MCID: IGG014
MIFTS: 23

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 58

Characteristics:

Orphanet epidemiological data:

58
igg4-related sclerosing cholangitis
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare hepatic diseases
Rare systemic and rhumatological diseases


External Ids:

ICD10 via Orphanet 33 K83.0
Orphanet 58 ORPHA447764

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. Affiliated tissues include pancreas, liver and myeloid.

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 35)
# Related Disease Score Top Affiliating Genes
1 sclerosing cholangitis 11.1
2 cholangitis 11.1
3 cholangiocarcinoma 10.9
4 intrahepatic cholangiocarcinoma 10.9
5 autoimmune pancreatitis 10.9
6 cholangitis, primary sclerosing 10.8
7 pancreatitis 10.7
8 igg4-related disease 10.7
9 obstructive jaundice 10.7
10 primary biliary cholangitis 10.4
11 bile duct cancer 10.4
12 liver cirrhosis 10.4
13 retroperitoneal fibrosis 10.4
14 autoimmune disease 10.3
15 anemia, autoimmune hemolytic 10.3
16 deficiency anemia 10.3
17 cholecystitis 10.3
18 autoimmune hepatitis 10.3
19 klatskin's tumor 10.3
20 hemolytic anemia 10.3
21 myasthenia gravis 10.1
22 gallbladder disease 1 10.1
23 sialadenitis 10.1
24 portal hypertension 10.1
25 respiratory failure 10.1
26 choledocholithiasis 10.1
27 cholestasis 10.1
28 pericholangitis 10.1
29 neuroma 10.1
30 ischemia 10.1
31 liver disease 10.1
32 ileus 10.1
33 chromosomal triplication 10.1
34 dysphagia 10.1
35 secondary sclerosing cholangitis 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

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Bile Usefulness for Detecting IgG4-related Sclerosing Cholangitis Recruiting NCT02616705

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:

40
Pancreas, Liver, Myeloid, T Cells

Publications for Igg4-Related Sclerosing Cholangitis

Articles related to Igg4-Related Sclerosing Cholangitis:

(show top 50) (show all 167)
# Title Authors PMID Year
1
IgG4-related Sclerosing Cholangitis Complicated with Cholangiocarcinoma and Detected by Forkhead Box P3 Immunohistochemical Staining. 61
33087673 2021
2
Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort. 61
33664004 2021
3
Clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020: (Revision of the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012). 61
33586343 2021
4
False-positive PET/CT finding of hepatic inflammatory pseudotumor in IgG4-related sclerosing cholangitis. 61
33582317 2021
5
Immunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report. 61
33511195 2021
6
Role of Endoscopic Procedures in the Diagnosis of IgG4-Related Pancreatobiliary Disease. 61
33537218 2021
7
IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma. 61
33776680 2021
8
IgG4-related sclerosing cholangitis- A great mimicker. 61
33156480 2020
9
IgG4-related autoimmune liver disease. 61
33267565 2020
10
Risks and characteristics of pancreatic cancer and pancreatic relapse in autoimmune pancreatitis patients. 61
32583452 2020
11
The Diagnosis of Autoimmune Pancreatitis Using Endoscopic Ultrasonography. 61
33255660 2020
12
Usefulness of ultrasonography to assess the response to steroidal therapy for the rare case of type 2b immunoglobulin G4-related sclerosing cholangitis without pancreatitis: A case report. 61
33344580 2020
13
Isolated IgG4-related sclerosing cholangitis with normal serum IgG4 levels-A case report. 61
33235755 2020
14
Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review. 61
33025818 2020
15
Cholangioscopy findings in IgG4-related sclerosing cholangitis. 61
33032352 2020
16
IgG4-related sclerosing cholangitis presenting as an isolated intrahepatic stenosis: a rare presentation of a rare disease. 61
32916720 2020
17
Differential Diagnosis of IgG4-Related Sclerosing Cholangitis. 61
32749881 2020
18
Reply to "Differential Diagnosis of IgG4-Related Sclerosing Cholangitis". 61
32749887 2020
19
Usefulness of peroral cholangioscopy in the differential diagnosis of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma: a single-center retrospective study. 61
32831026 2020
20
Klatskin tumor diagnosed concurrently with IgG4 related sclerosing cholangitis: A case report. 61
32846862 2020
21
Review of primary sclerosing cholangitis with increased IgG4 levels. 61
32684731 2020
22
[Recent Updates of Immunoglobulin G4-related Pancreatobiliary Disease]. 61
32448857 2020
23
Role of Peroral Cholangioscopy in the Diagnosis of Primary Sclerosing Cholangitis. 61
32365686 2020
24
Animal Models of Autoimmune Liver Diseases: a Comprehensive Review. 61
32076943 2020
25
IgG4-related Sclerosing Cholangitis Mimicking Cholangiocarcinoma Diagnosed by Endoscopic Ultrasound-guided Fine-needle Aspiration. 61
31875638 2020
26
A man in his fifties with abdominal pain, itching and weight loss. 61
32105028 2020
27
Peribiliary glands: development, dysfunction, related conditions and imaging findings. 61
31707436 2020
28
Challenging biliary strictures: pathophysiological features, differential diagnosis, diagnostic algorithms, and new clinically relevant biomarkers - part 1. 61
32595761 2020
29
Management of biliary stricture in patients with IgG4-related sclerosing cholangitis. 61
32353060 2020
30
IgG4-Related Sclerosing Cholangitis: A Clinical and Imaging Review. 61
31509439 2019
31
Validity and safety of endoscopic biliary stenting for biliary stricture associated with IgG4-related pancreatobiliary disease during steroid therapy. 61
31673612 2019
32
Benign hilar bile duct strictures resected as perihilar cholangiocarcinoma. 61
31386198 2019
33
IgG4-Producing MALT Lymphoma in the Renal Hilum. 61
31911772 2019
34
Diagnosis and management of IgG4-related disease. 61
31288262 2019
35
Correction: Activated T-Follicular Helper 2 Cells Are Associated With Disease Activity in IgG4-Related Sclerosing Cholangitis and Pancreatitis. 61
31313691 2019
36
[Paying attention to the diagnosis and treatment of IgG4-related sclerosing cholangitis]. 61
31269612 2019
37
Correction: Activated T-Follicular Helper 2 Cells Are Associated With Disease Activity in IgG4-Related Sclerosing Cholangitis and Pancreatitis. 61
31082857 2019
38
A rare case of localized IgG4-related sclerosing cholecystitis mimicking gallbladder cancer. 61
31191779 2019
39
IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis. 61
30205418 2019
40
Activated T-Follicular Helper 2 Cells Are Associated With Disease Activity in IgG4-Related Sclerosing Cholangitis and Pancreatitis. 61
31033594 2019
41
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients. 61
30701685 2019
42
A Curious Case of Autoimmune Pancreatitis with IgG4-related Sclerosing Cholangitis. 61
31058036 2019
43
[Diagnosis of extra pancreatic lesions (focused on IgG4-related sclerosing cholangitis)]. 61
30971666 2019
44
Clinical practice guidelines for IgG4-related sclerosing cholangitis. 61
30575336 2019
45
[Intraductal papillary neoplasm of bile duct developed in a patient with IgG4-related sclerosing cholangitis, autoimmune pancreatitis, and myasthenia gravis]. 61
31080225 2019
46
[Clinical guidelines for IgG4-related sclerosing cholangitis]. 61
31406069 2019
47
The Immunobiology of Receptor Activator for Nuclear Factor Kappa B Ligand and Myeloid-Derived Suppressor Cell Activation in Immunoglobulin G4-Related Sclerosing Cholangitis. 61
29774578 2018
48
Immunoglobulin G4-related sclerosing cholangitis. 61
30045615 2018
49
A good response to steroid therapy in IgG4-related sclerosing cholangitis: a case report. 61
30324147 2018
50
Advances in IgG4-related pancreatobiliary diseases. 61
30047448 2018

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
12.22 CCR8 CCL1
2
Show member pathways
11.71 CCR8 CCL1
3
Show member pathways
10.96 CCR8 CCL1

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 CCR8 CCL1
2 immune response GO:0006955 9.26 CCR8 CCL1
3 positive regulation of cytosolic calcium ion concentration GO:0007204 9.16 CCR8 CCL1
4 chemotaxis GO:0006935 8.96 CCR8 CCL1
5 chemokine-mediated signaling pathway GO:0070098 8.62 CCR8 CCL1

Sources for Igg4-Related Sclerosing Cholangitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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