PSC
MCID: CHL119
MIFTS: 57

Cholangitis, Primary Sclerosing (PSC)

Categories: Gastrointestinal diseases, Liver diseases, Rare diseases

Aliases & Classifications for Cholangitis, Primary Sclerosing

MalaCards integrated aliases for Cholangitis, Primary Sclerosing:

Name: Cholangitis, Primary Sclerosing 56 74 52 13
Primary Sclerosing Cholangitis 12 74 52 25 58 36 29 15 71
Psc 56 25 58
Cholangitis, Sclerosing 43 71
Sclerosing Cholangitis 52 25
Cholangitis Primary Sclerosing 54

Characteristics:

Orphanet epidemiological data:

58
primary sclerosing cholangitis
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/1000000 (Europe),1-5/10000 (Europe),1-9/100000 (Worldwide),1-9/1000000 (Worldwide),1-9/100000 (Norway),1-9/100000 (Sweden),1-5/10000 (Sweden),1-9/1000000 (Netherlands),1-9/100000 (Netherlands),1-9/1000000 (Canada),<1/1000000 (Spain),1-9/1000000 (Spain); Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare hepatic diseases


Summaries for Cholangitis, Primary Sclerosing

Genetics Home Reference : 25 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 cholangitis and sclerosing cholangitis, and has symptoms including icterus An important gene associated with Cholangitis, Primary Sclerosing is PSC (Cholangitis, Primary Sclerosing), and among its related pathways/superpathways are Defensins and Etoposide Pathway, Pharmacokinetics/Pharmacodynamics. The drugs Prednisone and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include liver, testes and thyroid, and related phenotypes are autoimmunity and splenomegaly

Disease Ontology : 12 A sclerosing cholangitis characterized by fibroobliterative inflammation of the biliary tract, leading to cirrhosis and portal hypertension.

NIH Rare Diseases : 52 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. As the condition worsens it may cause jaundice , an enlarged spleen, and eventually liver cirrhosis and failure. 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 : 56 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)

KEGG : 36 Primary sclerosing cholangitis (PSC) is a rare progressive disease of the liver characterized by cholestasis and bile duct fibrosis, leading to decreased quality of life, cirrhosis, and the need for liver transplantation within an average of 12 years. The disease occurs in children and adults, and is closely linked with inflammatory bowel disease (IBD), most often ulcerative colitis (UC). The clinical presentation is variable. Typical symptoms include right upper quadrant abdominal discomfort, fatigue, pruritus, and weight loss. The most frequent abnormal physical exam findings are jaundice, hepatomegaly, and splenomegaly. The pathogenesis of PSC remains unclear but hypotheses include genetic factors lymphocyte recruitment and activation, portal bacteraemia, and bile salt toxicity. Ursodeoxycholic acid (UDCA) is widely used for PSC patients and UDCA can lead to improvements in serum hepatobiliary enzymes. UDCA has been tested alone and in combination with corticosteroids or other immunosuppressant agents. However, there is no medical treatment for PSC with enough evidence to show a delay in disease progression, and new therapy is needed.

Wikipedia : 74 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 574)
# Related Disease Score Top Affiliating Genes
1 cholangitis 32.5 PRTN3 NR1I2 MPO MADCAM1 GPT GGT1
2 sclerosing cholangitis 32.5 PRTN3 NR1I2 MST1 MPO MADCAM1 GPT
3 cholestasis 32.0 NR1I2 GPT GGT1 ABCB4
4 ulcerative colitis 31.9 PRTN3 MPO CTSG CCR6 BPI
5 inflammatory bowel disease 31.7 PRTN3 NR1I2 MST1 MPO MADCAM1 ENO1
6 acute cholangitis 31.5 GPT GGT1
7 liver disease 31.5 KRT19 GPT GGT1 DLAT ABCB4
8 cholelithiasis 31.5 GPT GGT1 ABCB4
9 cystic fibrosis 31.5 PRTN3 MPO DMBT1 CTSG CFTR BPI
10 bile duct cysts 31.5 KRT19 GPT GGT1 ABCB4
11 biliary tract disease 31.4 NR1I2 KRT19 GPT GGT1 DLAT CCR6
12 gallbladder disease 31.4 GPT GGT1 CFTR CCR6 ABCB4
13 vasculitis 31.3 PRTN3 MPO CTSG BPI
14 autoimmune hepatitis 31.3 HLA-A GPT GGT1 DLAT
15 biliary atresia 31.3 GPT GGT1 ABCB4
16 chronic cholangitis 31.3 DLAT ABCB4
17 primary biliary cirrhosis 31.3 PRTN3 NR1I2 KRT19 HLA-A GPT GGT1
18 suppurative cholangitis 31.2 GPT GGT1 DLAT ABCB4
19 respiratory failure 31.2 MPO GPT CFTR CCR6
20 bilirubin metabolic disorder 31.2 GPT GGT1 ABCB4
21 autoimmune disease 31.2 PRTN3 HLA-A ENO1 DLAT CCR6
22 alpha-1-antitrypsin deficiency 31.2 PRTN3 MPO GPT CFTR
23 pneumonia 31.1 MPO GPT CFTR BPI
24 choledocholithiasis 31.0 GPT GGT1 ABCB4
25 extrahepatic cholestasis 31.0 MPO GPT GGT1 ABCB4
26 peritonitis 31.0 PRTN3 MPO CTSG
27 ileitis 30.9 MPO MADCAM1 CCR6
28 bronchiectasis 30.9 MPO CTSG CFTR BPI
29 polyarteritis nodosa 30.9 PRTN3 MPO GPT
30 syphilis 30.9 PRTN3 GPT CCR6
31 exanthem 30.9 PRTN3 MPO GPT CCR6
32 polycystic liver disease 30.8 KRT19 GGT1 CFTR
33 alcohol use disorder 30.8 GPT GGT1 CCR6
34 viral infectious disease 30.8 HLA-A GPT GGT1 CCR6
35 fatty liver disease, nonalcoholic 1 30.7 GPT GGT1
36 intrahepatic cholestasis of pregnancy 30.7 NR1I2 GPT ABCB4
37 bile duct disease 30.7 NR1I2 KRT19 GPT GGT1 DLAT CCR6
38 mastoiditis 30.7 PRTN3 MPO
39 viral hepatitis 30.6 KRT19 GPT GGT1
40 acute pancreatitis 30.2 MPO GPT CFTR
41 ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis 13.1
42 sclerosing cholangitis, neonatal 12.9
43 primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome 12.5
44 isolated neonatal sclerosing cholangitis 12.4
45 immunodeficiency 14 11.4
46 parenteral nutrition-associated cholestasis 10.8 GPT GGT1 ABCB4
47 purulent acute otitis media 10.7 PRTN3 MPO
48 common bile duct disease 10.7 GPT GGT1 ABCB4
49 cholangiocarcinoma 10.7
50 intrahepatic cholangiocarcinoma 10.7

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 31 (show top 50) (show all 55)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
2 splenomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0001744
3 hepatomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0002240
4 portal hypertension 58 31 frequent (33%) Frequent (79-30%) HP:0001409
5 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
6 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
7 ascites 58 31 frequent (33%) Frequent (79-30%) HP:0001541
8 cirrhosis 58 31 frequent (33%) Frequent (79-30%) HP:0001394
9 elevated hepatic transaminase 58 31 frequent (33%) Frequent (79-30%) HP:0002910
10 hepatic fibrosis 58 31 frequent (33%) Frequent (79-30%) HP:0001395
11 hepatosplenomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0001433
12 palmar telangiectasia 58 31 frequent (33%) Frequent (79-30%) HP:0100869
13 abnormal large intestine physiology 58 31 frequent (33%) Frequent (79-30%) HP:0012700
14 elevated alkaline phosphatase of hepatic origin 58 31 frequent (33%) Frequent (79-30%) HP:0010638
15 spider hemangioma 58 31 frequent (33%) Frequent (79-30%) HP:0012522
16 dilated superficial abdominal veins 58 31 frequent (33%) Frequent (79-30%) HP:0030168
17 ulcerative colitis 58 31 frequent (33%) Frequent (79-30%) HP:0100279
18 fatigue 58 31 occasional (7.5%) Occasional (29-5%) HP:0012378
19 type i diabetes mellitus 58 31 occasional (7.5%) Occasional (29-5%) HP:0100651
20 renal insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0000083
21 pruritus 58 31 occasional (7.5%) Occasional (29-5%) HP:0000989
22 congestive heart failure 58 31 occasional (7.5%) Occasional (29-5%) HP:0001635
23 hepatitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012115
24 abdominal pain 58 31 occasional (7.5%) Occasional (29-5%) HP:0002027
25 osteopenia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000938
26 osteoporosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000939
27 depressivity 58 31 occasional (7.5%) Occasional (29-5%) HP:0000716
28 celiac disease 58 31 occasional (7.5%) Occasional (29-5%) HP:0002608
29 thyroiditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100646
30 cholelithiasis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001081
31 jaundice 58 31 occasional (7.5%) Occasional (29-5%) HP:0000952
32 low levels of vitamin d 58 31 occasional (7.5%) Occasional (29-5%) HP:0100512
33 low levels of vitamin e 58 31 occasional (7.5%) Occasional (29-5%) HP:0100513
34 hypoalbuminemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003073
35 low levels of vitamin a 58 31 occasional (7.5%) Occasional (29-5%) HP:0004905
36 prolonged prothrombin time 58 31 occasional (7.5%) Occasional (29-5%) HP:0008151
37 pleural effusion 58 31 occasional (7.5%) Occasional (29-5%) HP:0002202
38 pancreatitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001733
39 generalized amyotrophy 58 31 occasional (7.5%) Occasional (29-5%) HP:0003700
40 low levels of vitamin k 58 31 occasional (7.5%) Occasional (29-5%) HP:0011892
41 chronic hepatic failure 58 31 occasional (7.5%) Occasional (29-5%) HP:0100626
42 hepatocellular carcinoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001402
43 cholangiocarcinoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0030153
44 polyclonal elevation of igm 58 31 occasional (7.5%) Occasional (29-5%) HP:0003459
45 adenocarcinoma of the large intestine 58 31 occasional (7.5%) Occasional (29-5%) HP:0040275
46 uveitis 58 31 very rare (1%) Very rare (<4-1%) HP:0000554
47 encephalopathy 58 31 very rare (1%) Very rare (<4-1%) HP:0001298
48 acute hepatic failure 58 31 very rare (1%) Very rare (<4-1%) HP:0006554
49 neoplasm of the gallbladder 58 31 very rare (1%) Very rare (<4-1%) HP:0100575
50 abnormality of eosinophils 58 Excluded (0%)

Clinical features from OMIM:

613806

UMLS symptoms related to Cholangitis, Primary Sclerosing:


icterus

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

26 (show all 41)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-103 9.83 CTSG
2 Increased shRNA abundance (Z-score > 2) GR00366-A-106 9.83 DLAT
3 Increased shRNA abundance (Z-score > 2) GR00366-A-111 9.83 CTSG
4 Increased shRNA abundance (Z-score > 2) GR00366-A-119 9.83 PRTN3
5 Increased shRNA abundance (Z-score > 2) GR00366-A-13 9.83 CTSG
6 Increased shRNA abundance (Z-score > 2) GR00366-A-133 9.83 PRTN3
7 Increased shRNA abundance (Z-score > 2) GR00366-A-145 9.83 CTSG
8 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.83 DLAT KRT19
9 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.83 TCF4
10 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.83 CTSG
11 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.83 PRTN3
12 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.83 CTSG
13 Increased shRNA abundance (Z-score > 2) GR00366-A-166 9.83 DLAT
14 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.83 KRT19
15 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.83 KRT19
16 Increased shRNA abundance (Z-score > 2) GR00366-A-179 9.83 PRTN3
17 Increased shRNA abundance (Z-score > 2) GR00366-A-180 9.83 TCF4
18 Increased shRNA abundance (Z-score > 2) GR00366-A-209 9.83 PRTN3
19 Increased shRNA abundance (Z-score > 2) GR00366-A-21 9.83 CTSG
20 Increased shRNA abundance (Z-score > 2) GR00366-A-211 9.83 CTSG
21 Increased shRNA abundance (Z-score > 2) GR00366-A-34 9.83 KRT19
22 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.83 DLAT KRT19
23 Increased shRNA abundance (Z-score > 2) GR00366-A-46 9.83 TCF4
24 Increased shRNA abundance (Z-score > 2) GR00366-A-48 9.83 TCF4
25 Increased shRNA abundance (Z-score > 2) GR00366-A-5 9.83 DLAT
26 Increased shRNA abundance (Z-score > 2) GR00366-A-75 9.83 KRT19
27 Increased shRNA abundance (Z-score > 2) GR00366-A-76 9.83 TCF4
28 Increased shRNA abundance (Z-score > 2) GR00366-A-77 9.83 KRT19
29 Increased shRNA abundance (Z-score > 2) GR00366-A-82 9.83 PRTN3
30 Increased shRNA abundance (Z-score > 2) GR00366-A-99 9.83 PRTN3
31 Decreased shRNA abundance (Z-score < -2) GR00366-A-103 9.7 HLA-A
32 Decreased shRNA abundance (Z-score < -2) GR00366-A-110 9.7 HLA-A
33 Decreased shRNA abundance (Z-score < -2) GR00366-A-131 9.7 GPR35
34 Decreased shRNA abundance (Z-score < -2) GR00366-A-143 9.7 GPR35
35 Decreased shRNA abundance (Z-score < -2) GR00366-A-156 9.7 HLA-A
36 Decreased shRNA abundance (Z-score < -2) GR00366-A-178 9.7 HLA-A
37 Decreased shRNA abundance (Z-score < -2) GR00366-A-19 9.7 HLA-A
38 Decreased shRNA abundance (Z-score < -2) GR00366-A-31 9.7 HLA-A
39 Decreased shRNA abundance (Z-score < -2) GR00366-A-43 9.7 CTSG
40 Decreased shRNA abundance (Z-score < -2) GR00366-A-7 9.7 HLA-A
41 Decreased shRNA abundance (Z-score < -2) GR00366-A-74 9.7 GPR35

MGI Mouse Phenotypes related to Cholangitis, Primary Sclerosing:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.4 ABCB4 CCR6 CFTR CTSG DMBT1 GGT1

Drugs & Therapeutics for Cholangitis, Primary Sclerosing

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

(show top 50) (show all 100)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
3
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
5
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
6 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
7
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
8
Ceftriaxone Approved Phase 4 73384-59-5 5361919 5479530
9
Levofloxacin Approved, Investigational Phase 4 100986-85-4 149096
10
Ofloxacin Approved Phase 4 82419-36-1 4583
11
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
12 Immunosuppressive Agents Phase 4
13 Immunologic Factors Phase 4
14 Anti-Inflammatory Agents Phase 4
15 Hormones Phase 4
16 Hormone Antagonists Phase 4
17 glucocorticoids Phase 4
18 Antineoplastic Agents, Hormonal Phase 4
19 Methylprednisolone Acetate Phase 4
20 Anti-Bacterial Agents Phase 4
21 Antibiotics, Antitubercular Phase 4
22 Cytochrome P-450 Enzyme Inhibitors Phase 4
23
Vancomycin Approved Phase 2, Phase 3 1404-90-6 14969 441141
24
Ursodeoxycholic acid Approved, Investigational Phase 3 128-13-2 31401
25
Simvastatin Approved Phase 3 79902-63-9 54454
26
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
27 Hypolipidemic Agents Phase 3
28 Lipid Regulating Agents Phase 3
29 Anticholesteremic Agents Phase 3
30 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 3
31 Clofibric Acid Phase 3 882-09-7
32
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
33
Curcumin Approved, Experimental, Investigational Phase 1, Phase 2 458-37-7 969516
34
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
35
Sulfasalazine Approved Phase 2 599-79-1 5353980 5359476
36
Aldesleukin Approved Phase 2 85898-30-2, 110942-02-4
37
Fenofibrate Approved Phase 1, Phase 2 49562-28-9 3339
38
Fluorouracil Approved Phase 2 51-21-8 3385
39
Pancrelipase Approved, Investigational Phase 2 53608-75-6
40
Gemcitabine Approved Phase 2 95058-81-4 60750
41
Cisplatin Approved Phase 2 15663-27-1 2767 441203 84093
42
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
43
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
44
Tretinoin Approved, Investigational, Nutraceutical Phase 2 302-79-4 444795 5538
45 Alkylating Agents Phase 2
46 Mitomycins Phase 2
47 2-chloro-3'-deoxyadenosine Phase 2
48 Anti-HIV Agents Phase 2
49 Anti-Retroviral Agents Phase 2
50 TAK-652 Phase 2

Interventional clinical trials:

(show top 50) (show all 120)
# 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 A Prospective Multi-centric Belgian Trial to Validate the Use of Golimumab Serum Level Analysis Using the Dried Blood Spot (DBS) Methodology Unknown status NCT02910375 Phase 4
3 A Study of Safety and Efficacy of Leflunomide for Maintenance of Remission in IgG4 Related Disease Completed NCT02703194 Phase 4 Prednisone;Leflunomide
4 Randomized Trial of Short Antibiotic Course to Decrease Post ERCP Cholangitis Recruiting NCT03087656 Phase 4 Ceftriaxone;Levofloxacin
5 Multicenter Randomized Trial Comparing 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 and Inflammatory Bowel Disease- a Randomized Placebo-Controlled Cross-Over Trial Unknown status NCT00161148 Phase 3 Probiotics
7 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
8 Multicentered Randomized Trial of High-dose Urso in Primary Sclerosing Cholangitis Completed NCT00059202 Phase 2, Phase 3 Ursodeoxycholic Acid
9 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
10 Diagnosis of Intraepithelial Neoplasia in Patients With Long Standing Ulcerative Colitis With Chromoscopic Guided Endomicroscopy Completed NCT00352404 Phase 3
11 Digital SpyGlass Cholangioscopy Confirmed Common Bile Duct Stones Clearance Without Fluoroscopy Completed NCT02967926 Phase 3
12 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
13 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
14 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
15 Effect of Simvastatin on the Prognosis of Primary Sclerosing Cholangitis (PSC); A Randomized, Double-blind, Placebo Controlled Multicenter Study Not yet recruiting NCT04133792 Phase 3 Simvastatin 40mg;Placebo oral tablet
16 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
17 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
18 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
19 Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis Completed NCT00004762 Phase 2 cladribine
20 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
21 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
22 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
23 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
24 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
25 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
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 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
28 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
29 Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis. Completed NCT02424175 Phase 1, Phase 2
30 A Pilot Study of the Safety and Efficacy of AST-120 in the Treatment of Active Pouchitis Completed NCT00583076 Phase 2 AST-120
31 Rituximab (RTX) for IgG4-related Disease (IgG4-RD): a Prospective,Open-label Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
32 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) Recruiting NCT03722576 Phase 2 Vidofludimus calcium
33 A Randomized, Placebo-controlled Pilot Study of Sulfasalazine for the Treatment of Primary Sclerosing Cholangitis (PSC) Recruiting NCT03561584 Phase 2 Sulfasalazine;Placebo
34 An Efficacy Trial of Low Dose All-trans Retinoic Acid (ATRA) in Patients With Primary Sclerosing Cholangitis Recruiting NCT03359174 Phase 2 All-trans retinoic acid
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 Induction of Regulatory t Cells by Low Dose IL2 in Autoimmune and Inflammatory Diseases: a Transnosographic Approach Recruiting NCT01988506 Phase 2 Interleukin 2
37 A Proof-of-Concept and Dose-Ranging Study Investigating the Efficacy and Safety of HTD1801 in Adult Subjects With Primary Sclerosing Cholangitis (PSC) Active, not recruiting NCT03333928 Phase 2 HTD1801;Placebo
38 Intra-arterial Injection of Umbilical Cord Mesenchymal Stem Cells in Primary Sclerosing Cholangitis Active, not recruiting NCT03516006 Phase 1, Phase 2 UCMSC;UDCA
39 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
40 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
41 Pilot Study of Fenofibrate in Primary Sclerosing Cholangitis Terminated NCT01142323 Phase 1, Phase 2 fenofibrate
42 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
43 Open Label, Phase II Investigation of Thalidomide for the Treatment of Primary Sclerosing Cholangitis Terminated NCT00953615 Phase 2 Thalidomide
44 Definitive Chemoradiation With Gemcitabine and Continuous 5- FU (Fluorouracil)Followed by High Dose Rate Brachytherapy or Stereotactic Body Radiation Therapy Boost in Locally Advanced Intra or Extrahepatic Cholangiocarcinoma Terminated NCT00983541 Phase 2 Fluorouracil (5-FU);Gemcitabine
45 Primary Sclerosing Cholangitis: Value of Computerized Tomographic Cholangiography and Intraductal Volumetric Measurement in Determining the Prognosis Withdrawn NCT00588458 Phase 2
46 A Pilot Study of Oral Hymecromone to Treat Adults With Primary Sclerosing Cholangitis Withdrawn NCT02780752 Phase 1, Phase 2 hymecromone
47 Phase 1 Study of Intrahepatic Reinfusion of Highly Purified CD133+ Stem Cells in Patients With End-Stage Liver Disease Unknown status NCT01025622 Phase 1
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 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

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 29

Anatomical Context for Cholangitis, Primary Sclerosing

MalaCards organs/tissues related to Cholangitis, Primary Sclerosing:

40
Liver, Testes, Thyroid, Spleen, Skin, Eye, Colon

Publications for Cholangitis, Primary Sclerosing

Articles related to Cholangitis, Primary Sclerosing:

(show top 50) (show all 4752)
# Title Authors PMID Year
1
Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis. 61 54 56
12783301 2003
2
Genome-wide association analysis in primary sclerosing cholangitis identifies two non-HLA susceptibility loci. 61 56
21151127 2011
3
Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease. 56 61
18667695 2008
4
Hepatic endothelial CCL25 mediates the recruitment of CCR9+ gut-homing lymphocytes to the liver in primary sclerosing cholangitis. 61 56
15557349 2004
5
Primary sclerosing cholangitis. 56 61
7877651 1995
6
Gene-centric association mapping of chromosome 3p implicates MST1 in IBD pathogenesis. 56
19079170 2008
7
New insights into autoimmune cholangitis through animal models. 54 61
20460897 2010
8
Gut homing receptors on CD8 T cells are retinoic acid dependent and not maintained by liver dendritic or stellate cells. 61 54
19233184 2009
9
Cystic fibrosis transmembrane conductance regulator gene polymorphisms in patients with primary sclerosing cholangitis. 54 61
18992954 2009
10
PXR: a xenobiotic receptor of diverse function implicated in pharmacogenetics. 54 61
19018724 2008
11
Disturbed colocalization of multidrug resistance protein 2 and radixin in human cholestatic liver diseases. 61 54
17725603 2008
12
What is the role of cystic fibrosis transmembrane conductance regulator dysfunction in primary sclerosing cholangitis? 54 61
17719926 2007
13
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. 54 61
17719933 2007
14
High aspartate to alanine aminotransferase ratio is an indicator of cirrhosis and poor outcome in patients with primary sclerosing cholangitis. 54 61
17498256 2007
15
Mucin-producing bile duct carcinoma arising from primary sclerosing cholangitis: a case report. 61 54
17384480 2007
16
MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes. 61 54
17295178 2007
17
Analysis of MAdCAM-1 and ICAM-1 polymorphisms in 365 Scandinavian patients with primary sclerosing cholangitis. 61 54
16750586 2006
18
Polymorphisms in the steroid and xenobiotic receptor gene influence survival in primary sclerosing cholangitis. 61 54
16952547 2006
19
Serum reactivity against bacterial pyruvate dehydrogenase: increasing the specificity of anti-mitochondrial antibodies for the diagnosis of primary biliary cirrhosis. 61 54
17162370 2006
20
Cholestasis and cholestatic syndromes. 61 54
16550034 2006
21
Expression of epithelial cellular adhesion molecule (Ep-CAM) in chronic (necro-)inflammatory liver diseases and hepatocellular carcinoma. 54 61
16364680 2006
22
Lack of association of common cystic fibrosis transmembrane conductance regulator gene mutations with primary sclerosing cholangitis. 61 54
15784035 2005
23
Induction of colitis in cftr-/- mice results in bile duct injury. 54 61
15064232 2004
24
BSEP and MDR3 haplotype structure in healthy Caucasians, primary biliary cirrhosis and primary sclerosing cholangitis. 61 54
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. 54 61
15101998 2004
26
Novel association of HLA-haplotypes with primary sclerosing cholangitis (PSC) in a southern European population. 61 54
14567462 2003
27
Expression of deleted in malignant brain tumor-1 (DMBT1) molecule in biliary epithelium is augmented in hepatolithiasis: possible participation in lithogenesis. 54 61
12870778 2003
28
Heterogeneity of ductular reactions in adult rat and human liver revealed by novel expression of deleted in malignant brain tumor 1. 54 61
12368192 2002
29
Expression of hepatic transporters OATP-C and MRP2 in primary sclerosing cholangitis. 61 54
11454187 2001
30
Autoimmune regulator AIRE: evidence for genetic differences between autoimmune hepatitis and hepatitis as part of the autoimmune polyglandular syndrome type 1. 54 61
11343230 2001
31
MAdCAM-1 expressed in chronic inflammatory liver disease supports mucosal lymphocyte adhesion to hepatic endothelium (MAdCAM-1 in chronic inflammatory liver disease). 61 54
11343233 2001
32
Overlap syndrome between autoimmune hepatitis and primary sclerosing cholangitis in two cases. 61 54
10833101 2000
33
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) 61 54
10824890 2000
34
Reduced hepatic content of dehydroepiandrosterone sulphotransferase in chronic liver diseases. 61 54
10726960 2000
35
Immunohistochemical study on phenotypical changes of hepatocytes in liver disease with reference to extracellular matrix composition. 61 54
9928763 1999
36
Biliary lactoferrin concentrations are increased in active inflammatory bowel disease: a factor in the pathogenesis of primary sclerosing cholangitis? 61 54
9791051 1998
37
Antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis: defined specificities may be associated with distinct clinical features. 61 54
9831423 1998
38
Human leukocyte antigen-C genes and susceptibility to primary sclerosing cholangitis. 61 54
9731555 1998
39
Identification and characterization of autoantibodies against catalase and alpha-enolase in patients with primary sclerosing cholangitis. 54 61
9649223 1998
40
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. 54 61
9502419 1998
41
An atypical presentation for primary sclerosing cholangitis. 61 54
9365127 1997
42
Anti-lactoferrin autoantibodies: relation between epitopes and iron-binding domain. 54 61
8864834 1996
43
Two spatially distant epitopes of human lactoferrin. 54 61
8880213 1996
44
Anti-neutrophil cytoplasmic antibodies (ANCA) directed against bactericidal/permeability increasing protein (BPI): a new seromarker for inflammatory bowel disease and associated disorders. 54 61
8603534 1996
45
Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis. 61 54
8617131 1996
46
Distribution of lactoferrin and 60/65 kDa heat shock protein in normal and inflamed human intestine and liver. 54 61
8566841 1996
47
HLA-DR and HLA-DQ are not markers for rapid disease progression in primary sclerosing cholangitis. 54 61
7875491 1995
48
Biliary epithelial expression of pyruvate dehydrogenase complex in primary biliary cirrhosis: an immunohistochemical and immunoelectron microscopic study. 61 54
7821922 1995
49
Anti-lactoferrin antibodies and other types of ANCA in ulcerative colitis, primary sclerosing cholangitis, and Crohn's disease. 61 54
8432453 1993
50
Lactoferrin, anti-lactoferrin antibodies and inflammatory disease. 61 54
8296671 1993

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
11.26 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 extracellular space GO:0005615 9.81 PRTN3 MST1 MPO GPT GGT1 ENO1
2 cell surface GO:0009986 9.65 HLA-A ENO1 CTSG CFTR CCR6
3 extracellular exosome GO:0070062 9.36 PRTN3 MPO KRT19 HLA-A GPT GGT1
4 azurophil granule lumen GO:0035578 9.26 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 defense response to Gram-positive bacterium GO:0050830 9.43 HLA-A DMBT1 CTSG
2 immune response GO:0006955 9.35 MADCAM1 HLA-A CTSG CCR6 BPI
3 defense response to Gram-negative bacterium GO:0050829 9.33 DMBT1 CTSG BPI
4 antimicrobial humoral response GO:0019730 8.8 PRTN3 CTSG BPI

Sources for Cholangitis, Primary Sclerosing

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