarcinoma and IgG4-Related Sclerosing Cholangitis. ( 22977830 )
2012
49
A case of IgG4-related sclerosing cholangitis mimicking an intrahepatic cholangiocellular carcinoma. ( 22291208 )
2012
50
Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification. ( 21947649 )
2012

Variations for Igg4-Related Sclerosing Cholangitis

Expression for Igg4-Related Sclerosing Cholangitis

Search GEO for disease gene expression data for Igg4-Related Sclerosing Cholangitis.

Pathways for Igg4-Related Sclerosing Cholangitis

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.32 CCL1 CCR8
2
Show member pathways
11.91 CCL1 CCR8
3
Show member pathways
11.06 CCL1 CCR8
4
Show member pathways
10.69 CCL1 CCR8

GO Terms for Igg4-Related Sclerosing Cholangitis

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

# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.32 CCL1 CCR8
2 immune response GO:0006955 9.26 CCL1 CCR8
3 chemotaxis GO:0006935 9.16 CCL1 CCR8
4 positive regulation of cytosolic calcium ion concentration GO:0007204 8.96 CCL1 CCR8
5 chemokine-mediated signaling pathway GO:0070098 8.62 CCL1 CCR8

Sources for Igg4-Related Sclerosing Cholangitis

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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