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

MCID: SCL009 MIFTS: 53	Sclerosing Cholangitis Categories: Gastrointestinal diseases, Rare diseases, Liver diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs Download Expand all tables

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Aliases & Classifications for Sclerosing Cholangitis

MalaCards integrated aliases for Sclerosing Cholangitis:

Name: Sclerosing Cholangitis ¹² ⁷⁶ ¹⁵

Cholangitis, Sclerosing ⁴⁴ ⁷³

Primary Sclerosing Cholangitis ⁷³

Fibrosing Cholangitis ¹²

Classifications:

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

See all MalaCards categories (disease lists)

ICD10: ³³

Diseases of the digestive system

Disorders of gallbladder, biliary tract and pancreas

Other diseases of biliary tract

Cholangitis

External Ids:

Disease Ontology ¹² DOID:14268

ICD10 ³³ K83.0

MeSH ⁴⁴ D015209

SNOMED-CT ⁶⁸ 197443000 235917005 4032000

UMLS ⁷³ C0008313

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Summaries for Sclerosing Cholangitis

MalaCards based summary : Sclerosing Cholangitis, also known as cholangitis, sclerosing, is related to sclerosing cholangitis, neonatal and autoimmune pancreatitis, and has symptoms including icterus An important gene associated with Sclerosing Cholangitis is CLDN1 (Claudin 1), and among its related pathways/superpathways are Allograft rejection and HTLV-I infection. The drugs Vancomycin and Ceftriaxone have been mentioned in the context of this disorder. Affiliated tissues include liver, eye and skin, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Wikipedia : ⁷⁶ 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 269)

#	Related Disease	Score	Top Affiliating Genes
1	sclerosing cholangitis, neonatal	35.1	CLDN1 DCDC2
2	autoimmune pancreatitis	31.7	CFTR HLA-DRB1 TNF
3	autoimmune hepatitis	30.9	CD40LG GGT1 HLA-DRB1
4	pure red-cell aplasia	30.6	ALB HLA-B HLA-DRB1
5	burns	30.6	ALB TNF
6	gastroenteritis	30.2	ALB FUT2 TNF
7	primary biliary cirrhosis	30.1	ABCB4 ALB CD40LG GGT1
8	peritonitis	30.0	ALB MPO TNF
9	psoriatic arthritis	29.9	HLA-B HLA-C HLA-DRB1 TNF
10	hepatitis a	29.8	ALB CD40LG TNF
11	obstructive jaundice	29.8	ALB GGT1 GPBAR1 TNF
12	suppurative cholangitis	29.7	ABCB4 MPO TNF
13	autoimmune disease	29.6	CD40LG HLA-B HLA-DRB1 TNF
14	acquired immunodeficiency syndrome	29.5	ALB CD40LG HLA-B TNF
15	cholestasis	29.5	ABCB11 ABCB4 GGT1 NR1I2
16	churg-strauss syndrome	29.5	CD40LG MPO TNF
17	liver disease	29.4	ABCB11 ABCB4 ALB GGT1 TNF
18	celiac disease 1	29.1	ALB CD40LG TNF
19	pneumonia	29.0	CD40LG MPO TNF
20	cholangitis, primary sclerosing	29.0	ABCB4 BPI CFTR CTSG FUT2 GGT1
21	cystic fibrosis	28.8	BPI CFTR CTSG MPO
22	bronchiectasis	28.6	BPI CFTR CTSG MPO
23	glomerulonephritis	28.6	ALB CD40LG MPO
24	bile duct disease	28.2	ABCB11 ABCB4 ALB GGT1 GPBAR1 NR1I2
25	biliary tract disease	28.1	ABCB11 ABCB4 ALB GGT1 GPBAR1 NR1I2
26	intrahepatic cholestasis	27.8	ABCB11 ABCB4 ALB GGT1 GPBAR1 NR1I2
27	systemic lupus erythematosus	27.8	ALB CD40LG CTSG HLA-DRB1 MPO TNF
28	inflammatory bowel disease	27.7	ALB BPI CTSG HLA-B HLA-DRB1 MPO
29	cholangitis	26.7	ABCB11 ABCB4 ALB CD40LG CFTR CLDN1
30	ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis	12.7
31	secondary sclerosing cholangitis	12.5
32	igg4-related sclerosing cholangitis	12.2
33	pancreatitis, sclerosing cholangitis, and sicca complex	12.2
34	isolated neonatal sclerosing cholangitis	12.1
35	bile duct carcinoma	11.6
36	cholangiocarcinoma	11.6
37	postherpetic neuralgia	10.9	HLA-B HLA-DRB1
38	idiopathic inflammatory myopathy	10.8	HLA-B HLA-DRB1
39	glaucomatocyclitic crisis	10.8	HLA-B HLA-C
40	colitis	10.8
41	ulcerative colitis	10.7
42	beryllium disease	10.7	HLA-DRB1 TNF
43	pityriasis rosea	10.7	HLA-B HLA-C HLA-DRB1
44	pars planitis	10.6	HLA-B HLA-DRB1
45	anteroseptal myocardial infarction	10.6	ALB FUT2 HLA-DRB1
46	pancreatitis	10.6
47	paraneoplastic pemphigus	10.6	HLA-B HLA-C HLA-DRB1
48	chronic beryllium disease	10.6	HLA-DRB1 TNF
49	autoimmune inner ear disease	10.6	MPO TNF
50	hepatitis	10.6

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

GenomeRNAi Phenotypes related to Sclerosing Cholangitis according to GeneCards Suite gene sharing:

²⁶ (show all 31)

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	Increased shRNA abundance (Z-score > 2)	GR00366-A-100	10.15	MPO
2	Increased shRNA abundance (Z-score > 2)	GR00366-A-103	10.15	HLA-C HLA-B CTSG
3	Increased shRNA abundance (Z-score > 2)	GR00366-A-110	10.15	HLA-C HLA-B
4	Increased shRNA abundance (Z-score > 2)	GR00366-A-118	10.15	HLA-C HLA-B
5	Increased shRNA abundance (Z-score > 2)	GR00366-A-124	10.15	HLA-C
6	Increased shRNA abundance (Z-score > 2)	GR00366-A-126	10.15	MPO
7	Increased shRNA abundance (Z-score > 2)	GR00366-A-13	10.15	CTSG
8	Increased shRNA abundance (Z-score > 2)	GR00366-A-130	10.15	GGT1
9	Increased shRNA abundance (Z-score > 2)	GR00366-A-143	10.15	GGT1
10	Increased shRNA abundance (Z-score > 2)	GR00366-A-149	10.15	HLA-C CTSG
11	Increased shRNA abundance (Z-score > 2)	GR00366-A-156	10.15	HLA-C HLA-B
12	Increased shRNA abundance (Z-score > 2)	GR00366-A-157	10.15	MPO
13	Increased shRNA abundance (Z-score > 2)	GR00366-A-161	10.15	CTSG
14	Increased shRNA abundance (Z-score > 2)	GR00366-A-162	10.15	HLA-C
15	Increased shRNA abundance (Z-score > 2)	GR00366-A-169	10.15	GGT1
16	Increased shRNA abundance (Z-score > 2)	GR00366-A-176	10.15	MPO
17	Increased shRNA abundance (Z-score > 2)	GR00366-A-178	10.15	HLA-C HLA-B
18	Increased shRNA abundance (Z-score > 2)	GR00366-A-186	10.15	MPO
19	Increased shRNA abundance (Z-score > 2)	GR00366-A-19	10.15	HLA-C HLA-B
20	Increased shRNA abundance (Z-score > 2)	GR00366-A-202	10.15	HLA-C
21	Increased shRNA abundance (Z-score > 2)	GR00366-A-21	10.15	CTSG
22	Increased shRNA abundance (Z-score > 2)	GR00366-A-29	10.15	MPO
23	Increased shRNA abundance (Z-score > 2)	GR00366-A-31	10.15	HLA-C HLA-B
24	Increased shRNA abundance (Z-score > 2)	GR00366-A-36	10.15	MPO
25	Increased shRNA abundance (Z-score > 2)	GR00366-A-43	10.15	CTSG
26	Increased shRNA abundance (Z-score > 2)	GR00366-A-47	10.15	HLA-C
27	Increased shRNA abundance (Z-score > 2)	GR00366-A-57	10.15	MPO
28	Increased shRNA abundance (Z-score > 2)	GR00366-A-7	10.15	HLA-C GGT1 MPO HLA-B
29	Increased shRNA abundance (Z-score > 2)	GR00366-A-70	10.15	MPO
30	Increased shRNA abundance (Z-score > 2)	GR00366-A-74	10.15	HLA-C HLA-B
31	Increased shRNA abundance (Z-score > 2)	GR00366-A-93	10.15	HLA-C

MGI Mouse Phenotypes related to Sclerosing Cholangitis:

⁴⁶

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	homeostasis/metabolism	MP:0005376	9.7	ABCB11 ABCB4 ALB CD40LG CFTR CLDN1
2	liver/biliary system	MP:0005370	9.23	ABCB11 ABCB4 ALB CFTR CTSG GPBAR1

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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 94)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Vancomycin

Approved

Phase 4,Phase 3,Phase 1,Not Applicable

1404-90-6

441141 14969

Ceftriaxone

Approved

Phase 4

73384-59-5

5479530 5361919

Levofloxacin

Approved, Investigational

Phase 4

100986-85-4

149096

Ofloxacin

Approved

Phase 4

82419-36-1

4583

Leflunomide

Approved, Investigational

Phase 4

75706-12-6

3899

Methylprednisolone

Approved, Vet_approved

Phase 4

83-43-2

6741

Prednisolone

Approved, Vet_approved

Phase 4

50-24-8

5755

Prednisone

Approved, Vet_approved

Phase 4,Not Applicable

53-03-2

5865

Anti-Bacterial Agents

Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable

Anti-Infective Agents

Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable

Liver Extracts

Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable

Antibiotics, Antitubercular

Phase 4,Phase 2,Not Applicable

Antibodies

Phase 4,Phase 2,Phase 1,Not Applicable

Anti-Inflammatory Agents

Phase 4,Phase 1,Phase 2

Antirheumatic Agents

Phase 4,Phase 1,Phase 2

glucocorticoids

Phase 4,Phase 1

Hormone Antagonists

Phase 4,Phase 1

Hormones

Phase 4,Phase 1

Hormones, Hormone Substitutes, and Hormone Antagonists

Phase 4,Phase 1

Immunoglobulin G

Phase 4,Phase 1,Phase 2,Not Applicable

Immunoglobulins

Phase 4,Phase 2,Phase 1,Not Applicable

Immunosuppressive Agents

Phase 4,Phase 2,Phase 1

Bile Acids and Salts

Phase 4,Phase 2,Phase 1

Gastrointestinal Agents

Phase 4,Phase 2,Phase 3,Phase 1

Antitubercular Agents

Phase 4

Cytochrome P-450 CYP1A2 Inhibitors

Phase 4

Cytochrome P-450 Enzyme Inhibitors

Phase 4

Renal Agents

Phase 4

Topoisomerase Inhibitors

Phase 4

Antineoplastic Agents, Hormonal

Phase 4

Methylprednisolone acetate

Phase 4

Methylprednisolone Hemisuccinate

Phase 4

Prednisolone acetate

Phase 4

Prednisolone hemisuccinate

Phase 4

Prednisolone phosphate

Phase 4

Bezafibrate

Approved, Investigational

Phase 3

41859-67-0

39042

Ursodeoxycholic acid

Approved, Investigational

Phase 2, Phase 3,Phase 1

128-13-2

31401

Antimetabolites

Phase 3,Phase 1,Phase 2,Not Applicable

Hypolipidemic Agents

Phase 3,Phase 1,Phase 2

Lipid Regulating Agents

Phase 3,Phase 1,Phase 2

Cholagogues and Choleretics

Phase 2, Phase 3,Phase 1

Cladribine

Approved, Investigational

Phase 2

4291-63-8

20279

rituximab

Approved

Phase 1, Phase 2

174722-31-7

10201696

Curcumin

Approved, Investigational

Phase 1, Phase 2

458-37-7

969516

Aldesleukin

Approved

Phase 2

85898-30-2, 110942-02-4

Dobutamine

Approved

Phase 2

34368-04-2

36811

Norepinephrine

Approved

Phase 2

51-41-2

439260

Fenofibrate

Approved

Phase 1, Phase 2

49562-28-9

3339

Tretinoin

Approved, Investigational, Nutraceutical

Phase 2,Phase 1

302-79-4

5538 444795

Anti-HIV Agents

Phase 2

Interventional clinical trials:

(show top 50) (show all 92)

#	Name	Status	NCT ID	Phase	Drugs
1	Effect and Safety of Oral Vancomycin in Primary Sclerosing Cholangitis Patients	Unknown status	NCT02605213	Phase 4	Vancomycin;Placebo
2	Antibiotics to Decrease Post ERCP Cholangitis	Recruiting	NCT03087656	Phase 4	Ceftriaxone;Levofloxacin
3	Leflunomide for Maintenance of Remission in IgG4 Related Disease	Recruiting	NCT02703194	Phase 4	Prednisone;Leflunomide
4	A Pilot Study to Characterize Bile Acid Metabolism and Dysbiosis in Primary Sclerosing Cholangitis	Active, not recruiting	NCT02464020	Phase 4	Vancomycin
5	Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis	Unknown status	NCT01398917	Phase 3
6	Probiotics in Patients With Primary Sclerosing Cholangitis	Unknown status	NCT00161148	Phase 3	Probiotics
7	The Effect of Bezafibrate on Cholestatic Itch	Unknown status	NCT02701166	Phase 3	Bezafibrate;Placebo
8	Trial of High-dose Urso in Primary Sclerosing Cholangitis	Completed	NCT00059202	Phase 2, Phase 3	Ursodeoxycholic Acid
9	Primary Sclerosing Cholangitis With Oral Vancomycin by the Study of Its Antimicrobial and Immunomodulating Effects	Active, not recruiting	NCT01802073	Phase 3	Oral Vancomycin
10	Efficacy and Safety of Vedolizumab Intravenous (IV) in the Treatment of Primary Sclerosing Cholangitis in Subjects With Underlying Inflammatory Bowel Disease	Withdrawn	NCT03035058	Phase 3	Vedolizumab;Placebo
11	PERSEUS: Preliminary Efficacy and Safety of Cenicriviroc in Adult Subjects With Primary Sclerosing Cholangitis	Completed	NCT02653625	Phase 2	Cenicriviroc 150 mg
12	Phase 2 Study of NGM282 in Patients With Primary Sclerosing Cholangitis	Completed	NCT02704364	Phase 2
13	Obeticholic Acid (OCA) in Primary Sclerosing Cholangitis (PSC)	Completed	NCT02177136	Phase 2	OCA;Placebo
14	Norursodeoxycholic Acid in the Treatment of Primary Sclerosing Cholangitis	Completed	NCT01755507	Phase 2	norUDCA;Placebo
15	Open Label Study to Evaluate Safety and Efficacy of LUM001 in Patients With Primary Sclerosing Cholangitis	Completed	NCT02061540	Phase 2	LUM001
16	Simtuzumab (GS-6624) in the Prevention of Progression of Liver Fibrosis in Subjects With Primary Sclerosing Cholangitis (PSC)	Completed	NCT01672853	Phase 2
17	Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis	Completed	NCT00004762	Phase 2	cladribine
18	Rituximab in IgG4-RD: A Phase 1-2 Trial	Completed	NCT01584388	Phase 1, Phase 2	Rituximab
19	Mitomycin C Therapy for Patients With Primary Sclerosing Cholangitis	Recruiting	NCT01688024	Phase 2	Mitomycin C;Normal saline
20	A Trial of BTT1023 in Patients With Primary Sclerosing Cholangitis	Recruiting	NCT02239211	Phase 2	BTT1023
21	A Study Evaluating the Safety and Efficacy of Curcumin in Patients With Primary Sclerosing Cholangitis	Recruiting	NCT02978339	Phase 1, Phase 2	Curcumin
22	A Research Study to Evaluate Safety and Efficacy of DUR-928 in Subjects With Primary Sclerosing Cholangitis (PSC)	Recruiting	NCT03394781	Phase 2	DUR-928
23	A POC and Dose-Ranging Study of HTD1801 in PSC Patients	Recruiting	NCT03333928	Phase 2	HTD1801;Placebo
24	An Efficacy Trial of Low Dose All-trans Retinoic Acid in Patients With Primary Sclerosing Cholangitis	Recruiting	NCT03359174	Phase 2	All-trans retinoic acid
25	Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases	Recruiting	NCT01988506	Phase 2	Interleukin 2
26	Molecular Adsorbent Recirculating System (MARS®) in Hypoxic Hepatitis	Recruiting	NCT01690845	Phase 2
27	Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis.	Active, not recruiting	NCT02424175	Phase 1, Phase 2
28	Umbilical Cord Mesenchymal Stem Cells in Primary Sclerosing Cholangitis	Active, not recruiting	NCT03516006	Phase 1, Phase 2	UCMSC;UDCA
29	Safety, Tolerability, and Efficacy of GS-9674 in Adults With Primary Sclerosing Cholangitis Without Cirrhosis	Active, not recruiting	NCT02943460	Phase 2	GS-9674;Placebo to match GS-9674
30	A Study of Oral Hymecromone to Treat Adults With Primary Sclerosing Cholangitis	Not yet recruiting	NCT02780752	Phase 1, Phase 2	hymecromone
31	Sulfasalazine for the Treatment of Primary Sclerosing Cholangitis	Not yet recruiting	NCT03561584	Phase 2	Sulfasalazine;Placebo
32	A Single-arm,Phase IIa,Safety and Efficacy Trial of Selected MSCs in the Treatment of Patients With PSC & AiH	Not yet recruiting	NCT02997878	Phase 1, Phase 2
33	Thalidomide for the Treatment of Primary Sclerosing Cholangitis (PSC)	Terminated	NCT00953615	Phase 2	Thalidomide
34	Pilot Study of Fenofibrate for PSC	Terminated	NCT01142323	Phase 1, Phase 2	fenofibrate
35	The Value of CT Cholangiography in Primary Sclerosing Cholangitis	Withdrawn	NCT00588458	Phase 2
36	Gastrointestinal Microbiota in Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin	Completed	NCT01322386	Phase 1	Vancomycin
37	Erlotinib for Chemoprevention in Trisomy 7 Positive Primary Sclerosing Cholangitis (PSC)	Completed	NCT00955149	Phase 1	Erlotinib (Tarceva)
38	Safety and Efficacy Study of Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis	Completed	NCT01088607	Phase 1	ursodeoxycholic acid (UDCA)
39	A Pilot Study of Vancomycin or Metronidazole in Patients With Primary Sclerosing Cholangitis	Completed	NCT01085760	Phase 1	Vancomycin;Vancomycin;Metronidazole;Metronidazole;Vancomycin
40	Minocycline in Primary Sclerosing Cholangitis (PSC)	Completed	NCT00630942	Phase 1	Minocycline
41	A Pilot Study of Xifaxan to Treat Patients With PSC	Completed	NCT01695174	Phase 1	Xifaxan
42	Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis	Completed	NCT00004842	Phase 1	budesonide
43	Evaluation of DHA for the Treatment of PSC	Completed	NCT00325013	Phase 1	Docosahexaenoic Acid (DHA)
44	Combination Therapy With Ursodeoxycholic Acid (UDCA) and All-Trans Retinoic Acid (ATRA) for Treatment of Primary Sclerosing Cholangitis	Completed	NCT01456468	Phase 1	Oral all-trans retinoic acid (ATRA)
45	A Study of HTD1801 in Healthy Subjects	Completed	NCT03099603	Phase 1	HTD1801
46	Optical Frequency Domain Imaging (OFDI) for Biliary Stricture Imaging	Completed	NCT01439620	Phase 1
47	Biliary Excretion of Conjugated Bile Acids in Humans Measured by 11C-cholylsarcosine PET/CT	Completed	NCT01879735	Phase 1	11C-CSar;ICG
48	Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin	Recruiting	NCT02137668	Phase 1	Oral Vancomycin
49	Treatment of IgG4-Related Disease With Revlimid and Rituximab	Recruiting	NCT02705638	Phase 1	Rituximab;Lenalidomide
50	A Study Of Ursolic Acid For Primary Sclerosing Cholangitis	Withdrawn	NCT03216876	Phase 1	Ursolic acid

Search NIH Clinical Center for Sclerosing Cholangitis

Cochrane evidence based reviews: cholangitis, sclerosing

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Genetic Tests for Sclerosing Cholangitis

Sources

Anatomical Context for Sclerosing Cholangitis

MalaCards organs/tissues related to Sclerosing Cholangitis:

⁴¹

Liver, Eye, Skin, Colon, T Cells, Testes, Thyroid

Sources

Publications for Sclerosing Cholangitis

Articles related to Sclerosing Cholangitis:

(show top 50) (show all 1150)

#	Title	Authors	Year
1	Clinical guidelines for primary sclerosing cholangitis 2017. ( 29951926 )	Isayama H....Takikawa H.	2018
2	The gut microbiota, bile acids and their correlation in primary sclerosing cholangitis associated with inflammatory bowel disease. ( 29435321 )	Torres J....Hu J.	2018
3	Symptoms do not Correlate With Findings From Colonoscopy in Children with Inflammatory Bowel Disease and Primary Sclerosing Cholangitis. ( 29378308 )	Ricciuto A....Griffiths A.M.	2018
4	Tumor Necrosis Factor Alpha Inhibition for Inflammatory Bowel Disease after Liver Transplant for Primary Sclerosing Cholangitis. ( 29862092 )	Parekh R....Kaur N.	2018
5	Serum miRNA-122 is an independent biomarker of survival in patients with primary sclerosing cholangitis. ( 29922759 )	Friedrich K....Gotthardt D.N.	2018
6	Vedolizumab in patients with concurrent primary sclerosing cholangitis and inflammatory bowel disease does not improve liver biochemistry but is safe and effective for the bowel disease. ( 29377235 )	Christensen B....Rubin D.T.	2018
7	Editorial: the role of colonic inflammation in the progression of liver disease in primary sclerosing cholangitis-Authors' reply and Letter: the effects of colectomy prior to the diagnosis of primary sclerosing cholangitis on prognosis may have been overestimated. ( 29314129 )	Nordenvall C....Bergquist A.	2018
8	Lack of Correlation of Liver Tests With Fibrosis Stage at Diagnosis in Pediatric Primary Sclerosing Cholangitis. ( 29356767 )	Cotter J.M....Mack C.L.	2018
9	A promising medium-term follow-up of pediatric sclerosing cholangitis: Mild phenotype or early diagnosis? ( 29316057 )	Ferrari F....Iorio R.	2018
10	The role of MRI and ERCP in the evaluation of disease activity and severity in primary sclerosing cholangitis. ( 29901259 )	Tenca A....FAorkkilAo M.	2018
11	Peribiliary Cysts presenting as Primary Sclerosing Cholangitis. ( 29432923 )	Tamilarasan A.G....Lee A.U.	2018
12	Letter: the effects of colectomy prior to the diagnosis of primary sclerosing cholangitis on prognosis may have been overestimated. ( 29314130 )	Tao P....Wang S.Y.	2018
13	Reviewing the Risk of Colorectal Cancer in Inflammatory Bowel Disease After Liver Trasnplantation for Primary Sclerosing Cholangitis. ( 29361103 )	Rao B.B....Kowdley K.V.	2018
14	Differential diagnosis of cholangiocarcinoma and IgG4-related sclerosing cholangitis by fluorescence inA situ hybridization using transpapillary forceps biopsy specimens. ( 29532638 )	Kato A....Joh T.	2018
15	Endoscopic management of primary sclerosing cholangitis. ( 29883229 )	Tabibian J.H....Baron T.H.	2018
16	Effects of vedolizumab, adalimumab and infliximab on biliary inflammation in individuals with primary sclerosing cholangitis and inflammatory bowel disease. ( 29808485 )	Tse C.S....Lightner A.L.	2018
17	Kindler syndrome in a patient with colitis and primary sclerosing cholangitis: coincidence or association? ( 29634879 )	Roda A.8....Has C.	2018
18	Localized intrahepatic IgG4-related sclerosing cholangitis (IgG4-SC) as an additional type of IgG4-SC: a systematic analysis of 12 cases. ( 29313399 )	Mo Z....Zeng Q.	2018
19	Diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. ( 29781738 )	Taghavi S.A....Bagheri Lankarani K.	2018
20	Serological markers of extracellular matrix remodeling predict transplant-free survival in primary sclerosing cholangitis. ( 29851098 )	Nielsen M.J....Vesterhus M.	2018
21	Macrophages contribute to the pathogenesis of sclerosing cholangitis in mice. ( 29802947 )	Guicciardi M.E....Gores G.J.	2018
22	The significance of serum IgG<sub>4</sub>and CA19-9, autoantibodies in diagnosis and differential diagnosis of IgG<sub>4</sub>-related sclerosing cholangitis. ( 29272982 )	Tan L....Cao L.	2018
23	Secondary sclerosing cholangitis in critically ill patients after a traffic accident-a new entity that should be considered in death classification. ( 29484493 )	Zack F....BA1ttner A.	2018
24	Update in the Care and Management of Patients with Primary Sclerosing Cholangitis. ( 29886518 )	Sedki M....Levy C.	2018
25	Patient-reported outcome measures used in patients with primary sclerosing cholangitis: a systematic review. ( 29976215 )	Isa F....Calvert M.	2018
26	Sclerosing Cholangitis Like Changes on Magnetic Resonance Cholangiography in Patients with Drug Induced Liver Injury. ( 29966706 )	Ahmad J....Bjornsson E.S.	2018
27	Anesthetic Care of 2 Siblings With Neonatal Ichthyosis and Sclerosing Cholangitis Syndrome: Case Reports. ( 29738332 )	Khalifa C....Veyckemans F.	2018
28	Aberrant expression of redox regulatory proteins in patients with concomitant primary Sclerosing cholangitis/inflammatory bowel disease. ( 29852184 )	Petersen D.R....Shearn C.T.	2018
29	Right Sided Colonic Juvenile Polyp (polyps) in Pediatric Primary Sclerosing Cholangitis Patients. ( 29931365 )	Wyatt A....Kellermayer R.	2018
30	Cholestasis after TIPS placement in a patient with primary sclerosing cholangitis: an uncommon complication. ( 29278002 )	Salagre GarcA-a A....Castellano Tortajada G.	2018
31	Review article: the evidence that vancomycin is a therapeutic option for primary sclerosing cholangitis. ( 29411404 )	Damman J.L....Lindor K.D.	2018
32	Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation: a Nordic Multicentre Study. ( 29301479 )	LindstrAPm L....Bergquist A.	2018
33	An Imaging Biomarker for Assessing Hepatic Function in Patients with Primary Sclerosing Cholangitis. ( 29775791 )	Schulze J....Ringe K.I.	2018
34	Primary sclerosing cholangitis is associated with abnormalities in CFTR. ( 29807875 )	Werlin S....Ferec C.	2018
35	Crohn's Disease Behavior and Location is Altered when Associated with Primary Sclerosing Cholangitis. ( 29333790 )	Iny O....Maharshak N.	2018
36	High Risk of Advanced Colorectal Neoplasia in Patients With Primary Sclerosing Cholangitis Associated With Inflammatory Bowel Disease. ( 29378311 )	Shah S.C....Torres J.	2018
37	Association between smoking and risk of primary sclerosing cholangitis: A systematic review and meta-analysis. ( 29881604 )	Wijarnpreecha K....Ungprasert P.	2018
38	The impact of ileal pouch-anal anastomosis on graft survival following liver transplantation for primary sclerosing cholangitis. ( 29882252 )	Trivedi P.J....Ferguson J.	2018
39	Editorial: the role of colonic inflammation in the progression of liver disease in patients with primary sclerosing cholangitis. ( 29314124 )	Sabino J....Torres J.	2018
40	Loss of tolerance to gut immunity protein, glycoprotein 2 (GP2) is associated with progressive disease course in primary sclerosing cholangitis. ( 29321484 )	Tornai T....Papp M.	2018
41	No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients With Primary Sclerosing Cholangitis. ( 29803836 )	Ponsioen C.Y....Aabakken L.	2018
42	Quality of life and primary sclerosing cholangitis: the business of defining what counts. ( 29315689 )	Arndtz K....Hirschfield G.M.	2018
43	The Immunobiology of RANKL and Myeloid Derived Suppressor Cell Activation in IgG4-related Sclerosing Cholangitis. ( 29774578 )	Lian M....Ma X.	2018
44	Autoimmune hepatitis with sclerosing cholangitis in a patient with thiopurine methyltransferase deficiency: case presentation. ( 28523321 )	Man S.C....Gheban D.	2017
45	Very Severe Secondary Sclerosing Cholangitis as a Sequela of Influenza. ( 28683859 )	Pischke S....Lohse A.W.	2017
46	Increased cholestatic enzymes in two patients with long-term history of ulcerative colitis: consider primary biliary cholangitis not always primary sclerosing cholangitis. ( 28951510 )	Polychronopoulou E....Dalekos G.N.	2017
47	Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report. ( 28202062 )	Rungsakulkij N....Tannaphai P.	2017
48	Genetic association analysis identifies variants associated with disease progression in primary sclerosing cholangitis. ( 28779025 )	Alberts R....Weersma R.K.	2017
49	Efficacy of oral vancomycin in recurrent primary sclerosing cholangitis following liver transplantation. ( 28951512 )	Hey P....Gow P.	2017
50	Primary sclerosing cholangitis: A review and update. ( 29977644 )	Tabibian J.H....Bowlus C.L.	2017

Sources

Genes for Sclerosing Cholangitis

Genes related to Sclerosing Cholangitis (0 elite genes):

(show all 18)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence
1	CLDN1	Claudin 1	Protein Coding	53.55	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders Publications Summaries Variants (show sections)
2	DCDC2	Doublecortin Domain Containing 2	Protein Coding	43.27	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders Publications Variants (show sections)
3	ABCB4	ATP Binding Cassette Subfamily B Member 4	Protein Coding	24.38	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
4	GPBAR1	G Protein-Coupled Bile Acid Receptor 1	Protein Coding	23.6	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
5	HLA-DRB1	Major Histocompatibility Complex, Class II, DR Beta 1	Protein Coding	23.14	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
6	FUT2	Fucosyltransferase 2	Protein Coding	23.1	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
7	HLA-B	Major Histocompatibility Complex, Class I, B	Protein Coding	22.92	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
8	CFTR	Cystic Fibrosis Transmembrane Conductance Regulator	Protein Coding	22.84	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
9	ALB	Albumin	Protein Coding	22.83	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
10	NR1I2	Nuclear Receptor Subfamily 1 Group I Member 2	Protein Coding	22.57	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
11	CTSG	Cathepsin G	Protein Coding	22.42	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
12	TNF	Tumor Necrosis Factor	Protein Coding	22.34	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
13	CD40LG	CD40 Ligand	Protein Coding	22.33	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
14	BPI	Bactericidal Permeability Increasing Protein	Protein Coding	22.26	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
15	ABCB11	ATP Binding Cassette Subfamily B Member 11	Protein Coding	22.26	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
16	MPO	Myeloperoxidase	Protein Coding	22.19	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
17	HLA-C	Major Histocompatibility Complex, Class I, C	Protein Coding	22.14	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
18	GGT1	Gamma-Glutamyltransferase 1	Protein Coding	13.37	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (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:

(show all 11)

#	Super pathways	Score	Top Affiliating Genes
1	Allograft rejection ¹ ³⁷ Show member pathways Antigen processing and presentation ³⁷ Asthma ³⁷ Autoimmune thyroid disease ³⁷ CTL Mediated Apoptosis ⁶⁴ Graft-versus-host disease ³⁷ Intestinal immune network for IgA production ³⁷ THC Differentiation Pathway ⁶⁴ Type I diabetes mellitus ³⁷ Viral myocarditis ³⁷	12.6	CD40LG HLA-B HLA-C HLA-DRB1 TNF
2	HTLV-I infection ³⁷	12.08	HLA-B HLA-C HLA-DRB1 TNF
3	Folate Metabolism ¹ Show member pathways Selenium Micronutrient Network ¹ thioredoxin pathway ¹ Vitamin B12 Metabolism ¹	11.89	ALB GGT1 MPO TNF
4	Phagosome ³⁷	11.64	HLA-B HLA-C HLA-DRB1 MPO
5	Bile secretion ³⁷	11.35	ABCB11 ABCB4 CFTR
6	Cell adhesion molecules (CAMs) ³⁷	11.18	CD40LG CLDN1 HLA-B HLA-C HLA-DRB1
7	Farnesoid X Receptor Pathway ¹	10.77	ABCB11 ABCB4
8	Drug Induction of Bile Acid Pathway ¹	10.71	ABCB11 NR1I2
9	Nuclear Receptors in Lipid Metabolism and Toxicity ¹	10.68	ABCB11 ABCB4 NR1I2
10	LDL Oxidation in Atherogenesis ⁶⁴	10.64	MPO TNF
11	Etoposide Pathway, Pharmacokinetics/Pharmacodynamics ⁶¹	10.58	MPO NR1I2

Sources

GO Terms for Sclerosing Cholangitis

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

(show all 11)

#	Name	GO ID	Score	Top Affiliating Genes
1	integral component of plasma membrane	GO:0005887	9.86	ABCB11 ABCB4 BPI CD40LG CFTR CLDN1
2	azurophil granule lumen	GO:0035578	9.65	BPI CTSG MPO
3	recycling endosome membrane	GO:0055038	9.58	CFTR HLA-B HLA-C
4	ER to Golgi transport vesicle membrane	GO:0012507	9.54	HLA-B HLA-C HLA-DRB1
5	cell surface	GO:0009986	9.5	CD40LG CFTR CTSG HLA-B HLA-C HLA-DRB1
6	MHC class I protein complex	GO:0042612	9.46	HLA-B HLA-C
7	extracellular exosome	GO:0070062	9.4	ABCB11 ABCB4 ALB BPI CFTR CTSG
8	integral component of lumenal side of endoplasmic reticulum membrane	GO:0071556	9.33	HLA-B HLA-C HLA-DRB1
9	intercellular canaliculus	GO:0046581	9.32	ABCB11 ABCB4
10	integral component of membrane	GO:0016021	10.27	ABCB11 ABCB4 CD40LG CFTR CLDN1 FUT2
11	plasma membrane	GO:0005886	10.21	ABCB11 ABCB4 CD40LG CFTR CLDN1 CTSG

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

(show all 12)

#	Name	GO ID	Score	Top Affiliating Genes
1	neutrophil degranulation	GO:0043312	9.85	BPI CTSG HLA-B HLA-C MPO
2	defense response to bacterium	GO:0042742	9.71	BPI CTSG MPO TNF
3	response to lipopolysaccharide	GO:0032496	9.67	ABCB4 CLDN1 CTSG MPO
4	interferon-gamma-mediated signaling pathway	GO:0060333	9.61	HLA-B HLA-C HLA-DRB1
5	response to dexamethasone	GO:0071548	9.51	ABCB4 CLDN1
6	antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent	GO:0002480	9.49	HLA-B HLA-C
7	xenobiotic transport	GO:0042908	9.48	ABCB4 NR1I2
8	antigen processing and presentation	GO:0019882	9.33	HLA-B HLA-C HLA-DRB1
9	drug export	GO:0046618	9.32	ABCB4 NR1I2
10	regulation of immunoglobulin secretion	GO:0051023	9.26	CD40LG TNF
11	immune response	GO:0006955	9.17	BPI CD40LG CTSG HLA-B HLA-C HLA-DRB1
12	negative regulation of growth of symbiont in host	GO:0044130	9.13	CTSG MPO TNF

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	ABCB11 ABCB4 CFTR
2	peptide antigen binding	GO:0042605	8.8	HLA-B HLA-C HLA-DRB1

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 ExPASy

¹⁸ FDA Approved Drugs

¹⁹ FMA

²⁰ GeneAnalytics

²¹ GeneCards

²² GeneGo (Thomson Reuters)

²³ GeneHancer via GeneCards

²⁴ GeneReviews

²⁵ Genetics Home Reference

²⁶ GenomeRNAi

²⁷ GEO DataSets

²⁸ GO

²⁹ GTR

³⁰ HGMD

³¹ HMDB

³² HPO

³³ ICD10

³⁴ ICD10 via Orphanet

³⁵ ICD9CM

³⁶ IUPHAR

³⁷ KEGG

³⁸ LifeMap

³⁹ LncRNADisease

⁴⁰ LOVD

⁴¹ MalaCards

⁴² MedGen

⁴³ MedlinePlus

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 NIH Rare Diseases

⁵⁴ NINDS

⁵⁵ Novoseek

⁵⁶ Novus Biologicals

⁵⁷ OMIM

⁵⁸ OMIM via Orphanet

⁵⁹ Orphanet

⁶⁰ PathCards

⁶¹ PharmGKB

⁶² PubMed

⁶³ PubMed Health

⁶⁴ QIAGEN

⁶⁵ R&D Systems

⁶⁶ Reactome

⁶⁷ Sino Biological

⁶⁸ SNOMED-CT

⁶⁹ SNOMED-CT via HPO

⁷⁰ SNOMED-CT via Orphanet

⁷¹ TGDB

⁷² Tocris

⁷³ UMLS

⁷⁴ UMLS via Orphanet

⁷⁵ UniProtKB/Swiss-Prot

⁷⁶ Wikipedia