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

MCID: SCL009 MIFTS: 48	Sclerosing Cholangitis Categories: Gastrointestinal diseases, Liver diseases, Rare diseases
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Aliases & Classifications for Sclerosing Cholangitis

MalaCards integrated aliases for Sclerosing Cholangitis:

Name: Sclerosing Cholangitis ¹² ⁷⁴ ⁵⁸ ¹⁵ ¹⁷

Primary Sclerosing Cholangitis ⁷¹

Cholangitis, Sclerosing ⁷¹

Fibrosing Cholangitis ¹²

Classifications:

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

See all MalaCards categories (disease lists)

Orphanet: ⁵⁸

Rare hepatic diseases

External Ids:

Disease Ontology ¹² DOID:14268

MeSH ⁴⁴ D015209

SNOMED-CT ⁶⁷ 4032000

ICD10 ³² K83.09

UMLS via Orphanet ⁷² C0008313

Orphanet ⁵⁸ ORPHA447771

EFO ¹⁷ EFO_0004268

UMLS ⁷¹ C0008313 C0566602

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Summaries for Sclerosing Cholangitis

MalaCards based summary : Sclerosing Cholangitis, also known as primary sclerosing cholangitis, is related to sclerosing cholangitis, neonatal and ulcerative colitis, and has symptoms including icterus An important gene associated with Sclerosing Cholangitis is CLDN1 (Claudin 1), and among its related pathways/superpathways are Tuberculosis and Cell adhesion molecules. The drugs Simvastatin and Ursodeoxycholic acid have been mentioned in the context of this disorder. Affiliated tissues include liver, t cells and colon, and related phenotypes are digestive/alimentary and liver/biliary system

Wikipedia : ⁷⁴ Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder... more...

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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 683)

#	Related Disease	Score	Top Affiliating Genes
1	sclerosing cholangitis, neonatal	33.3	DCDC2 CLDN1
2	ulcerative colitis	32.1	TNF TLR4 PRTN3 NOD2 HLA-DRB1
3	colitis	31.9	TNF TLR4 NOD2 MADCAM1 HLA-DRB1
4	autoimmune pancreatitis	31.8	TNF TLR4 HLA-DRB1 CFTR ALB
5	autoimmune hepatitis	31.6	TNF HLA-DRB1 GPT F2 ALB
6	pouchitis	31.5	TNF TLR4 NOD2
7	liver cirrhosis	31.5	TNF TLR4 HLA-DRB1 GPT F2 ALB
8	cholangitis, primary sclerosing	31.5	TNF PRTN3 NR1I2 NR1H4 NOD2 MIR222
9	pericholangitis	31.3	ABCB4 ABCB11
10	crohn's disease	31.3	TNF TLR4 NOD2 MADCAM1 HLA-DRB1 FUT2
11	ileitis	31.3	TNF TLR4 NOD2 MADCAM1
12	acquired immunodeficiency syndrome	31.1	TNF MADCAM1 HLA-B ALB
13	cholestasis	31.1	NR1I2 NR1H4 GPT GPBAR1 F2 ALB
14	obstructive jaundice	31.1	TNF TLR4 GPT GPBAR1 F2 ALB
15	choledocholithiasis	31.0	GPT F2 ALB ABCB4
16	acute pancreatitis	31.0	TNF TLR4 GPT CFTR
17	acute cholangitis	31.0	GPT F2 ALB
18	spondylitis	31.0	TNF NOD2 HLA-B
19	esophageal varix	31.0	GPT F2 ALB
20	portal hypertension	30.9	TNF TLR4 NR1H4 GPT F2 CFTR
21	cholecystitis	30.9	TLR4 GPT F2 ALB ABCB4
22	graft-versus-host disease	30.9	TNF HLA-DRB1 HLA-B
23	liver disease	30.9	TNF NR1I2 NR1H4 GPT F2 ALB
24	ascending cholangitis	30.8	MADCAM1 GPT F2 ALB
25	diarrhea	30.8	TNF TLR4 NOD2 GPT CFTR ALB
26	primary biliary cholangitis	30.8	PRTN3 NR1I2 NR1H4 HLA-DRB1 GPT GPBAR1
27	echinococcosis	30.8	TNF TLR4 HLA-DRB1 HLA-B
28	hepatic coma	30.8	GPT F2 ALB
29	autoimmune hepatitis type 1	30.8	HLA-DRB1 GPT
30	cholelithiasis	30.7	NR1H4 GPT ALB ABCB4
31	bile duct cysts	30.7	GPT ALB ABCB4 ABCB11
32	klatskin's tumor	30.7	GPT F2 ALB
33	autoimmune gastritis	30.7	TNF HLA-DRB1 HLA-B
34	acalculous cholecystitis	30.7	GPT F2 ALB ABCB4
35	suppurative cholangitis	30.7	GPT ABCB4
36	bacterial infectious disease	30.7	TNF TLR4 NOD2 CFTR
37	bilirubin metabolic disorder	30.7	GPT F2 ALB ABCB4 ABCB11
38	hepatic encephalopathy	30.6	TNF GPT F2 ALB
39	celiac disease 1	30.6	TNF TLR4 NOD2 HLA-DRB1 HLA-B GPT
40	inflammatory bowel disease	30.6	TNF TLR4 PRTN3 NR1I2 NR1H4 NOD2
41	spondyloarthropathy 1	30.6	TNF TLR4 PRTN3 NOD2 HLA-B
42	viral hepatitis	30.6	TNF TLR4 HLA-DRB1 GPT F2 ALB
43	pleural empyema	30.5	GPT F2 ALB
44	acute liver failure	30.5	GPT F2 ALB
45	hepatitis a	30.5	TNF TLR4 GPT F2 ALB
46	proteasome-associated autoinflammatory syndrome 1	30.5	TNF TLR4 ALB
47	red cell aplasia	30.5	HLA-DRB1 HLA-B ALB
48	pure red-cell aplasia	30.5	HLA-DRB1 HLA-B ALB
49	ileus	30.4	TNF F2 CFTR
50	alopecia areata	30.4	TNF HLA-DRB1 HLA-B

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

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Symptoms & Phenotypes for Sclerosing Cholangitis

UMLS symptoms related to Sclerosing Cholangitis:

icterus

MGI Mouse Phenotypes related to Sclerosing Cholangitis:

⁴⁶

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	digestive/alimentary	MP:0005381	9.61	ABCB4 ALB CFTR F2 FUT2 NOD2
2	liver/biliary system	MP:0005370	9.28	ABCB11 ABCB4 ALB CFTR GPBAR1 NR1H4

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Drugs & Therapeutics for Sclerosing Cholangitis

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

(show top 50) (show all 72)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Simvastatin

Approved

Phase 3

79902-63-9

54454

Ursodeoxycholic acid

Approved, Investigational

Phase 3

128-13-2

31401

Bezafibrate

Approved, Investigational

Phase 3

41859-67-0

39042

Lipid Regulating Agents

Phase 3

Hypolipidemic Agents

Phase 3

Antimetabolites

Phase 3

Hydroxymethylglutaryl-CoA Reductase Inhibitors

Phase 3

Anticholesteremic Agents

Phase 3

Clofibric Acid

Phase 3

882-09-7

Cladribine

Approved, Investigational

Phase 2

4291-63-8

20279

Curcumin

Approved, Experimental, Investigational

Phase 1, Phase 2

458-37-7

969516

Sulfasalazine

Approved

Phase 2

599-79-1

5353980 5359476

Aldesleukin

Approved

Phase 2

110942-02-4, 85898-30-2

Fenofibrate

Approved

Phase 1, Phase 2

49562-28-9

3339

Tretinoin

Approved, Investigational, Nutraceutical

Phase 2

302-79-4

5538 444795

Volixibat

Experimental, Investigational

Phase 2

1025216-57-2

Mitomycins

Phase 2

Alkylating Agents

Phase 2

Anti-Inflammatory Agents

Phase 1, Phase 2

Hormones

Phase 2

2-chloro-3'-deoxyadenosine

Phase 2

Anti-Inflammatory Agents, Non-Steroidal

Phase 1, Phase 2

Antirheumatic Agents

Phase 1, Phase 2

Anti-HIV Agents

Phase 2

Antiviral Agents

Phase 2

TAK-652

Phase 2

Anti-Retroviral Agents

Phase 2

Antibodies, Monoclonal

Phase 2

Calcium, Dietary

Phase 2

Liver Extracts

Phase 2

Bile Acids and Salts

Phase 2

Streptococcal polysaccharide type III group B

Phase 2

Analgesics, Non-Narcotic

Phase 2

Analgesics

Phase 2

Interleukin-2

Phase 2

Immunosuppressive Agents

Phase 2

Immunologic Factors

Phase 2

Angiogenesis Inhibitors

Phase 2

Keratolytic Agents

Phase 2

Dermatologic Agents

Phase 2

Calcium

Nutraceutical

Phase 2

7440-70-2

271

Budesonide

Approved

Phase 1

51333-22-3

63006 5281004

Vancomycin

Approved

Phase 1

1404-90-6

441141 14969

Minocycline

Approved, Investigational

Phase 1

10118-90-8

5281021

Rifaximin

Approved, Investigational

Phase 1

80621-81-4

6436173 46783403

Lenalidomide

Approved

Phase 1

191732-72-6

216326

rituximab

Approved

Phase 1

174722-31-7

10201696

Anti-Asthmatic Agents

Phase 1

Respiratory System Agents

Phase 1

Hormone Antagonists

Phase 1

Interventional clinical trials:

(show top 50) (show all 100)

#	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	Multicenter Randomized Trial Comparing Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis	Unknown status	NCT01398917	Phase 3
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	The Effect of Bezafibrate on Cholestatic Itch	Unknown status	NCT02701166	Phase 3	Bezafibrate;Placebo
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	Multicentered Randomized Trial of High-dose Urso in Primary Sclerosing Cholangitis	Completed	NCT00059202	Phase 2, Phase 3	Ursodeoxycholic Acid
7	Digital SpyGlass Cholangioscopy Confirmed Common Bile Duct Stones Clearance Without Fluoroscopy	Completed	NCT02967926	Phase 3
8	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
9	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	Recruiting	NCT03890120	Phase 3	Cilofexor;Placebo
10	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
11	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
12	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	Not yet 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
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	Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis	Completed	NCT00004762	Phase 2	cladribine
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	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	OCA;Placebo
18	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
19	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
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 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
22	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
23	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
24	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
25	A Phase 2, Randomized, Double-Blind, Placebo Controlled Study Evaluating the Safety, Tolerability, and Efficacy of Cilofexor in Subjects With Primary Sclerosing Cholangitis Without Cirrhosis	Completed	NCT02943460	Phase 2	Cilofexor;Placebo to match Cilofexor
26	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
27	Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis.	Completed	NCT02424175	Phase 1, Phase 2
28	A Randomized, Placebo-controlled Pilot Study of Sulfasalazine for the Treatment of Primary Sclerosing Cholangitis (PSC)	Recruiting	NCT03561584	Phase 2	Sulfasalazine;Placebo
29	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
30	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
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	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
33	Intra-arterial Injection of Umbilical Cord Mesenchymal Stem Cells in Primary Sclerosing Cholangitis	Active, not recruiting	NCT03516006	Phase 1, Phase 2	UCMSC;UDCA
34	Induction of Regulatory t Cells by Low Dose IL2 in Autoimmune and Inflammatory Diseases: a Transnosographic Approach	Active, not recruiting	NCT01988506	Phase 2	Interleukin 2
35	A Phase 2, Randomized, Double-Blind Study of HTD1801 vs Ursodeoxycholic Acid (UDCA) in Adolescents With Primary Sclerosing Cholangitis (PSC)	Not yet recruiting	NCT03678480	Phase 2	HTD1801;Ursodeoxycholic Acid
36	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)	Suspended	NCT04024813	Phase 2	Seladelpar;Placebo to match Seladelpar
37	Pilot Study of Fenofibrate in Primary Sclerosing Cholangitis	Terminated	NCT01142323	Phase 1, Phase 2	fenofibrate
38	Open Label, Phase II Investigation of Thalidomide for the Treatment of Primary Sclerosing Cholangitis	Terminated	NCT00953615	Phase 2	Thalidomide
39	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
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	Primary Sclerosing Cholangitis: Value of Computerized Tomographic Cholangiography and Intraductal Volumetric Measurement in Determining the Prognosis	Withdrawn	NCT00588458	Phase 2
42	Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis	Completed	NCT00004842	Phase 1	budesonide
43	A First in Human, Randomized, Double-Blind Study to Assess Safety, Tolerability, and Pharmacokinetics of Single, Ascending Doses of HTD1801 in Healthy Subjects	Completed	NCT03099603	Phase 1	HTD1801
44	Evaluation of Docosahexaenoic Acid (DHA) for the Treatment of Primary Sclerosing Cholangitis (PSC)	Completed	NCT00325013	Phase 1	Docosahexaenoic Acid (DHA)
45	A Pilot Study of Vancomycin or Metronidazole in Patients With Primary Sclerosing Cholangitis	Completed	NCT01085760	Phase 1	Vancomycin;Vancomycin;Metronidazole;Metronidazole;Vancomycin
46	Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis: A Pilot Withdrawal/Reinstitution Trial	Completed	NCT01088607	Phase 1	ursodeoxycholic acid (UDCA)
47	The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin.	Completed	NCT01322386	Phase 1	Vancomycin
48	Open Label Pilot Study Evaluating Minocycline in Patients With Primary Sclerosing Cholangitis (PSC)	Completed	NCT00630942	Phase 1	Minocycline
49	A Pilot Study of Xifaxan in Patients With Primary Sclerosing Cholangitis	Completed	NCT01695174	Phase 1	Xifaxan
50	An Open Label Pilot Trial of Erlotinib (Tarceva) in Primary Sclerosing Cholangitis With Trisomy 7	Completed	NCT00955149	Phase 1	Erlotinib (Tarceva)

Search NIH Clinical Center for Sclerosing Cholangitis

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Genetic Tests for Sclerosing Cholangitis

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Anatomical Context for Sclerosing Cholangitis

MalaCards organs/tissues related to Sclerosing Cholangitis:

⁴⁰

Liver, T Cells, Colon, Neutrophil, Pancreas, Spleen, Kidney

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Publications for Sclerosing Cholangitis

Articles related to Sclerosing Cholangitis:

(show top 50) (show all 6311)

#	Title	Authors	PMID	Year
1	Nonalcoholic Steatohepatitis Is the Most Rapidly Increasing Indication for Liver Transplantation in the United States. ⁶¹	Younossi ZM...Henry L	32531342	2021
2	Cerebral Vascular Thrombosis Associated With Ulcerative Colitis and Primary Sclerosing Cholangitis. ⁶¹	Crook SM...Quinton RA	32773435	2021
3	Trends in Incidence of Autoimmune Liver Diseases and Increasing Incidence of Autoimmune Hepatitis. ⁶¹	Lamba M...Stedman CAM	32526342	2021
4	Hepatic NFAT signaling regulates the expression of inflammatory cytokines in cholestasis. ⁶¹	Cai SY...Boyer JL	33039404	2021
5	Eligibility for Liver Transplantation in Patients with Perihilar Cholangiocarcinoma. ⁶¹	Vugts JJA...Buettner S	32901308	2021
6	Primary Sclerosing Cholangitis-Associated Pouchitis: A Distinct Clinical Phenotype. ⁶¹	Quinn KP...Raffals LE	33549866	2021
7	Management of primary sclerosing cholangitis and its complications: an algorithmic approach. ⁶¹	Prokopic M...Beuers U	33377990	2021
8	Systematic Review and Meta-Analysis of Outcomes after Ileal Pouch-Anal Anastomosis in Primary Sclerosing Cholangitis-Ulcerative Colitis. ⁶¹	Barnes EL...Herfarth HH	33544128	2021
9	The role of oral vancomycin in inducing remission for biologic-experienced ulcerative colitis with concomitant primary sclerosing cholangitis and liver transplantation. ⁶¹	Rahman AU...Charles R	33130984	2021
10	[Surgical treatment of primary sclerosing cholangitis : Experiences from 30 years in a single center cohort with 173 consecutive patients]. ⁶¹	Branchi V...Matthaei H	32488382	2021
11	Geo-epidemiology and environmental co-variate mapping of primary biliary cholangitis and primary sclerosing cholangitis. ⁶¹	Dyson JK...Jeffreys Jones DE	33474546	2021
12	Exome Sequencing in Patient-Parent Trios Suggests New Candidate Genes for Early-onset Primary Sclerosing Cholangitis. ⁶¹	Haisma SM...van Diemen CC	33590606	2021
13	Impact of inflammatory bowel disease and azathioprine on long-term outcomes after liver transplantation for primary sclerosing cholangitis. ⁶¹	Peverelle M...Gow PJ	33569894	2021
14	Associations of neopterin and kynurenine-tryptophan ratio with survival in primary sclerosing cholangitis. ⁶¹	Dhillon AK...Hov JR	33583308	2021
15	Systematic review: non-invasive prognostic tests for primary sclerosing cholangitis. ⁶¹	Mazhar A...Russo MW	33608929	2021
16	Alterations of the salivary and fecal microbiome in patients with primary sclerosing cholangitis. ⁶¹	Lapidot Y...Safran M	32949377	2021
17	A Patient with Primary Sclerosing Cholangitis and Diffuse Biliary Dilation. ⁶¹	Fejleh MP...Kim S	32598888	2021
18	Overexpression of Cancer-Associated Stem Cell Gene OLFM4 in the Colonic Epithelium of Patients With Primary Sclerosing Cholangitis. ⁶¹	Neyazi M...Geremia A	33570127	2021
19	Clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020 (Revision of the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012). ⁶¹	Nakazawa T...Unno T	33586343	2021
20	A case with life-threatening secondary sclerosing cholangitis caused by nivolumab. ⁶¹	Yoshikawa Y...Chayama K	33200345	2021
21	Autoimmune Hepatitis: Predictors of Native Liver Survival in Children and Adolescents. ⁶¹	Porta G...Brazilian Group for the Study of Pediatric Liver Diseases	33500120	2021
22	False-positive PET/CT finding of hepatic inflammatory pseudotumor in IgG4-related sclerosing cholangitis. ⁶¹	Yildirim M...Artas G	33582317	2021
23	Liver stiffness across different chronic liver disease under therapy with statin in a real life cohort. ⁶¹	Markova AA...Wedemeyer H	32282399	2021
24	Gut Microbiome: A Potential Modifiable Risk Factor in Biliary Atresia. ⁶¹	Jain V...Dhawan A	33427793	2021
25	A case of juvenile eosinophilic cholangitis: Rapid peripheral blood hypereosinophilia after admission leading to diagnosis. ⁶¹	Fukatsu S...Itoi T	33553674	2021
26	Discriminant equation using mucosally expressed cytokines and transcription factor for making definite diagnosis of inflammatory bowel disease unclassified. ⁶¹	Okuno H...Ogawa Y	33593285	2021
27	Comprehensive assessment of ECM turnover using serum biomarkers establishes PBC as a high-turnover autoimmune liver disease. ⁶¹	Vesterhus M...Thorburn D	33225252	2021
28	Difficulty in differentiating between IgG4-related hepatic inflammatory pseudotumor and intrahepatic cholangiocarcinoma. ⁶¹	Hamano A...Takei Y	33037585	2021
29	Treatment and outcome of ulcerative colitis during the first 10 years after diagnosis in a prospectively followed population-based cohort. ⁶¹	Ronnblom A...Karlbom U	33577739	2021
30	Prednisolone-responsive primary sclerosing cholangitis with autoimmune hemolytic anemia: a case report and review of the literature. ⁶¹	Kawaguchi T...Torimura T	33038000	2021
31	Fibrates for Itch (FITCH) in Fibrosing Cholangiopathies: A Double-Blind, Randomized, Placebo-Controlled Trial. ⁶¹	de Vries E...Netherlands Association for the Study of the Liver-Cholestasis Working Group	33031833	2021
32	De novo Colorectal and Pancreatic Cancer in Liver Transplant Recipients: Identifying the Higher Risk Populations. ⁶¹	Nasser-Ghodsi N...Watt KD	33544906	2021
33	Anti-glycoprotein 2 (anti-GP2) IgA and anti-neutrophil cytoplasmic antibodies to serine proteinase 3 (PR3-ANCA): antibodies to predict severe disease, poor survival and cholangiocarcinoma in primary sclerosing cholangitis. ⁶¹	Wunsch E...Milkiewicz P	33159471	2021
34	Editorial: serologic antibodies in primary sclerosing cholangitis-a tell-tale sign of compromised gut-liver immunity? Authors' reply. ⁶¹	Wunsch E...Milkiewicz P	33368503	2021
35	Acquired factor V inhibitor: Success of steroids in a patient with primary sclerosing cholangitis, ulcero haemorragic rectocolitis, biological Biermer's disease. ⁶¹	Pineau-Vincent F...Laribi K	33516887	2021
36	Antineutrophil cytoplasmic antibody profiles differ according to type of primary sclerosing cholangitis and autoimmune hepatitis. ⁶¹	Crescente JG...Cancado ELR	33567045	2021
37	Disease-Specific Waitlist Outcomes in Liver Transplantation. ⁶¹	Nagai S...Moonka D	33423330	2021
38	Liver Transplantation for Langerhans Cell Histiocytosis: A U.S. Population-Based Analysis and Systematic Review of the Literature. ⁶¹	Ziogas IA...Alexopoulos SP	33484600	2021
39	Oral Vancomycin or Ursodeoxycholic acid for Pediatric Primary Sclerosing Cholangitis? The Uncontroversial Need for Randomized Controlled Trials. ⁶¹	Assis DN...Levy C	33403699	2021
40	Guideline review: British Society of Gastroenterology/UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. ⁶¹	Nicoletti A...Thorburn D	33456743	2021
41	Effectiveness of Ursodeoxycholic Acid in the Treatment of Primary Sclerosing Cholangitis with Ulcerative Colitis: A Pediatric Case. ⁶¹	Fujita Y...Yoshihara S	33551380	2021
42	Letter to the Editor: Imaging monitoring of primary sclerosing cholangitis patients. ⁶¹	Li J...He S	33427311	2021
43	Manganese and copper levels in patients with primary biliary cirrhosis and primary sclerosing cholangitis. ⁶¹	Dastych M...Dastych M	33428478	2021
44	Editorial: serologic antibodies in primary sclerosing cholangitis - a tell-tale sign of compromised gut-liver immunity? ⁶¹	Tornai D...Papp M	33368511	2021
45	Immunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report. ⁶¹	Kim JS...Kim HS	33511195	2021
46	Presentation and Outcomes of Autoimmune Hepatitis Type 1 and Type 2 in Children: A Single-center Study. ⁶¹	Roepe IG...Miloh T	32796427	2021
47	Predicting Outcomes in Pediatric Ulcerative Colitis for Management Optimization: Systematic Review and Consensus Statements From the Pediatric Inflammatory Bowel Disease-Ahead Program. ⁶¹	Orlanski-Meyer E...Turner D	32976826	2021
48	Antigen presentation, autoantibody production, and therapeutic targets in autoimmune liver disease. ⁶¹	Horst AK...Tiegs G	33110250	2021
49	Liver transplant recipients have an increased risk of developing colorectal adenomas: Results from a retrospective study. ⁶¹	Herrero JI...Betes M	33190329	2021
50	NAFLD exacerbates cholangitis and promotes cholangiocellular carcinoma in mice. ⁶¹	Maeda S...Chuma M	33506599	2021

Sources

Genes for Sclerosing Cholangitis

Genes related to Sclerosing Cholangitis (0 elite genes):

(show all 36)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence
1	CLDN1	Claudin 1	Protein Coding	43.15	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders Publications Summaries Variants (show sections)
2	DCDC2	Doublecortin Domain Containing 2	Protein Coding	33.38	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders Publications Variants (show sections)
3	ABCB4	ATP Binding Cassette Subfamily B Member 4	Protein Coding	15.83	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
4	GPT	Glutamic--Pyruvic Transaminase	Protein Coding	15.43	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
5	GPBAR1	G Protein-Coupled Bile Acid Receptor 1	Protein Coding	14.9	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
6	ABCB11	ATP Binding Cassette Subfamily B Member 11	Protein Coding	14.75	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
7	ALB	Albumin	Protein Coding	14.49	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
8	TNF	Tumor Necrosis Factor	Protein Coding	14.42	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
9	CFTR	CF Transmembrane Conductance Regulator	Protein Coding	14.41	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
10	NR1H4	Nuclear Receptor Subfamily 1 Group H Member 4	Protein Coding	14.41	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
11	MIR222	MicroRNA 222	RNA Gene	14.34	DISEASES inferred ¹⁵ ¹⁵ GeneCards inferred via : Publications (show sections)
12	HLA-DRB1	Major Histocompatibility Complex, Class II, DR Beta 1	Protein Coding	14.2	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
13	MADCAM1	Mucosal Vascular Addressin Cell Adhesion Molecule 1	Protein Coding	14.2	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
14	FUT2	Fucosyltransferase 2	Protein Coding	14.02	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
15	NR1I2	Nuclear Receptor Subfamily 1 Group I Member 2	Protein Coding	14.02	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
16	NOD2	Nucleotide Binding Oligomerization Domain Containing 2	Protein Coding	13.91	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
17	HLA-B	Major Histocompatibility Complex, Class I, B	Protein Coding	13.77	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
18	PRTN3	Proteinase 3	Protein Coding	13.52	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
19	TLR4	Toll Like Receptor 4	Protein Coding	13.43	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
20	F2	Coagulation Factor II, Thrombin	Protein Coding	13.38	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
21	IL10	Interleukin 10	Protein Coding	13.37	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
22	IL6	Interleukin 6	Protein Coding	13.34	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
23	HP	Haptoglobin	Protein Coding	13.33	DISEASES inferred ¹⁵ GeneCards inferred via : Summaries (show sections)
24	MST1	Macrophage Stimulating 1	Protein Coding	13.26	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
25	CCR5	C-C Motif Chemokine Receptor 5	Protein Coding	13.24	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
26	IL2	Interleukin 2	Protein Coding	13.17	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
27	CXCL8	C-X-C Motif Chemokine Ligand 8	Protein Coding	13.1	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
28	GGT1	Gamma-Glutamyltransferase 1	Protein Coding	13.1	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
29	ICAM1	Intercellular Adhesion Molecule 1	Protein Coding	13.04	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
30	MPO	Myeloperoxidase	Protein Coding	13.04	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
31	CTLA4	Cytotoxic T-Lymphocyte Associated Protein 4	Protein Coding	13.04	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
32	HLA-DQB1	Major Histocompatibility Complex, Class II, DQ Beta 1	Protein Coding	13.01	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
33	SULT2A1	Sulfotransferase Family 2A Member 1	Protein Coding	12.9	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
34	CEACAM5	CEA Cell Adhesion Molecule 5	Protein Coding	12.86	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
35	ABCC2	ATP Binding Cassette Subfamily C Member 2	Protein Coding	12.83	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
36	CLDN16	Claudin 16	Protein Coding	12.81	DISEASES inferred ¹⁵ GeneCards inferred via : Variants (show sections)

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	Tuberculosis ³⁶	11.92	TNF TLR4 NOD2 HLA-DRB1
2	Cell adhesion molecules ³⁶	11.8	MADCAM1 HLA-DRB1 HLA-B CLDN1
3	Rheumatoid arthritis ³⁶	11.58	TNF TLR4 HLA-DRB1
4	Bile secretion ³⁶	11.51	NR1H4 CFTR ABCB4 ABCB11
5	IgA-Producing B Cells in the Intestine ⁶⁴	11.4	TNF TLR4 MADCAM1
6	Farnesoid X Receptor Pathway ¹	10.61	NR1H4 ABCB4 ABCB11
7	Nuclear Receptors in Lipid Metabolism and Toxicity ¹	10.47	NR1I2 NR1H4 ABCB4 ABCB11
8	Drug Induction of Bile Acid Pathway ¹	10.36	NR1I2 NR1H4 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	plasma membrane	GO:0005886	10.13	TNF TLR4 PRTN3 NOD2 MADCAM1 HLA-DRB1
2	extracellular exosome	GO:0070062	9.91	PRTN3 HLA-DRB1 HLA-B GPT FUT2 F2
3	integral component of plasma membrane	GO:0005887	9.56	TNF TLR4 HLA-DRB1 HLA-B CLDN1 CFTR
4	recycling endosome membrane	GO:0055038	9.54	HLA-B CFTR ABCB11
5	cell surface	GO:0009986	9.17	TNF TLR4 NOD2 HLA-DRB1 HLA-B CFTR
6	intercellular canaliculus	GO:0046581	9.16	ABCB4 ABCB11

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

(show all 20)

#	Name	GO ID	Score	Top Affiliating Genes
1	immune response	GO:0006955	9.97	TNF TLR4 MADCAM1 HLA-DRB1 HLA-B
2	defense response to bacterium	GO:0042742	9.88	TNF TLR4 NR1H4 NOD2
3	positive regulation of I-kappaB kinase/NF-kappaB signaling	GO:0043123	9.78	TNF TLR4 NOD2 HLA-DRB1
4	positive regulation of interleukin-6 production	GO:0032755	9.77	TNF TLR4 NOD2
5	positive regulation of JNK cascade	GO:0046330	9.75	TNF TLR4 NOD2
6	negative regulation of tumor necrosis factor production	GO:0032720	9.71	TLR4 NR1H4 NOD2
7	cellular response to lipopolysaccharide	GO:0071222	9.71	TNF TLR4 NR1H4 NOD2
8	negative regulation of interleukin-6 production	GO:0032715	9.7	TNF TLR4 NR1H4
9	positive regulation of NIK/NF-kappaB signaling	GO:1901224	9.69	TNF TLR4 NOD2
10	positive regulation of interleukin-8 production	GO:0032757	9.67	TNF TLR4 NOD2
11	positive regulation of interleukin-1 beta production	GO:0032731	9.63	TNF TLR4 NOD2
12	positive regulation of chemokine (C-X-C motif) ligand 2 production	GO:2000343	9.56	TNF TLR4
13	T-helper 1 type immune response	GO:0042088	9.55	TLR4 HLA-DRB1
14	positive regulation of insulin secretion involved in cellular response to glucose stimulus	GO:0035774	9.54	NR1H4 HLA-DRB1 CFTR
15	positive regulation of cytokine production involved in inflammatory response	GO:1900017	9.5	TNF TLR4 NOD2
16	negative regulation of interferon-gamma production	GO:0032689	9.46	TLR4 NR1H4 NOD2 HLA-DRB1
17	positive regulation of humoral immune response mediated by circulating immunoglobulin	GO:0002925	9.43	TNF NOD2
18	detection of bacterium	GO:0016045	9.43	NOD2 HLA-DRB1 HLA-B
19	positive regulation of ERK1 and ERK2 cascade	GO:0070374	9.43	TNF TLR4 NOD2 MIR222 HLA-DRB1 GPBAR1
20	cellular response to bile acid	GO:1903413	8.8	NR1H4 GPBAR1 ABCB4

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

#	Name	GO ID	Score	Top Affiliating Genes
1	ATPase activity, coupled to transmembrane movement of substances	GO:0042626	9.13	CFTR ABCB4 ABCB11
2	bile acid receptor activity	GO:0038181	8.62	NR1H4 GPBAR1

Sources

Sources for Sclerosing Cholangitis

¹ BioSystems

² BitterDB

³ CDC

⁴ Cell Signaling Technology

⁵ ClinicalTrials

⁶ ClinVar

⁷ CNVD

⁸ Cochrane Library

⁹ Cosmic

¹⁰ dbSNP

¹¹ DGIdb

¹² Disease Ontology

¹³ diseasecard

¹⁴ DiseaseEnhancer

¹⁵ DISEASES

¹⁶ DrugBank

¹⁷ EFO

¹⁸ ExPASy

¹⁹ FMA

²⁰ GARD

²¹ GeneAnalytics

²² GeneCards

²³ GeneGo (Thomson Reuters)

²⁴ GeneHancer via GeneCards

²⁵ GeneReviews

²⁶ GenomeRNAi

²⁷ GEO DataSets

²⁸ GO

²⁹ GTR

³⁰ HMDB

³¹ HPO

³² ICD10

³³ ICD10 via Orphanet

³⁴ ICD9CM

³⁵ IUPHAR

³⁶ KEGG

³⁷ LifeMap

³⁸ LncRNADisease

³⁹ LOVD

⁴⁰ MalaCards

⁴¹ MedGen

⁴² MedlinePlus

⁴³ MedlinePlus Genetics

⁴⁴ MeSH

⁴⁵ MESH via Orphanet

⁴⁶ MGI

⁴⁷ miR2Disease

⁴⁸ NCBI Bookshelf

⁴⁹ NCI

⁵⁰ NCIt

⁵¹ NDF-RT

⁵² NIH Clinical Center

⁵³ NINDS

⁵⁴ Novoseek

⁵⁵ Novus Biologicals

⁵⁶ OMIM via Orphanet

⁵⁷ OMIM® (Updated 05-Mar-2021)

⁵⁸ Orphanet

⁵⁹ PathCards

⁶⁰ PharmGKB

⁶¹ PubMed

⁶² PubMed Health

⁶³ QIAGEN

⁶⁴ R&D Systems

⁶⁵ Reactome

⁶⁶ Sino Biological

⁶⁷ SNOMED-CT

⁶⁸ SNOMED-CT via HPO

⁶⁹ TGDB

⁷⁰ Tocris

⁷¹ UMLS

⁷² UMLS via Orphanet

⁷³ UniProtKB/Swiss-Prot

⁷⁴ Wikipedia