PSC
MCID: CHL119
MIFTS: 56

Cholangitis, Primary Sclerosing (PSC)

Categories: Gastrointestinal diseases, Liver diseases, Rare diseases
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Aliases & Classifications for Cholangitis, Primary Sclerosing

MalaCards integrated aliases for Cholangitis, Primary Sclerosing:

Name: Cholangitis, Primary Sclerosing 57 19 75
Primary Sclerosing Cholangitis 11 19 42 58 75 28 14 71 33
Sclerosing Cholangitis 19 42 33
Psc 57 42 58
Cholangitis, Sclerosing 43 71
Psc - [primary Sclerosing Cholangitis] 33
Cholangitis Primary Sclerosing 53

Characteristics:


Inheritance:

Primary Sclerosing Cholangitis: Multigenic/multifactorial 58

Prevelance:

Primary Sclerosing Cholangitis: 1-9/1000000 (Europe, Worldwide, Netherlands, Canada, Spain) 1-9/100000 (Europe, Worldwide, Norway, Norway, Sweden, Netherlands, Singapore) 1-5/10000 (Sweden) <1/1000000 (Spain) 58

Age Of Onset:

Primary Sclerosing Cholangitis: Adolescent,Adult,Childhood,Elderly 58

Classifications:

Orphanet: 58  
Rare hepatic diseases


Summaries for Cholangitis, Primary Sclerosing

MedlinePlus Genetics: 42 Primary sclerosing cholangitis is a condition that affects the bile ducts. These ducts carry bile (a fluid that helps to digest fats) from the liver, where bile is produced, to the gallbladder, where it is stored, and to the small intestine, where it aids in digestion. Primary sclerosing cholangitis occurs because of inflammation in the bile ducts (cholangitis) that leads to scarring (sclerosis) and narrowing of the ducts. As a result, bile cannot be released to the gallbladder and small intestine, and it builds up in the liver.Primary sclerosing cholangitis is usually diagnosed around age 40, and for unknown reasons, it affects men twice as often as women. Many people have no signs or symptoms of the condition when they are diagnosed, but routine blood tests reveal liver problems. When apparent, the earliest signs and symptoms of primary sclerosing cholangitis include extreme tiredness (fatigue), discomfort in the abdomen, and severe itchiness (pruritus). As the condition worsens, affected individuals may develop yellowing of the skin and whites of the eyes (jaundice) and an enlarged spleen (splenomegaly). Eventually, the buildup of bile damages the liver cells, causing chronic liver disease (cirrhosis) and liver failure. Without bile available to digest them, fats pass through the body. As a result, weight loss and shortages of vitamins that are absorbed with and stored in fats (fat-soluble vitamins) can occur. A fat-soluble vitamin called vitamin D helps absorb calcium and helps bones harden, and lack of this vitamin can cause thinning of the bones (osteoporosis) in people with primary sclerosing cholangitis.Primary sclerosing cholangitis is often associated with another condition called inflammatory bowel disease, which is characterized by inflammation of the intestines that causes open sores (ulcers) in the intestines and abdominal pain. However, the reason for this link is unclear. Approximately 70 percent of people with primary sclerosing cholangitis have inflammatory bowel disease, most commonly a form of the condition known as ulcerative colitis. In addition, people with primary sclerosing cholangitis are more likely to have an autoimmune disorder, such as type 1 diabetes, celiac disease, or thyroid disease, than people without the condition. Autoimmune disorders occur when the immune system malfunctions and attacks the body's tissues and organs. People with primary sclerosing cholangitis also have an increased risk of developing cancer, particularly cancer of the bile ducts (cholangiocarcinoma).

MalaCards based summary: Cholangitis, Primary Sclerosing, also known as primary sclerosing cholangitis, is related to portal hypertension and autoimmune pancreatitis, and has symptoms including icterus An important gene associated with Cholangitis, Primary Sclerosing is SEMA4D (Semaphorin 4D), and among its related pathways/superpathways are Defensins and Etoposide Pathway, Pharmacokinetics/Pharmacodynamics. The drugs Bezafibrate and Simvastatin have been mentioned in the context of this disorder. Affiliated tissues include liver, small intestine and thyroid, and related phenotypes are autoimmunity and portal hypertension

GARD: 19 Primary sclerosing cholangitis (PSC) is characterized by inflammation in the bile ducts (cholangitis) that leads to scarring (sclerosis), narrowing of the ducts, and a buildup of bile in the liver. Early signs and symptoms include extreme tiredness, abdominal pain, and itchiness. Other complications may include weight loss, vitamin deficiency, and osteoporosis. Many people with PSC develop other autoimmune conditions such as inflammatory bowel disease, type 1 diabetes, celiac disease, or thyroid disease. PSC is also a risk factor for cancer of the bile ducts (cholangiocarcinoma).

OMIM®: 57 Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease characterized by fibroobliterative inflammation of the biliary tract, leading to cirrhosis and portal hypertension. It is a major indication for liver transplantation (Sheth et al., 2003). Approximately 75 to 80% of PSC cases are associated with inflammatory bowel disease (IBD; see 266600), and 2.5 to 7.5% of patients with IBD develop PSC (Lee and Kaplan, 1995). (613806) (Updated 08-Dec-2022)

Orphanet: 58 Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure.

Disease Ontology: 11 A sclerosing cholangitis characterized by fibroobliterative inflammation of the biliary tract, leading to cirrhosis and portal hypertension.

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

Related Diseases for Cholangitis, Primary Sclerosing

Diseases in the Sclerosing Cholangitis family:

Cholangitis, Primary Sclerosing Secondary Sclerosing Cholangitis

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

(show top 50) (show all 775)
# Related Disease Score Top Affiliating Genes
1 portal hypertension 32.1 GPT GGT1 CCR6
2 autoimmune pancreatitis 31.8 ENO1 CFTR CCR6
3 choledocholithiasis 31.6 GPT GGT1 ABCB4
4 gallbladder disease 31.6 GPT CFTR CCR6 ABCB4
5 autoimmune cholangitis 31.5 DLAT CCR6 ABCB4
6 acute cholangitis 31.5 GPT ABCB4
7 biliary tract disease 31.3 KRT19 GPT CCR6 ABCB4
8 cystic fibrosis 31.3 PRTN3 MPO CTSG CFTR BPI ABCB4
9 cholestasis 31.2 NR1I2 GPT GGT1 ABCB4
10 vasculitis 31.2 PRTN3 MPO CTSG BPI
11 biliary atresia 31.2 GPT GGT1 ABCB4
12 respiratory failure 31.2 MPO GPT CFTR CCR6
13 chronic cholangitis 31.2 GPT DLAT ABCB4
14 inflammatory bowel disease 31.1 PRTN3 NR1I2 MST1 MPO MADCAM1 ENO1
15 acute pancreatitis 31.1 MPO GPT CFTR
16 suppurative cholangitis 31.0 GPT DLAT ABCB4
17 extrahepatic cholestasis 31.0 GPT GGT1 ABCB4
18 alpha-1-antitrypsin deficiency 31.0 PRTN3 MPO GPT CFTR BPI
19 sclerosing cholangitis 30.9 NR1I2 MADCAM1 KRT19 GPT DLAT CCR6
20 cholangitis 30.9 PRTN3 NR1I2 MPO MADCAM1 GPT GGT1
21 peritonitis 30.9 PRTN3 MPO CTSG
22 primary biliary cholangitis 30.9 PRTN3 NR1I2 KRT19 GPT GGT1 ENO1
23 ileitis 30.8 MPO MADCAM1 CCR6
24 progressive familial intrahepatic cholestasis 30.8 NR1I2 CFTR ABCB4
25 exanthem 30.8 PRTN3 MPO GPT CCR6
26 autoimmune hepatitis 30.8 HLA-A GPT GGT1 DLAT
27 bilirubin metabolic disorder 30.8 NR1I2 GPT GGT1 CFTR ABCB4
28 autoimmune disease 30.8 PRTN3 HLA-A ENO1 DLAT CCR6
29 polyarteritis nodosa 30.8 PRTN3 MPO GPT
30 liver disease 30.7 NR1I2 KRT19 GPT GGT1 DLAT ABCB4
31 bronchiectasis 30.7 MPO CTSG CFTR BPI
32 skin disease 30.7 PRTN3 MPO HLA-A GPT CCR6
33 granulomatosis with polyangiitis 30.7 PRTN3 MPO CTSG CCR6 BPI
34 alcohol use disorder 30.7 GPT GGT1 CCR6
35 igg4-related disease 30.6 PRTN3 MPO CCR6
36 viral hepatitis 30.6 MPO KRT19 GPT GGT1
37 syphilis 30.6 PRTN3 GPT CCR6
38 behcet syndrome 30.6 PRTN3 MPO HLA-A ENO1 CCR6
39 microscopic polyangiitis 30.6 PRTN3 MPO
40 polycystic liver disease 30.5 KRT19 CFTR ABCB4
41 mastoiditis 30.5 PRTN3 MPO
42 cholecystolithiasis 30.5 GPT ABCB4
43 ascending cholangitis 30.5 MADCAM1 GPT ABCB4
44 bile duct cysts 30.4 KRT19 GPT ABCB4
45 intrahepatic cholestasis 30.4 NR1I2 GPT GGT1 ABCB4
46 intrahepatic cholestasis of pregnancy 30.4 NR1I2 GPT ABCB4
47 bile duct disease 30.4 NR1I2 KRT19 GPT CCR6 ABCB4
48 pericholangitis 30.4 GPT ABCB4
49 hashimoto thyroiditis 30.2 KRT19 ENO1 CCR6
50 ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis 12.0

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



Diseases related to Cholangitis, Primary Sclerosing

Symptoms & Phenotypes for Cholangitis, Primary Sclerosing

Human phenotypes related to Cholangitis, Primary Sclerosing:

58 30 (show top 50) (show all 55)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 autoimmunity 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002960
2 portal hypertension 58 30 Frequent (33%) Frequent (79-30%)
HP:0001409
3 fever 58 30 Frequent (33%) Frequent (79-30%)
HP:0001945
4 ascites 58 30 Frequent (33%) Frequent (79-30%)
HP:0001541
5 elevated hepatic transaminase 58 30 Frequent (33%) Frequent (79-30%)
HP:0002910
6 hepatic fibrosis 58 30 Frequent (33%) Frequent (79-30%)
HP:0001395
7 cirrhosis 58 30 Frequent (33%) Frequent (79-30%)
HP:0001394
8 weight loss 58 30 Frequent (33%) Frequent (79-30%)
HP:0001824
9 hepatosplenomegaly 58 30 Frequent (33%) Frequent (79-30%)
HP:0001433
10 palmar telangiectasia 58 30 Frequent (33%) Frequent (79-30%)
HP:0100869
11 abnormal large intestine physiology 58 30 Frequent (33%) Frequent (79-30%)
HP:0012700
12 elevated alkaline phosphatase of hepatic origin 58 30 Frequent (33%) Frequent (79-30%)
HP:0010638
13 spider hemangioma 58 30 Frequent (33%) Frequent (79-30%)
HP:0012522
14 dilated superficial abdominal veins 58 30 Frequent (33%) Frequent (79-30%)
HP:0030168
15 ulcerative colitis 58 30 Frequent (33%) Frequent (79-30%)
HP:0100279
16 depression 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000716
17 osteopenia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000938
18 fatigue 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012378
19 type i diabetes mellitus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100651
20 renal insufficiency 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000083
21 congestive heart failure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001635
22 hepatitis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012115
23 osteoporosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000939
24 celiac disease 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002608
25 thyroiditis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100646
26 cholelithiasis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001081
27 jaundice 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000952
28 abdominal pain 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002027
29 low levels of vitamin d 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100512
30 low levels of vitamin e 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100513
31 hypoalbuminemia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003073
32 low levels of vitamin a 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0004905
33 prolonged prothrombin time 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0008151
34 pruritus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000989
35 generalized amyotrophy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003700
36 pleural effusion 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002202
37 pancreatitis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001733
38 low levels of vitamin k 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0011892
39 chronic hepatic failure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100626
40 hepatocellular carcinoma 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001402
41 cholangiocarcinoma 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0030153
42 polyclonal elevation of igm 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003459
43 adenocarcinoma of the large intestine 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0040275
44 uveitis 58 30 Very rare (1%) Very rare (<4-1%)
HP:0000554
45 encephalopathy 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001298
46 acute hepatic failure 58 30 Very rare (1%) Very rare (<4-1%)
HP:0006554
47 neoplasm of the gallbladder 58 30 Very rare (1%) Very rare (<4-1%)
HP:0100575
48 splenomegaly 58 Frequent (79-30%)
49 hepatomegaly 58 Frequent (79-30%)
50 abnormality of eosinophils 58 Excluded (0%)

Clinical features from OMIM®:

613806 (Updated 08-Dec-2022)

UMLS symptoms related to Cholangitis, Primary Sclerosing:


icterus

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

25 (show all 37)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-103 10.01 CTSG GPR35
2 Increased shRNA abundance (Z-score > 2) GR00366-A-105 10.01 GPR35
3 Increased shRNA abundance (Z-score > 2) GR00366-A-106 10.01 DLAT
4 Increased shRNA abundance (Z-score > 2) GR00366-A-111 10.01 CTSG
5 Increased shRNA abundance (Z-score > 2) GR00366-A-118 10.01 HLA-A
6 Increased shRNA abundance (Z-score > 2) GR00366-A-119 10.01 PRTN3
7 Increased shRNA abundance (Z-score > 2) GR00366-A-129 10.01 GPR35
8 Increased shRNA abundance (Z-score > 2) GR00366-A-13 10.01 CTSG
9 Increased shRNA abundance (Z-score > 2) GR00366-A-133 10.01 PRTN3
10 Increased shRNA abundance (Z-score > 2) GR00366-A-145 10.01 CTSG
11 Increased shRNA abundance (Z-score > 2) GR00366-A-146 10.01 DLAT KRT19
12 Increased shRNA abundance (Z-score > 2) GR00366-A-147 10.01 TCF4
13 Increased shRNA abundance (Z-score > 2) GR00366-A-149 10.01 CTSG GPR35
14 Increased shRNA abundance (Z-score > 2) GR00366-A-152 10.01 PRTN3
15 Increased shRNA abundance (Z-score > 2) GR00366-A-161 10.01 CTSG
16 Increased shRNA abundance (Z-score > 2) GR00366-A-166 10.01 DLAT
17 Increased shRNA abundance (Z-score > 2) GR00366-A-169 10.01 KRT19
18 Increased shRNA abundance (Z-score > 2) GR00366-A-177 10.01 KRT19
19 Increased shRNA abundance (Z-score > 2) GR00366-A-179 10.01 PRTN3
20 Increased shRNA abundance (Z-score > 2) GR00366-A-180 10.01 TCF4
21 Increased shRNA abundance (Z-score > 2) GR00366-A-198 10.01 GPR35
22 Increased shRNA abundance (Z-score > 2) GR00366-A-199 10.01 GPR35
23 Increased shRNA abundance (Z-score > 2) GR00366-A-209 10.01 PRTN3
24 Increased shRNA abundance (Z-score > 2) GR00366-A-21 10.01 CTSG
25 Increased shRNA abundance (Z-score > 2) GR00366-A-211 10.01 CTSG
26 Increased shRNA abundance (Z-score > 2) GR00366-A-34 10.01 KRT19
27 Increased shRNA abundance (Z-score > 2) GR00366-A-43 10.01 DLAT KRT19
28 Increased shRNA abundance (Z-score > 2) GR00366-A-46 10.01 TCF4
29 Increased shRNA abundance (Z-score > 2) GR00366-A-48 10.01 TCF4
30 Increased shRNA abundance (Z-score > 2) GR00366-A-5 10.01 DLAT
31 Increased shRNA abundance (Z-score > 2) GR00366-A-74 10.01 HLA-A
32 Increased shRNA abundance (Z-score > 2) GR00366-A-75 10.01 KRT19
33 Increased shRNA abundance (Z-score > 2) GR00366-A-76 10.01 TCF4
34 Increased shRNA abundance (Z-score > 2) GR00366-A-77 10.01 KRT19
35 Increased shRNA abundance (Z-score > 2) GR00366-A-82 10.01 PRTN3
36 Increased shRNA abundance (Z-score > 2) GR00366-A-89 10.01 GPR35
37 Increased shRNA abundance (Z-score > 2) GR00366-A-99 10.01 PRTN3

MGI Mouse Phenotypes related to Cholangitis, Primary Sclerosing:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.8 ABCB4 CCR6 CFTR CTSG GGT1 GPR35
2 hematopoietic system MP:0005397 9.4 ABCB4 CCR6 CFTR CTSG GGT1 HLA-A

Drugs & Therapeutics for Cholangitis, Primary Sclerosing

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

(show top 50) (show all 75)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
2
Simvastatin Approved Phase 3 79902-63-9 54454
3
Ursodeoxycholic acid Approved, Investigational Phase 2, Phase 3 128-13-2 31401
4
Clofibric Acid Phase 3 882-09-7 2797
5 Anticholesteremic Agents Phase 3
6 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 3
7
Mitomycin Approved Phase 2 50-07-7 5746
8
Curcumin Approved, Investigational Phase 1, Phase 2 458-37-7, 84765-67-3 969516
9
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
10
Aldesleukin Approved Phase 2 110942-02-4
11
Sulfasalazine Approved Phase 2 599-79-1 5353980 5339 5359476
12
Thalidomide Approved, Investigational, Withdrawn Phase 2 50-35-1 5426
13
Fenofibrate Approved Phase 1, Phase 2 49562-28-9 3339
14
Isotretinoin Approved, Investigational, Nutraceutical Phase 2 302-79-4, 4759-48-2 5538 444795 5282379
15
Cenicriviroc Investigational Phase 2 497223-25-3 6320532 11285792
16
Seladelpar Investigational Phase 2 851528-79-5 11476297 11236126
17
Volixibat Experimental, Investigational Phase 2 1025216-57-2
18 Antibiotics, Antitubercular Phase 2
19 Alkylating Agents Phase 2
20 Hormones Phase 2
21 Calcium, Dietary Phase 2
22 Antiviral Agents Phase 2
23 Anti-Retroviral Agents Phase 2
24 Anti-HIV Agents Phase 2
25 Immunoglobulins Phase 2
26 Antibodies, Monoclonal Phase 2
27 Antibodies Phase 2
28 2-chloro-3'-deoxyadenosine Phase 2 14485373
29 Interleukin-2 Phase 2
30 Analgesics Phase 2
31 Antirheumatic Agents Phase 2
32 Anti-Inflammatory Agents, Non-Steroidal Phase 2
33 Analgesics, Non-Narcotic Phase 2
34 Anti-Inflammatory Agents Phase 2
35 Bile Acids and Salts Phase 2
36 Lipid Regulating Agents Phase 1, Phase 2
37 Antimetabolites Phase 1, Phase 2
38 Hypolipidemic Agents Phase 1, Phase 2
39 Angiogenesis Inhibitors Phase 2
40 Immunosuppressive Agents Phase 2
41 Immunologic Factors Phase 2
42 Keratolytic Agents Phase 2
43 Dermatologic Agents Phase 2
44
Calcium Nutraceutical Phase 2 7440-70-2 271
45
Metronidazole Approved Phase 1 443-48-1, 69198-10-3 4173
46
Minocycline Approved, Investigational Phase 1 10118-90-8, 13614-98-7 54675783 5281021
47
Rifaximin Approved, Investigational Phase 1 80621-81-4 46783403 6436173
48
Erlotinib Approved, Investigational Phase 1 183319-69-9, 183321-74-6 176870
49
Budesonide Approved Phase 1 51333-22-3 5281004 40000 63006
50
Rituximab Approved Phase 1 174722-31-7

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 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 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
25 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
26 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
27 Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis. Completed NCT02424175 Phase 1, Phase 2
28 Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis Completed NCT00004762 Phase 2 cladribine
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 A Randomized, Placebo-controlled Pilot Study of Sulfasalazine for the Treatment of Primary Sclerosing Cholangitis (PSC) Recruiting NCT03561584 Phase 2 Sulfasalazine;Placebo
34 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
35 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
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 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
41 Open Label, Phase II Investigation of Thalidomide for the Treatment of Primary Sclerosing Cholangitis Terminated NCT00953615 Phase 2 Thalidomide
42 Pilot Study of Fenofibrate in Primary Sclerosing Cholangitis Terminated NCT01142323 Phase 1, Phase 2 fenofibrate
43 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
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 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
47 Hepatic Transport of Conjugated Bile Acids in Humans Quantified by 11C-cholylsarcosine PET/CT Completed NCT01879735 Phase 1 11C-CSar;ICG
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 Cholangitis, Primary Sclerosing

Cochrane evidence based reviews: cholangitis, sclerosing

Genetic Tests for Cholangitis, Primary Sclerosing

Genetic tests related to Cholangitis, Primary Sclerosing:

# Genetic test Affiliating Genes
1 Primary Sclerosing Cholangitis 28

Anatomical Context for Cholangitis, Primary Sclerosing

Organs/tissues related to Cholangitis, Primary Sclerosing:

MalaCards : Liver, Small Intestine, Thyroid, Pancreas, Spleen, Skin, T Cells

Publications for Cholangitis, Primary Sclerosing

Articles related to Cholangitis, Primary Sclerosing:

(show top 50) (show all 6977)
# Title Authors PMID Year
1
Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis. 53 62 57
12783301 2003
2
Genome-wide association analysis in primary sclerosing cholangitis identifies two non-HLA susceptibility loci. 62 57
21151127 2011
3
Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease. 62 57
18667695 2008
4
Hepatic endothelial CCL25 mediates the recruitment of CCR9+ gut-homing lymphocytes to the liver in primary sclerosing cholangitis. 62 57
15557349 2004
5
Primary sclerosing cholangitis. 62 57
7877651 1995
6
Gene-centric association mapping of chromosome 3p implicates MST1 in IBD pathogenesis. 57
19079170 2008
7
New insights into autoimmune cholangitis through animal models. 53 62
20460897 2010
8
Gut homing receptors on CD8 T cells are retinoic acid dependent and not maintained by liver dendritic or stellate cells. 53 62
19233184 2009
9
Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis. 53 62
18992954 2009
10
PXR: a xenobiotic receptor of diverse function implicated in pharmacogenetics. 53 62
19018724 2008
11
Disturbed colocalization of multidrug resistance protein 2 and radixin in human cholestatic liver diseases. 53 62
17725603 2008
12
What is the role of cystic fibrosis transmembrane conductance regulator dysfunction in primary sclerosing cholangitis? 53 62
17719926 2007
13
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. 53 62
17719933 2007
14
High aspartate to alanine aminotransferase ratio is an indicator of cirrhosis and poor outcome in patients with primary sclerosing cholangitis. 53 62
17498256 2007
15
Mucin-producing bile duct carcinoma arising from primary sclerosing cholangitis: a case report. 53 62
17384480 2007
16
MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes. 53 62
17295178 2007
17
Analysis of MAdCAM-1 and ICAM-1 polymorphisms in 365 Scandinavian patients with primary sclerosing cholangitis. 53 62
16750586 2006
18
Polymorphisms in the steroid and xenobiotic receptor gene influence survival in primary sclerosing cholangitis. 53 62
16952547 2006
19
Serum reactivity against bacterial pyruvate dehydrogenase: increasing the specificity of anti-mitochondrial antibodies for the diagnosis of primary biliary cirrhosis. 53 62
17162370 2006
20
Cholestasis and cholestatic syndromes. 53 62
16550034 2006
21
Expression of epithelial cellular adhesion molecule (Ep-CAM) in chronic (necro-)inflammatory liver diseases and hepatocellular carcinoma. 53 62
16364680 2006
22
Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis. 53 62
15784035 2005
23
Induction of colitis in cftr-/- mice results in bile duct injury. 53 62
15064232 2004
24
BSEP and MDR3 haplotype structure in healthy Caucasians, primary biliary cirrhosis and primary sclerosing cholangitis. 53 62
14999697 2004
25
Site-characteristic expression and induction of trefoil factor family 1, 2 and 3 and malignant brain tumor-1 in normal and diseased intrahepatic bile ducts relates to biliary pathophysiology. 53 62
15101998 2004
26
Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population. 53 62
14567462 2003
27
Expression of deleted in malignant brain tumor-1 (DMBT1) molecule in biliary epithelium is augmented in hepatolithiasis: possible participation in lithogenesis. 53 62
12870778 2003
28
Heterogeneity of ductular reactions in adult rat and human liver revealed by novel expression of deleted in malignant brain tumor 1. 53 62
12368192 2002
29
Gamma-glutamyltransferase: value of its measurement in paediatrics. 53 62
11853185 2002
30
Expression of hepatic transporters OATP-C and MRP2 in primary sclerosing cholangitis. 53 62
11454187 2001
31
MAdCAM-1 expressed in chronic inflammatory liver disease supports mucosal lymphocyte adhesion to hepatic endothelium (MAdCAM-1 in chronic inflammatory liver disease). 53 62
11343233 2001
32
Autoimmune regulator AIRE: evidence for genetic differences between autoimmune hepatitis and hepatitis as part of the autoimmune polyglandular syndrome type 1. 53 62
11343230 2001
33
Overlap syndrome between autoimmune hepatitis and primary sclerosing cholangitis in two cases. 53 62
10833101 2000
34
Anti-neutrophil cytoplasmic antibodies in patients with chronic liver diseases: prevalence, antigen specificity and predictive value for diagnosis of autoimmune liver disease. Swedish Internal Medicine Liver Club (SILK) 53 62
10824890 2000
35
Reduced hepatic content of dehydroepiandrosterone sulphotransferase in chronic liver diseases. 53 62
10726960 2000
36
Hepatic jagged1 expression studies. 53 62
10534349 1999
37
Immunohistochemical study on phenotypical changes of hepatocytes in liver disease with reference to extracellular matrix composition. 53 62
9928763 1999
38
Biliary lactoferrin concentrations are increased in active inflammatory bowel disease: a factor in the pathogenesis of primary sclerosing cholangitis? 53 62
9791051 1998
39
Antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis: defined specificities may be associated with distinct clinical features. 53 62
9831423 1998
40
Human leukocyte antigen-C genes and susceptibility to primary sclerosing cholangitis. 53 62
9731555 1998
41
Identification and characterization of autoantibodies against catalase and alpha-enolase in patients with primary sclerosing cholangitis. 53 62
9649223 1998
42
Immunolocalization of putative human liver progenitor cells in livers from patients with end-stage primary biliary cirrhosis and sclerosing cholangitis using the monoclonal antibody OV-6. 53 62
9502419 1998
43
An atypical presentation for primary sclerosing cholangitis. 53 62
9365127 1997
44
Two spatially distant epitopes of human lactoferrin. 53 62
8880213 1996
45
Anti-lactoferrin autoantibodies: relation between epitopes and iron-binding domain. 53 62
8864834 1996
46
Anti-neutrophil cytoplasmic antibodies (ANCA) directed against bactericidal/permeability increasing protein (BPI): a new seromarker for inflammatory bowel disease and associated disorders. 53 62
8603534 1996
47
Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis. 53 62
8617131 1996
48
Distribution of lactoferrin and 60/65 kDa heat shock protein in normal and inflamed human intestine and liver. 53 62
8566841 1996
49
HLA-DR and HLA-DQ are not markers for rapid disease progression in primary sclerosing cholangitis. 53 62
7875491 1995
50
Biliary epithelial expression of pyruvate dehydrogenase complex in primary biliary cirrhosis: an immunohistochemical and immunoelectron microscopic study. 53 62
7821922 1995

Variations for Cholangitis, Primary Sclerosing

Expression for Cholangitis, Primary Sclerosing

Search GEO for disease gene expression data for Cholangitis, Primary Sclerosing.

Pathways for Cholangitis, Primary Sclerosing

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.24 PRTN3 CTSG CCR6 BPI
2 10.13 NR1I2 MPO

GO Terms for Cholangitis, Primary Sclerosing

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

# Name GO ID Score Top Affiliating Genes
1 azurophil granule lumen GO:0035578 9.23 PRTN3 MPO CTSG BPI

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.36 SEMA4D MADCAM1 HLA-A CTSG CCR6 BPI

Sources for Cholangitis, Primary Sclerosing

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57 OMIM® (Updated 08-Dec-2022)
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72 UMLS via Orphanet
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