Sclerosing Cholangitis disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: SCL009 MIFTS: 46	Sclerosing Cholangitis Categories: Gastrointestinal diseases, Liver diseases, Rare diseases	Data Licensing For inquiries, contact: [email protected]
Genes Tissues Related diseases Publications Symptoms & Phenotypes Drugs Expand all tables

Sources

Aliases & Classifications for Sclerosing Cholangitis

MalaCards integrated aliases for Sclerosing Cholangitis:

Name: Sclerosing Cholangitis ¹¹ ⁵⁸ ⁷⁵ ¹⁴ ¹⁶

Primary Sclerosing Cholangitis ⁷¹

Cholangitis, Sclerosing ⁷¹

Fibrosing Cholangitis ¹¹

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Gastrointestinal diseases Liver diseases

See all MalaCards categories (disease lists)

ICD11: ³³

Diseases of the digestive system

Diseases of liver

Autoimmune liver disease

Primary sclerosing cholangitis

Other specified primary sclerosing cholangitis

Primary sclerosing cholangitis with cirrhosis

Primary sclerosing cholangitis, unspecified

Orphanet: ⁵⁸

Rare hepatic diseases

External Ids:

Disease Ontology ¹¹ DOID:14268

MeSH ⁴³ D015209

SNOMED-CT ⁶⁸ 4032000

UMLS via Orphanet ⁷² C0008313

Orphanet ⁵⁸ ORPHA447771

EFO ¹⁶ EFO_0004268

ICD11 ³³ 1929388030 857962451/other 857962451/unspecified

UMLS ⁷¹ C0008313 C0566602

Sources

Summaries for Sclerosing Cholangitis

MalaCards based summary: Sclerosing Cholangitis, also known as primary sclerosing cholangitis, is related to sclerosing cholangitis, neonatal and cholangitis, and has symptoms including icterus An important gene associated with Sclerosing Cholangitis is CLDN1 (Claudin 1), and among its related pathways/superpathways are Metabolism of steroids and Synthesis of bile acids and bile salts. The drugs Bezafibrate and Simvastatin have been mentioned in the context of this disorder. Affiliated tissues include liver, skin and t cells, and related phenotype is liver/biliary system.

Wikipedia: ⁷⁵ Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder... more...

Sources

Related Diseases for Sclerosing Cholangitis

Diseases in the Sclerosing Cholangitis family:

Cholangitis, Primary Sclerosing

Secondary Sclerosing Cholangitis

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

(show top 50) (show all 787)

#	Related Disease	Score	Top Affiliating Genes
1	sclerosing cholangitis, neonatal	33.2	DCDC2 CLDN1
2	cholangitis	32.6	NR1H4 HP GPT GPBAR1 DCDC2 CLDN1
3	cholangitis, primary sclerosing	32.6	SLC10A2 SEPSECS NR1H4 MIRLET7I MIR222 MIR199A1
4	cholestasis	32.1	SLC10A2 NR1H4 GPT CYP7A1 ABCB4 ABCB11
5	autoimmune hepatitis	31.9	SEPSECS GPT ALB
6	obstructive jaundice	31.8	GPT ALB ABCB11
7	crohn's disease	31.8	ICOSLG HP CCR6 ALB
8	portal hypertension	31.7	NR1H4 MIR199A1 GPT CCR6 ALB
9	liver disease	31.5	SLC10A2 SEPSECS NR1H4 KRT19 GPT ALB
10	klatskin's tumor	31.5	KRT19 GPT ALB
11	bile duct cancer	31.4	MIR199A1 KRT19 GPT ALB
12	cholecystitis	31.4	HP GPT ALB ABCB4
13	choledocholithiasis	31.3	GPT ALB ABCB4 ABCB11
14	anemia, autoimmune hemolytic	31.3	ICOSLG HP CCR6
15	acute cholangitis	31.3	GPT ALB ABCB4
16	ascending cholangitis	31.3	GPT ALB ABCB4
17	esophageal varix	31.3	HP GPT ALB
18	autoimmune cholangitis	31.3	SEPSECS CCR6 ABCB4
19	primary biliary cholangitis	31.2	SLC10A2 SEPSECS NR1H4 MIR199A1 KRT19 ICOSLG
20	celiac disease 1	31.2	ICOSLG HP GPT CCR6 ALB
21	gallbladder cancer	31.2	MIR199A1 KRT19 GPT ALB
22	severe covid-19	31.2	GPT CCR6 ALB
23	viral hepatitis	31.1	SEPSECS KRT19 GPT ALB
24	pericholangitis	31.1	SLC10A2 NR1H4 GPT CYP7A1 ABCB4 ABCB11
25	acalculous cholecystitis	31.1	GPT ALB
26	deficiency anemia	31.0	ICOSLG HP GPT CCR6 ALB
27	colonic disease	31.0	MIR222 MIR199A1 ICOSLG CCR6
28	intrahepatic cholangiocarcinoma	31.0	MIR199A1 KRT19 ALB
29	chronic cholangitis	31.0	GPT ABCB4
30	bone disease	31.0	MIR222 MIR199A1 ICOSLG CCR6
31	viral infectious disease	31.0	MIR199A1 ICOSLG GPT CCR6
32	bile duct cysts	30.9	KRT19 GPT ALB ABCB4 ABCB11
33	intrahepatic cholestasis	30.9	SLC10A2 NR1H4 GPT ALB ABCB4 ABCB11
34	spondyloarthropathy 1	30.9	ICOSLG HP CCR6
35	gallbladder disease	30.9	NR1H4 MIR222 MIR199A1 GPT CCR6 ALB
36	respiratory failure	30.9	MIR199A1 GPT CCR6 ALB
37	cholelithiasis	30.9	NR1H4 HP GPT CYP7A1 ALB ABCB4
38	biliary atresia	30.8	SLC10A2 NR1H4 GPT ALB ABCB4 ABCB11
39	hypercholesterolemia, familial, 1	30.8	SLC10A2 NR1H4 GPT CYP7A1
40	bilirubin metabolic disorder	30.8	SLC10A2 NR1H4 HP GPT CYP7A1 ALB
41	intestinal disease	30.8	MIRLET7I MIR222 MIR199A1 ICOSLG CCR6
42	acute kidney failure	30.8	HP GPT ALB
43	suppurative cholangitis	30.7	SEPSECS GPT ALB ABCB4
44	immunoglobulin alpha deficiency	30.7	ICOSLG HP CCR6
45	hemochromatosis, type 1	30.6	MIR199A1 HP GPT ALB
46	non-alcoholic steatohepatitis	30.6	NR1H4 MIR199A1 GPT GPBAR1 CYP7A1 CCR6
47	alpha-1-antitrypsin deficiency	30.6	SEPSECS GPT ALB
48	exanthem	30.6	ICOSLG GPT CCR6 ALB
49	lymphatic system disease	30.5	MIRLET7I MIR222 MIR199A1 ICOSLG CCR6
50	extrahepatic cholestasis	30.5	SLC10A2 NR1H4 GPT CYP7A1 ABCB4 ABCB11

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

Sources

Symptoms & Phenotypes for Sclerosing Cholangitis

UMLS symptoms related to Sclerosing Cholangitis:

icterus

MGI Mouse Phenotypes related to Sclerosing Cholangitis:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	liver/biliary system	MP:0005370	9.28	ABCB11 ABCB4 ALB CYP7A1 GPBAR1 HP

Sources

Drugs & Therapeutics for Sclerosing Cholangitis

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

(show top 50) (show all 75)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Bezafibrate

Approved, Investigational

Phase 3

41859-67-0

39042

Simvastatin

Approved

Phase 3

79902-63-9

54454

Ursodeoxycholic acid

Approved, Investigational

Phase 2, Phase 3

128-13-2

31401

Clofibric Acid

Phase 3

882-09-7

2797

Anticholesteremic Agents

Phase 3

Hydroxymethylglutaryl-CoA Reductase Inhibitors

Phase 3

Mitomycin

Approved

Phase 2

50-07-7

5746

Curcumin

Approved, Investigational

Phase 1, Phase 2

458-37-7, 84765-67-3

969516

Cladribine

Approved, Investigational

Phase 2

4291-63-8

20279

Aldesleukin

Approved

Phase 2

110942-02-4

Sulfasalazine

Approved

Phase 2

599-79-1

5353980 5339 5359476

Thalidomide

Approved, Investigational, Withdrawn

Phase 2

50-35-1

5426

Fenofibrate

Approved

Phase 1, Phase 2

49562-28-9

3339

Isotretinoin

Approved, Investigational, Nutraceutical

Phase 2

302-79-4, 4759-48-2

5538 444795 5282379

Cenicriviroc

Investigational

Phase 2

497223-25-3

6320532 11285792

Seladelpar

Investigational

Phase 2

851528-79-5

11476297 11236126

Volixibat

Experimental, Investigational

Phase 2

1025216-57-2

Alkylating Agents

Phase 2

Hormones

Phase 2

Calcium, Dietary

Phase 2

Immunoglobulins

Phase 2

Antibodies

Phase 2

Antiviral Agents

Phase 2

Anti-Retroviral Agents

Phase 2

Anti-HIV Agents

Phase 2

Antibodies, Monoclonal

Phase 2

2-chloro-3'-deoxyadenosine

Phase 2

14485373

Interleukin-2

Phase 2

Analgesics

Phase 2

Antirheumatic Agents

Phase 2

Anti-Inflammatory Agents, Non-Steroidal

Phase 2

Analgesics, Non-Narcotic

Phase 2

Anti-Inflammatory Agents

Phase 2

Bile Acids and Salts

Phase 2

Antimetabolites

Phase 1, Phase 2

Hypolipidemic Agents

Phase 1, Phase 2

Lipid Regulating Agents

Phase 1, Phase 2

Keratolytic Agents

Phase 2

Dermatologic Agents

Phase 2

Angiogenesis Inhibitors

Phase 2

Immunosuppressive Agents

Phase 2

Immunologic Factors

Phase 2

Calcium

Nutraceutical

Phase 2

7440-70-2

271

Minocycline

Approved, Investigational

Phase 1

10118-90-8, 13614-98-7

54675783 5281021

Rifaximin

Approved, Investigational

Phase 1

80621-81-4

46783403 6436173

Erlotinib

Approved, Investigational

Phase 1

183319-69-9, 183321-74-6

176870

Budesonide

Approved

Phase 1

51333-22-3

5281004 40000 63006

Metronidazole

Approved

Phase 1

443-48-1, 69198-10-3

4173

Rituximab

Approved

Phase 1

174722-31-7

Lenalidomide

Approved

Phase 1

191732-72-6

216326

Interventional clinical trials:

(show top 50) (show all 114)

#	Name	Status	NCT ID	Phase	Drugs
1	Evaluation of Effectiveness and Safety of Oral Vancomycin in Treatment of Primary Sclerosing Cholangitis.	Unknown status	NCT02605213	Phase 4	Vancomycin;Placebo
2	The Effect of Bezafibrate on Cholestatic Itch	Unknown status	NCT02701166	Phase 3	Bezafibrate;Placebo
3	Probiotics in Patients With Primary Sclerosing Cholangitis and Inflammatory Bowel Disease- a Randomized Placebo-Controlled Cross-Over Trial	Unknown status	NCT00161148	Phase 3	Probiotics
4	Multicenter Randomized Trial Comparing Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis	Unknown status	NCT01398917	Phase 3
5	Treatment of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients With Oral Vancomycin by the Study of Its Antimicrobial and Immunomodulating Effects	Completed	NCT01802073	Phase 3	Oral Vancomycin
6	Digital SpyGlass Cholangioscopy Confirmed Common Bile Duct Stones Clearance Without Fluoroscopy	Completed	NCT02967926	Phase 3
7	Double-blind, Randomized, Placebo-controlled, Phase III Study Comparing norUrsodeoxycholic Acid Capsules With Placebo in the Treatment of Primary Sclerosing Cholangitis	Recruiting	NCT03872921	Phase 3	norUrsodeoxycholic Acid
8	Double Blind, Multicentric, Randomized, Placebo-controlled Trial, Evaluating the Efficacy of 24 Month of Bezafibrate in Primary Sclerosing Cholangitis With Persistent Cholestasis Despite Ursodeoxycholic Acid Therapy	Recruiting	NCT04309773	Phase 3	Bezafibrate (400mg) in addition to standard 15-20 mg/kg/jour UDCA therapy;Placebo of Bezafibrate in addition to standard UDCA therapy
9	A Prospective, Randomized, Multi-centered, Placebo-controlled Clinical Trial of Oral Vanycomycin in Adults With Primary Sclerosing Cholangitis	Recruiting	NCT03710122	Phase 2, Phase 3	Vancomycin
10	Effect of Simvastatin on the Prognosis of Primary Sclerosing Cholangitis (PSC); A Randomized, Double-blind, Placebo Controlled Multicenter Study	Recruiting	NCT04133792	Phase 3	Simvastatin 40mg;Placebo oral tablet
11	A Phase 3, Randomized, Double-Blind, Placebo-Controlled Study Evaluating the Safety, Tolerability, and Efficacy of Cilofexor in Non-Cirrhotic Subjects With Primary Sclerosing Cholangitis	Active, not recruiting	NCT03890120	Phase 3	Cilofexor;Placebo
12	Multicentered Randomized Trial of High-dose Urso in Primary Sclerosing Cholangitis	Terminated	NCT00059202	Phase 2, Phase 3	Ursodeoxycholic Acid;Placebo
13	A Randomized, Global, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Vedolizumab IV for the Treatment of Primary Sclerosing Cholangitis, With Underlying Inflammatory Bowel Disease	Withdrawn	NCT03035058	Phase 3	Vedolizumab;Placebo
14	Phase 2 Study of Mitomycin C Therapy for Biliary Strictures in Patients With Primary Sclerosing Cholangitis	Unknown status	NCT01688024	Phase 2	Mitomycin C;Normal saline
15	A Pilot, Open-Label Study to Evaluate the Safety, Tolerability and Efficacy of LUM001, an Apical Sodium-dependent Bile Acid Transporter Inhibitor (ASBTi), in Patients With Primary Sclerosing Cholangitis	Completed	NCT02061540	Phase 2	LUM001
16	A Phase 2, Randomized, Double Blind, Placebo Controlled, Parallel Group, Multiple Center Study to Evaluate the Safety, Tolerability, and Efficacy of NGM282 Administered for 12 Weeks in Patients With Primary Sclerosing Cholangitis	Completed	NCT02704364	Phase 2
17	Investigation of the Activity of Vidofludimus Calcium, a Novel, Orally Available, Small Molecule Inhibitor of Dihydroorotate Dehydrogenase, as a Treatment for Primary Sclerosing Cholangitis (PSC)	Completed	NCT03722576	Phase 2	Vidofludimus calcium
18	A Proof-of-Concept and Dose-Ranging Study Investigating the Efficacy and Safety of HTD1801 in Adult Subjects With Primary Sclerosing Cholangitis (PSC)	Completed	NCT03333928	Phase 2	HTD1801;Placebo
19	An Open-Label Pilot Study Evaluating the Safety and Efficacy of Curcumin in Patients With Primary Sclerosing Cholangitis	Completed	NCT02978339	Phase 1, Phase 2	Curcumin
20	PERSEUS: A Phase 2 Proof of Concept Study Investigating the Preliminary Efficacy and Safety of Cenicriviroc in Adult Subjects With Primary Sclerosing Cholangitis (PSC)	Completed	NCT02653625	Phase 2	Cenicriviroc 150 mg
21	A Phase 2, Randomized, Double Blind, Placebo Controlled, Multiple Center Study to Evaluate the Safety, Tolerability, and Efficacy of Seladelpar Administered for 24 Weeks in Adult Patients With Primary Sclerosing Cholangitis (PSC)	Completed	NCT04024813	Phase 2	Seladelpar;Placebo to match Seladelpar
22	A Phase 2b, Dose-Ranging, Randomized, Double-Blind, Placebo-Controlled Trial Evaluating the Safety and Efficacy of GS-6624, a Monoclonal Antibody Against Lysyl Oxidase Like 2 (LOXL2) in Subjects With Primary Sclerosing Cholangitis (PSC)	Completed	NCT01672853	Phase 2
23	Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis.	Completed	NCT02424175	Phase 1, Phase 2
24	Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis	Completed	NCT00004762	Phase 2	cladribine
25	A Single Arm, Two-stage, Multi-centre, Phase II Clinical Trial Investigating the Safety and Activity of the Use of BTT1023 Targeting Vascular Adhesion Protein (VAP-1), in the Treatment of Patients With Primary Sclerosing Cholangitis (PSC).	Completed	NCT02239211	Phase 2	BTT1023
26	A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Dose-Finding, Clinical Trial Evaluating the Efficacy and Safety of Obeticholic Acid in Subjects With Primary Sclerosing Cholangitis	Completed	NCT02177136	Phase 2	Obeticholic Acid (OCA);Placebo
27	A Phase 2, Randomized, Double-Blind, Placebo Controlled Study Evaluating the Safety, Tolerability, and Efficacy of GS-9674 in Subjects With Primary Sclerosing Cholangitis Without Cirrhosis	Completed	NCT02943460	Phase 2	Cilofexor;Placebo to match cilofexor
28	Double-blind,Randomized, Placebo-controlled, Phase II Dose-finding Study Comparing Different Doses of Norursodeoxycholic Acid Capsules With Placebo in the Treatment of Primary Sclerosing Cholangitis	Completed	NCT01755507	Phase 2	norUDCA;Placebo
29	Induction of Regulatory t Cells by Low Dose IL2 in Autoimmune and Inflammatory Diseases: a Transnosographic Approach	Completed	NCT01988506	Phase 2	Interleukin 2
30	A Randomized Double-Blind Placebo-Controlled Study to Evaluate the Efficacy and Safety of Volixibat in the Treatment of Cholestatic Pruritus in Patients With Primary Sclerosing Cholangitis	Recruiting	NCT04663308	Phase 2	Volixibat;Placebo
31	A Randomized, Double-blind, Dose-ranging, Placebo-controlled, Phase 2a Evaluation of the Safety, Tolerability, and Pharmacokinetics of PLN-74809 in Participants With Primary Sclerosing Cholangitis (PSC) and Suspected Liver Fibrosis (INTEGRIS-PSC)	Recruiting	NCT04480840	Phase 2	PLN-74809;Placebo
32	A Phase 2a, Randomized, Double-Blind, Placebo-Controlled, Clinical Trial Evaluating The Safety And Efficacy Of CM-101 In Subjects With Primary Sclerosing Cholangitis- The SPRING Study	Recruiting	NCT04595825	Phase 2
33	Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Effects of EP547 in Subjects With Cholestatic Pruritus Due to Primary Biliary Cholangitis or Primary Sclerosing Cholangitis	Recruiting	NCT05525520	Phase 2	EP547;Placebo
34	An Adaptive, Multicentre, Phase IIa, Multi-disease Trial Investigating the Safety & Activity of a Single Infusion of Selected Mesenchymal Stromal Cells in the Treatment of Patients With Primary Sclerosing Cholangitis & Autoimmune Hepatitis	Recruiting	NCT02997878	Phase 1, Phase 2
35	A Randomized, Placebo-controlled Pilot Study of Sulfasalazine for the Treatment of Primary Sclerosing Cholangitis (PSC)	Recruiting	NCT03561584	Phase 2	Sulfasalazine;Placebo
36	Intra-arterial Injection of Umbilical Cord Mesenchymal Stem Cells in Primary Sclerosing Cholangitis	Active, not recruiting	NCT03516006	Phase 1, Phase 2	UCMSC;UDCA
37	A Phase II, Multicenter, Double-Blind, Randomised, Placebo-Controlled Study and Open Label Long Term Extension to Evaluate the Safety and Efficacy of Elafibranor in Adult Participants With Primary Sclerosing Cholangitis (PSC).	Not yet recruiting	NCT05627362	Phase 2	Elafibranor 80 mg;Elafibranor 120 mg;Placebo Matched to Elafibranor 80 mg;Placebo Matched to Elafibranor 120 mg
38	An Open Label, Phase 2 Study to Evaluate the Effect of A3907 on Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics in Adults With Primary Sclerosing Cholangitis (PSC)	Not yet recruiting	NCT05642468	Phase 2	A3907
39	A Study of Oral Hymecromone to Treat Adolescents and Adults With Primary Sclerosing Cholangitis(HAAPS Study).	Not yet recruiting	NCT05295680	Phase 2	Hymecromone
40	An Efficacy Trial of Low Dose All-trans Retinoic Acid (ATRA) in Patients With Primary Sclerosing Cholangitis	Terminated	NCT03359174	Phase 2	All-trans retinoic acid
41	A Randomized, Open-label, Phase 2 Study to Evaluate Safety and Efficacy of DUR-928 in Subjects With Primary Sclerosing Cholangitis (PSC)	Terminated	NCT03394781	Phase 2	DUR-928
42	Open Label, Phase II Investigation of Thalidomide for the Treatment of Primary Sclerosing Cholangitis	Terminated	NCT00953615	Phase 2	Thalidomide
43	Pilot Study of Fenofibrate in Primary Sclerosing Cholangitis	Terminated	NCT01142323	Phase 1, Phase 2	fenofibrate
44	A Phase 2, Randomized, Double-Blind Study of HTD1801 vs Ursodeoxycholic Acid (UDCA) in Adolescents With Primary Sclerosing Cholangitis (PSC)	Withdrawn	NCT03678480	Phase 2	HTD1801;Ursodeoxycholic Acid
45	Primary Sclerosing Cholangitis: Value of Computerized Tomographic Cholangiography and Intraductal Volumetric Measurement in Determining the Prognosis	Withdrawn	NCT00588458	Phase 2
46	Open Label Pilot Study Evaluating Minocycline in Patients With Primary Sclerosing Cholangitis (PSC)	Completed	NCT00630942	Phase 1	Minocycline
47	A Pilot Study of Xifaxan in Patients With Primary Sclerosing Cholangitis	Completed	NCT01695174	Phase 1	Xifaxan
48	An Open Label Pilot Trial of Erlotinib (Tarceva) in Primary Sclerosing Cholangitis With Trisomy 7	Completed	NCT00955149	Phase 1	Erlotinib (Tarceva)
49	Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis	Completed	NCT00004842	Phase 1	budesonide
50	A Pilot Study to Characterize Bile Acid Metabolism and Dysbiosis in Primary Sclerosing Cholangitis	Completed	NCT02464020	Phase 1	Vancomycin

Search NIH Clinical Center for Sclerosing Cholangitis

Sources

Genetic Tests for Sclerosing Cholangitis

Sources

Anatomical Context for Sclerosing Cholangitis

Organs/tissues related to Sclerosing Cholangitis:

MalaCards : Liver, Skin, T Cells, Colon, Pancreas, Neutrophil, B Cells

Sources

Publications for Sclerosing Cholangitis

Articles related to Sclerosing Cholangitis:

(show top 50) (show all 6980)

#	Title	Authors	PMID	Year
1	Autoimmune conditions and pancreatic cancer risk in older American adults. ⁶²	Yuan F...Stolzenberg-Solomon RZ	36059225	2023
2	A Case of Successful Management of Adult-Onset Linear IgA Bullous Disease With Sulfasalazine During the COVID-19 Pandemic. ⁶²	Ashraf S...Tolland J	36468952	2022
3	Risks of colorectal cancer and biliary cancer according to accompanied primary sclerosing cholangitis in Korean patients with ulcerative colitis: a nationwide population-based study. ⁶²	Oh EH...Park SH	36453007	2022
4	Optimal timing of liver transplantation for liver cirrhosis caused by sclerosing cholangitis in a patient with Langerhans cell histiocytosis: a case report. ⁶²	Watakabe M...Koh K	36469185	2022
5	Development of a prognostic MRCP-score (DiStrict) for individuals with large-duct primary sclerosing cholangitis. ⁶²	Grigoriadis A...Bergquist A	36444388	2022
6	Low prevalence of primary sclerosing cholangitis in patients with inflammatory bowel disease in India. ⁶²	Singh A...Sood A	36453008	2022
7	Patient-Predicted Outcomes Are Associated with Quality of Life in Patients with Primary Sclerosing Cholangitis. ⁶²	Henson JB...Muir AJ	35347534	2022
8	Increased Risk of Non-conventional and Invisible Dysplasias in Patients with Primary Sclerosing Cholangitis and Inflammatory Bowel Disease. ⁶²	Zhang R...Choi WT	35771958	2022
9	Practical Guide for Radiological Diagnosis of Primary and Secondary Sclerosing Cholangitis. ⁶²	Pria HD...D'Ippolito G	36462808	2022
10	Inflammatory type 2 conventional dendritic cells contribute to murine and human cholangitis. ⁶²	Muller AL...Herkel J	35798133	2022
11	Inflammatory conditions play a role in recurrence of PSC after liver transplantation: An international multicentre study. ⁶²	Visseren T...Darwish Murad S	36426376	2022
12	Results of Intrahepatic Cholangiocarcinoma Resections: a Single-Center Analysis. ⁶²	Saglam K...Yilmaz S	34989985	2022
13	Fenofibrate Downregulates NF-κB Signaling to Inhibit Pro-inflammatory Cytokine Secretion in Human THP-1 Macrophages and During Primary Biliary Cholangitis. ⁶²	Gallucci GM...Ghonem NS	35838934	2022
14	Progressive cholestasis and associated sclerosing cholangitis are frequent complications of COVID-19 in patients with chronic liver disease. ⁶²	Hartl L...Trauner M	35596929	2022
15	Secondary sclerosing cholangitis after COVID-19 pneumonia: a report of two cases and review of the literature. ⁶²	Bauer U...Ehmer U	35953614	2022
16	COVID-19-associated secondary sclerosing cholangitis - A case series of 4 patients. ⁶²	Ferreira FB...Cardoso M	36347499	2022
17	IgG4-related sclerosing cholangitis involving the gallbladder mimicking a hilar cholangiocarcinoma. ⁶²	Lee YC...Choi KH	35272374	2022
18	Reducing relapse through maintenance steroid treatment can decrease the cancer risk in patients with IgG4-sclerosing cholangitis : based on a Japanese nationwide study. ⁶²	Kubota K...Collaborators	36403136	2022
19	Relationship Between Stages of Ileal Pouch-Anal Anastomosis, Timing of Restoration of Fecal Continuity, and Pouchitis. ⁶²	Sherrill GC...Barnes EL	35246803	2022
20	Waitlist mortality in patients with autoimmune liver diseases. ⁶²	Goyes D...Bonder A	35835366	2022
21	Loss of Apical Sodium Bile Acid Transporter Disrupts Bile Acid Circulation and Reduces Biliary Damage in Cholangitis. ⁶²	Meadows V...Francis H	36410025	2022
22	A common variant in the hepatobiliary phospholipid transporter ABCB4 modulates liver injury in PBC but not in PSC: prospective analysis in 867 patients. ⁶²	Kruk B...Krawczyk M	36397154	2022
23	Recent advances in surveillance colonoscopy for dysplasia in inflammatory bowel disease. ⁶²	Na SY...Moon W	36397275	2022
24	Gut-liver axis: Pathophysiological concepts and clinical implications. ⁶²	Tilg H...Trauner M	36208625	2022
25	Lack of EGFR catalytic activity in hepatocytes improves liver regeneration following DDC-induced cholestatic injury by promoting a pro-restorative inflammatory response. ⁶²	Lazcanoiturburu N...Sanchez A	36148647	2022
26	Hepatic dry copper weight in paediatric autoimmune liver disease. ⁶²	Nayagam JS...Samyn M	36332083	2022
27	Genetic link between rheumatoid arthritis and autoimmune liver diseases: A two-sample Mendelian randomization study. ⁶²	Fan J...He D	36446255	2022
28	Chemoprevention of Colitis-Associated Dysplasia or Cancer in Inflammatory Bowel Disease. ⁶²	Hsiao SW...Chen YY	35670121	2022
29	Microbial manipulation as therapy for primary sclerosing cholangitis. ⁶²	Bogatic D...Costello SP	36324251	2022
30	A Randomized, Dose-Finding, Proof-of-Concept Study of Berberine Ursodeoxycholate in Patients With Primary Sclerosing Cholangitis. ⁶²	Kowdley KV...Hirschfield GM	36327436	2022
31	A quantitative MRCP-derived score for medium-term outcome prediction in primary sclerosing cholangitis. ⁶²	Cristoferi L...Carbone M	36357293	2022
32	Adenosquamous Carcinoma of Extrahepatic Bile Duct in Primary Sclerosing Cholangitis. ⁶²	Chiang TH...Desai CS	36028730	2022
33	Survey uncovering variations in the management of primary sclerosing cholangitis across Europe. ⁶²	Eliasson J...Ytting H	36164416	2022
34	Disease burden in primary sclerosing cholangitis in the Netherlands: A long-term follow-up study. ⁶²	van Munster KN...EpiPSC2 Study Group	36328957	2022
35	The microbiota and the gut-liver axis in primary sclerosing cholangitis. ⁶²	Hov JR...Karlsen TH	36352157	2022
36	Algebraic topology-based machine learning using MRI predicts outcomes in primary sclerosing cholangitis. ⁶²	Singh Y...Erickson BJ	36396865	2022
37	The time is ripe for a randomized controlled trial of oral vancomycin in Primary Sclerosing Cholangitis. ⁶²	Halma JC...Carbone M	36442726	2022
38	Cholangiocarcinoma cell proliferation is enhanced in primary sclerosing cholangitis: A role for IL-17A. ⁶²	Lieshout R...de Vries AC	36346104	2022
39	PR3-ANCAs Detected by Third-Generation ELISA Predicts Severe Disease and Poor Survival in Primary Sclerosing Cholangitis. ⁶²	Lopens S...Milkiewicz P	36359524	2022
40	Prospective surveillance for cholangiocarcinoma in unselected individuals with primary sclerosing cholangitis. ⁶²	Villard C...Bergquist A	36410555	2022
41	Quantitative magnetic resonance cholangiopancreatography metrics are associated with disease severity and outcomes in people with primary sclerosing cholangitis. ⁶²	Cazzagon N...Arrive L	36277957	2022
42	Mesenchymal stem cells: A novel treatment option for primary sclerosing cholangitis. ⁶²	Lightner AL...Matthew Walsh R	36321586	2022
43	Fever of unknown origin due to Coxiella burnetii, an unexpected guest in primary sclerosing cholangitis. ⁶²	Cerrito L...Miele L	36371385	2022
44	Good outcomes of living donor liver transplant in primary sclerosing cholangitis: an experience from North India. ⁶²	Jadaun SS...Saigal S	36376772	2022
45	Ursodeoxycholic acid is associated with improved long-term outcome in patients with primary sclerosing cholangitis. ⁶²	Arizumi T...Japan PSC Study Group (JPSCSG)	36068441	2022
46	Genetic alterations during the neoplastic cascade towards cholangiocarcinoma in primary sclerosing cholangitis. ⁶²	Kamp EJ...de Vries AC	35897137	2022
47	Suppression of bile acid synthesis as a tipping point in the disease course of primary sclerosing cholangitis. ⁶²	Braadland PR...Marschall HU	36176935	2022
48	Berberine Ursodeoxycholate for the Treatment of Primary Sclerosing Cholangitis: The Search for the Elusive Pharmacologic Holy Grail Will Need to Continue. ⁶²	Yoshida EM	36327435	2022
49	Safety and efficacy of switching to pemafibrate from bezafibrate in patients with chronic liver disease. ⁶²	Tamai H...Okamura J	36378065	2022
50	Symptom Classes in Decompensated Liver Disease. ⁶²	Hansen L...Lee CS	34813941	2022

Sources

Genes for Sclerosing Cholangitis

Genes related to Sclerosing Cholangitis (0 elite genes):

(show all 41)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence
1	CLDN1	Claudin 1	Protein Coding	33.5	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders Publications Summaries (show sections)
2	DCDC2	Doublecortin Domain Containing 2	Protein Coding	22.59	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders Publications (show sections)
3	HP	Haptoglobin	Protein Coding	13.26	DISEASES inferred ¹⁴ GeneCards inferred via : Summaries (show sections)
4	PPM1F	Protein Phosphatase, Mg2+/Mn2+ Dependent 1F	Protein Coding	11.17	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders (show sections)
5	CCR6	C-C Motif Chemokine Receptor 6	Protein Coding	10.19	DISEASES inferred ¹⁴ ¹⁴
6	ABCB4	ATP Binding Cassette Subfamily B Member 4	Protein Coding	8.28	DISEASES inferred ¹⁴
7	GPT	Glutamic--Pyruvic Transaminase	Protein Coding	7.49	DISEASES inferred ¹⁴
8	NR1H4	Nuclear Receptor Subfamily 1 Group H Member 4	Protein Coding	7.43	DISEASES inferred ¹⁴
9	SEPSECS	Sep (O-Phosphoserine) TRNA:Sec (Selenocysteine) TRNA Synthase	Protein Coding	7.04	DISEASES inferred ¹⁴
10	ICOSLG	Inducible T Cell Costimulator Ligand	Protein Coding	7.02	DISEASES inferred ¹⁴ ¹⁴
11	ABCB11	ATP Binding Cassette Subfamily B Member 11	Protein Coding	7.01	DISEASES inferred ¹⁴
12	GPBAR1	G Protein-Coupled Bile Acid Receptor 1	Protein Coding	6.98	DISEASES inferred ¹⁴
13	ALB	Albumin	Protein Coding	6.83	DISEASES inferred ¹⁴
14	MIR222	MicroRNA 222	RNA Gene	6.82	DISEASES inferred ¹⁴ ¹⁴
15	KRT19	Keratin 19	Protein Coding	6.79	DISEASES inferred ¹⁴
16	SLC10A2	Solute Carrier Family 10 Member 2	Protein Coding	6.77	DISEASES inferred ¹⁴
17	MIR191	MicroRNA 191	RNA Gene	6.74	DISEASES inferred ¹⁴ ¹⁴
18	CYP7A1	Cytochrome P450 Family 7 Subfamily A Member 1	Protein Coding	6.73	DISEASES inferred ¹⁴
19	MIR199A1	MicroRNA 199a-1	RNA Gene	6.68	DISEASES inferred ¹⁴ ¹⁴
20	MIRLET7I	MicroRNA Let-7i	RNA Gene	6.66	DISEASES inferred ¹⁴ ¹⁴
21	TNF	Tumor Necrosis Factor	Protein Coding	6.65	DISEASES inferred ¹⁴
22	DLAT	Dihydrolipoamide S-Acetyltransferase	Protein Coding	6.6	DISEASES inferred ¹⁴
23	CD4	CD4 Molecule	Protein Coding	6.57	DISEASES inferred ¹⁴
24	DNAL1	Dynein Axonemal Light Chain 1	Protein Coding	6.45	DISEASES inferred ¹⁴
25	MADCAM1	Mucosal Vascular Addressin Cell Adhesion Molecule 1	Protein Coding	6.44	DISEASES inferred ¹⁴
26	IL6	Interleukin 6	Protein Coding	6.43	DISEASES inferred ¹⁴
27	NUP210	Nucleoporin 210	Protein Coding	6.4	DISEASES inferred ¹⁴
28	SLC10A1	Solute Carrier Family 10 Member 1	Protein Coding	6.37	DISEASES inferred ¹⁴
29	F2	Coagulation Factor II, Thrombin	Protein Coding	6.35	DISEASES inferred ¹⁴
30	FGF19	Fibroblast Growth Factor 19	Protein Coding	6.32	DISEASES inferred ¹⁴
31	NISCH	Nischarin	Protein Coding	6.31	DISEASES inferred ¹⁴
32	CD8A	CD8a Molecule	Protein Coding	6.31	DISEASES inferred ¹⁴
33	TNFRSF25	TNF Receptor Superfamily Member 25	Protein Coding	6.26	DISEASES inferred ¹⁴
34	AFP	Alpha Fetoprotein	Protein Coding	6.22	DISEASES inferred ¹⁴
35	SLC51A	Solute Carrier Family 51 Subunit Alpha	Protein Coding	6.14	DISEASES inferred ¹⁴
36	SP100	SP100 Nuclear Antigen	Protein Coding	6.1	DISEASES inferred ¹⁴
37	IL10	Interleukin 10	Protein Coding	6.1	DISEASES inferred ¹⁴
38	SERPINA1	Serpin Family A Member 1	Protein Coding	6.1	DISEASES inferred ¹⁴
39	NR1I2	Nuclear Receptor Subfamily 1 Group I Member 2	Protein Coding	6.1	DISEASES inferred ¹⁴
40	CRP	C-Reactive Protein	Protein Coding	6.09	DISEASES inferred ¹⁴
41	IL17A	Interleukin 17A	Protein Coding	6.08	DISEASES inferred ¹⁴

Sources

Variations for Sclerosing Cholangitis

Sources

Expression for Sclerosing Cholangitis

Search GEO for disease gene expression data for Sclerosing Cholangitis.

Sources

Pathways for Sclerosing Cholangitis

Pathways related to Sclerosing Cholangitis according to GeneCards Suite gene sharing:

#	Super pathways	Score	Top Affiliating Genes
1	Metabolism of steroids ⁶⁶ Show member pathways Activation of gene expression by SREBF (SREBP) ⁶⁶ allopregnanolone biosynthesis ⁶¹ androgen biosynthesis ⁶¹ Cholesterol synthesis disorders ⁷⁴ Defective CYP17A1 causes AH5 ⁶⁶ Regulation of cholesterol biosynthesis by SREBP (SREBF) ⁶⁶ Sterol regulatory element-binding proteins (SREBP) signaling ⁷⁴ Vitamin D (calciferol) metabolism ⁶⁶	12.36	SLC10A2 NR1H4 CYP7A1 ALB ABCB11
2	Synthesis of bile acids and bile salts ⁶⁶ Show member pathways Bile acid and bile salt metabolism ⁶⁶ Defective ABCB4 causes PFIC3, ICP3 and GBD1 ⁶⁶ Defective CYP7B1 causes SPG5A and CBAS3 ⁶⁶ Disorders of bile acid synthesis and biliary transport ⁷⁴ Recycling of bile acids and salts ⁶⁶ Synthesis of bile acids and bile salts via 24-hydroxycholesterol ⁶⁶ Synthesis of bile acids and bile salts via 27-hydroxycholesterol ⁶⁶ Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol ⁶⁶	11.66	SLC10A2 NR1H4 CYP7A1 ALB ABCB4 ABCB11
3	Nuclear receptors in lipid metabolism and toxicity ⁷⁴	11	NR1H4 CYP7A1 ABCB4 ABCB11
4	Cholestasis ⁷⁴	10.8	NR1H4 ABCB4 ABCB11
5	Drug induction of bile acid pathway ⁷⁴	10.73	NR1H4 CYP7A1 ABCB11
6	Bile acids synthesis and enterohepatic circulation ⁷⁴	10.51	SLC10A2 CYP7A1 ABCB11
7	Farnesoid X receptor pathway ⁷⁴	10.2	NR1H4 CYP7A1 ABCB4 ABCB11

Sources

GO Terms for Sclerosing Cholangitis

Cellular components related to Sclerosing Cholangitis according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	intercellular canaliculus	GO:0046581	8.92	ABCB4 ABCB11

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

#	Name	GO ID	Score	Top Affiliating Genes
1	bile acid and bile salt transport	GO:0015721	9.63	SLC10A2 CYP7A1 ABCB11
2	regulation of bile acid biosynthetic process	GO:0070857	9.56	NR1H4 CYP7A1
3	bile acid signaling pathway	GO:0038183	9.43	NR1H4 CYP7A1 ABCB11
4	cellular response to bile acid	GO:1903413	9.1	NR1H4 GPBAR1 ABCB4

Molecular functions related to Sclerosing Cholangitis according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	mRNA base-pairing translational repressor activity	GO:1903231	9.26	MIRLET7I MIR222 MIR199A1 MIR191
2	bile acid receptor activity	GO:0038181	8.92	NR1H4 GPBAR1