MCID: CHL119
MIFTS: 60

Cholangitis, Primary Sclerosing

Categories: Rare diseases, Liver diseases, Gastrointestinal diseases

Aliases & Classifications for Cholangitis, Primary Sclerosing

MalaCards integrated aliases for Cholangitis, Primary Sclerosing:

Name: Cholangitis, Primary Sclerosing 53 72 49 13
Primary Sclerosing Cholangitis 12 72 49 24 55 36 14 69
Psc 53 24 55
Sclerosing Cholangitis 49 24
Cholangitis Primary Sclerosing 51
Cholangitis, Sclerosing 69

Characteristics:

Orphanet epidemiological data:

55
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: 55  
Rare hepatic diseases


Summaries for Cholangitis, Primary Sclerosing

NIH Rare Diseases : 49 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). Last updated: 3/4/2013

MalaCards based summary : Cholangitis, Primary Sclerosing, also known as primary sclerosing cholangitis, is related to cholangitis and sclerosing cholangitis, and has symptoms including pruritus, fatigue and fever. An important gene associated with Cholangitis, Primary Sclerosing is PSC (Cholangitis, Primary Sclerosing), and among its related pathways/superpathways are Bile secretion and Farnesoid X Receptor Pathway. The drugs Vancomycin and Ceftriaxone have been mentioned in the context of this disorder. Affiliated tissues include liver, thyroid and skin, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

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

Genetics Home Reference : 24 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.

OMIM : 53 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)

Wikipedia : 72 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 all 27)
# Related Disease Score Top Affiliating Genes
1 cholangitis 31.3 ABCB4 CFTR DLAT GGT1
2 sclerosing cholangitis 30.7 ABCB4 BPI CFTR CTSG HLA-C MPO
3 inflammatory bowel disease 29.6 BPI CTSG MPO MST1 NR1I2
4 ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis 12.7
5 glaucomatocyclitic crisis 10.5 HLA-A HLA-C
6 autoimmune retinopathy 10.5 AIRE ENO1
7 cholecystolithiasis 10.4 ABCB4 GPR35
8 idiopathic bronchiectasis 10.4 CFTR HLA-A HLA-C
9 suppurative cholangitis 10.4 ABCB4 DLAT MPO
10 crescentic glomerulonephritis 10.4 BPI CTSG MPO
11 autoimmune disease of urogenital tract 10.4 ABCB4 DLAT SEPSECS
12 extrahepatic cholestasis 10.4 ABCB4 GGT1 MPO
13 vasculitis 10.3 BPI CTSG MPO
14 bronchiectasis 10.3 BPI CFTR CTSG MPO
15 wegener granulomatosis 10.3 BPI CTSG MPO
16 bile duct disease 10.3 ABCB4 GGT1 NR1I2
17 biliary tract disease 10.3 ABCB4 GGT1 NR1I2
18 gastrointestinal system disease 10.2 GGT1 MPO NR1I2
19 intrahepatic cholestasis 10.2 ABCB4 GGT1 NR1I2
20 pityriasis rosea 10.2 HLA-A HLA-C
21 cystic fibrosis 10.2 BPI CFTR CTSG MPO
22 cholestasis 10.1 ABCB4 GGT1 NR1I2
23 liver disease 10.1 ABCB4 DLAT GGT1 SEPSECS
24 primary biliary cirrhosis 10.0 ABCB4 DLAT GGT1 NR1I2 SEPSECS
25 cholestasis, progressive familial intrahepatic, 1 10.0 ABCB4 GGT1
26 autoimmune hepatitis 10.0 AIRE DLAT GGT1 HLA-A SEPSECS
27 primary biliary cholangitis 9.8

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



Diseases related to Cholangitis, Primary Sclerosing

Symptoms & Phenotypes for Cholangitis, Primary Sclerosing

Clinical features from OMIM:

613806

Human phenotypes related to Cholangitis, Primary Sclerosing:

55 31 (show top 50) (show all 56)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 pruritus 55 31 occasional (7.5%) Occasional (29-5%) HP:0000989
2 fatigue 55 31 occasional (7.5%) Occasional (29-5%) HP:0012378
3 fever 55 31 frequent (33%) Frequent (79-30%) HP:0001945
4 abdominal pain 55 31 occasional (7.5%) Occasional (29-5%) HP:0002027
5 hepatosplenomegaly 55 31 frequent (33%) Frequent (79-30%) HP:0001433
6 osteopenia 55 31 occasional (7.5%) Occasional (29-5%) HP:0000938
7 splenomegaly 55 31 frequent (33%) Frequent (79-30%) HP:0001744
8 hepatomegaly 55 31 frequent (33%) Frequent (79-30%) HP:0002240
9 renal insufficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0000083
10 portal hypertension 55 31 frequent (33%) Frequent (79-30%) HP:0001409
11 type i diabetes mellitus 55 31 occasional (7.5%) Occasional (29-5%) HP:0100651
12 osteoporosis 55 31 occasional (7.5%) Occasional (29-5%) HP:0000939
13 ascites 55 31 frequent (33%) Frequent (79-30%) HP:0001541
14 hepatitis 55 31 occasional (7.5%) Occasional (29-5%) HP:0012115
15 weight loss 55 31 frequent (33%) Frequent (79-30%) HP:0001824
16 congestive heart failure 55 31 occasional (7.5%) Occasional (29-5%) HP:0001635
17 elevated hepatic transaminases 55 31 frequent (33%) Frequent (79-30%) HP:0002910
18 hepatic fibrosis 55 31 frequent (33%) Frequent (79-30%) HP:0001395
19 celiac disease 55 31 occasional (7.5%) Occasional (29-5%) HP:0002608
20 autoimmunity 55 31 hallmark (90%) Very frequent (99-80%) HP:0002960
21 thyroiditis 55 31 occasional (7.5%) Occasional (29-5%) HP:0100646
22 cirrhosis 55 31 frequent (33%) Frequent (79-30%) HP:0001394
23 cholelithiasis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001081
24 jaundice 55 31 occasional (7.5%) Occasional (29-5%) HP:0000952
25 pancreatitis 55 31 occasional (7.5%) Occasional (29-5%) HP:0001733
26 vitamin d deficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0100512
27 acute hepatic failure 55 31 very rare (1%) Very rare (<4-1%) HP:0006554
28 encephalopathy 55 31 very rare (1%) Very rare (<4-1%) HP:0001298
29 uveitis 55 31 very rare (1%) Very rare (<4-1%) HP:0000554
30 pleural effusion 55 31 occasional (7.5%) Occasional (29-5%) HP:0002202
31 prolonged prothrombin time 55 31 occasional (7.5%) Occasional (29-5%) HP:0008151
32 elevated alkaline phosphatase of hepatic origin 55 31 frequent (33%) Frequent (79-30%) HP:0010638
33 spider hemangioma 55 31 frequent (33%) Frequent (79-30%) HP:0012522
34 abnormal large intestine physiology 55 31 frequent (33%) Frequent (79-30%) HP:0012700
35 dilated superficial abdominal veins 55 31 frequent (33%) Frequent (79-30%) HP:0030168
36 ulcerative colitis 55 31 frequent (33%) Frequent (79-30%) HP:0100279
37 palmar telangiectasia 55 31 frequent (33%) Frequent (79-30%) HP:0100869
38 hepatocellular carcinoma 55 31 occasional (7.5%) Occasional (29-5%) HP:0001402
39 hypoalbuminemia 55 31 occasional (7.5%) Occasional (29-5%) HP:0003073
40 polyclonal elevation of igm 55 31 occasional (7.5%) Occasional (29-5%) HP:0003459
41 generalized amyotrophy 55 31 occasional (7.5%) Occasional (29-5%) HP:0003700
42 vitamin a deficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0004905
43 vitamin k deficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0011892
44 cholangiocarcinoma 55 31 occasional (7.5%) Occasional (29-5%) HP:0030153
45 adenocarcinoma of the large intestine 55 31 occasional (7.5%) Occasional (29-5%) HP:0040275
46 vitamin e deficiency 55 31 occasional (7.5%) Occasional (29-5%) HP:0100513
47 chronic hepatic failure 55 31 occasional (7.5%) Occasional (29-5%) HP:0100626
48 neoplasm of the gallbladder 55 31 very rare (1%) Very rare (<4-1%) HP:0100575
49 depression 55 Occasional (29-5%)
50 cholestasis 55 Very frequent (99-80%)

UMLS symptoms related to Cholangitis, Primary Sclerosing:


icterus

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

25 (show all 36)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 10.21 MPO
2 Increased shRNA abundance (Z-score > 2) GR00366-A-103 10.21 CTSG HLA-C GPR35 HLA-A
3 Increased shRNA abundance (Z-score > 2) GR00366-A-105 10.21 GPR35
4 Increased shRNA abundance (Z-score > 2) GR00366-A-110 10.21 HLA-A HLA-C
5 Increased shRNA abundance (Z-score > 2) GR00366-A-118 10.21 HLA-C HLA-A
6 Increased shRNA abundance (Z-score > 2) GR00366-A-124 10.21 HLA-C
7 Increased shRNA abundance (Z-score > 2) GR00366-A-126 10.21 MPO
8 Increased shRNA abundance (Z-score > 2) GR00366-A-129 10.21 GPR35
9 Increased shRNA abundance (Z-score > 2) GR00366-A-13 10.21 CTSG
10 Increased shRNA abundance (Z-score > 2) GR00366-A-130 10.21 GGT1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-131 10.21 GPR35
12 Increased shRNA abundance (Z-score > 2) GR00366-A-143 10.21 GPR35 GGT1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-149 10.21 HLA-C CTSG GPR35
14 Increased shRNA abundance (Z-score > 2) GR00366-A-156 10.21 HLA-C HLA-A
15 Increased shRNA abundance (Z-score > 2) GR00366-A-157 10.21 MPO
16 Increased shRNA abundance (Z-score > 2) GR00366-A-161 10.21 CTSG
17 Increased shRNA abundance (Z-score > 2) GR00366-A-162 10.21 HLA-C
18 Increased shRNA abundance (Z-score > 2) GR00366-A-169 10.21 GGT1
19 Increased shRNA abundance (Z-score > 2) GR00366-A-176 10.21 MPO
20 Increased shRNA abundance (Z-score > 2) GR00366-A-178 10.21 HLA-C HLA-A
21 Increased shRNA abundance (Z-score > 2) GR00366-A-186 10.21 MPO
22 Increased shRNA abundance (Z-score > 2) GR00366-A-19 10.21 HLA-C HLA-A
23 Increased shRNA abundance (Z-score > 2) GR00366-A-198 10.21 GPR35
24 Increased shRNA abundance (Z-score > 2) GR00366-A-199 10.21 GPR35
25 Increased shRNA abundance (Z-score > 2) GR00366-A-202 10.21 HLA-C
26 Increased shRNA abundance (Z-score > 2) GR00366-A-21 10.21 CTSG
27 Increased shRNA abundance (Z-score > 2) GR00366-A-29 10.21 MPO
28 Increased shRNA abundance (Z-score > 2) GR00366-A-31 10.21 HLA-C HLA-A
29 Increased shRNA abundance (Z-score > 2) GR00366-A-36 10.21 MPO
30 Increased shRNA abundance (Z-score > 2) GR00366-A-43 10.21 CTSG
31 Increased shRNA abundance (Z-score > 2) GR00366-A-47 10.21 HLA-C
32 Increased shRNA abundance (Z-score > 2) GR00366-A-57 10.21 MPO
33 Increased shRNA abundance (Z-score > 2) GR00366-A-7 10.21 HLA-C GGT1 HLA-A MPO
34 Increased shRNA abundance (Z-score > 2) GR00366-A-70 10.21 MPO
35 Increased shRNA abundance (Z-score > 2) GR00366-A-74 10.21 HLA-C GPR35 HLA-A
36 Increased shRNA abundance (Z-score > 2) GR00366-A-93 10.21 HLA-C

MGI Mouse Phenotypes related to Cholangitis, Primary Sclerosing:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.32 ABCB4 AIRE CFTR CTSG DMBT1 EPCAM

Drugs & Therapeutics for Cholangitis, Primary Sclerosing

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

(show top 50) (show all 89)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vancomycin Approved Phase 4,Phase 3,Phase 1 1404-90-6 14969 441141
2
Ceftriaxone Approved Phase 4 73384-59-5 5479530 5361919
3
Levofloxacin Approved, Investigational Phase 4 100986-85-4 149096
4
Ofloxacin Approved Phase 4 82419-36-1 4583
5
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
6
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
7
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
8
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
9 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1
10 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1
11 Liver Extracts Phase 4,Phase 3,Phase 2,Phase 1
12 Antibiotics, Antitubercular Phase 4,Phase 2
13 Antibodies Phase 4,Phase 2,Phase 1
14 Anti-Inflammatory Agents Phase 4,Phase 1,Phase 2
15 Antirheumatic Agents Phase 4,Phase 1,Phase 2
16 glucocorticoids Phase 4,Phase 1
17 Hormone Antagonists Phase 4,Phase 1
18 Hormones Phase 4,Phase 1
19 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1
20 Immunoglobulins Phase 4,Phase 2,Phase 1
21 Immunosuppressive Agents Phase 4,Phase 2,Phase 1
22 Bile Acids and Salts Phase 4,Phase 2,Phase 1
23 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1
24 Antitubercular Agents Phase 4
25 Cytochrome P-450 CYP1A2 Inhibitors Phase 4
26 Cytochrome P-450 Enzyme Inhibitors Phase 4
27 Renal Agents Phase 4
28 Topoisomerase Inhibitors Phase 4
29 Antineoplastic Agents, Hormonal Phase 4
30 Methylprednisolone acetate Phase 4
31 Methylprednisolone Hemisuccinate Phase 4
32 Prednisolone acetate Phase 4
33 Prednisolone hemisuccinate Phase 4
34 Prednisolone phosphate Phase 4
35
Ursodeoxycholic acid Approved, Investigational Phase 2, Phase 3,Phase 1 128-13-2 31401
36
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
37 Antimetabolites Phase 3,Phase 1,Phase 2
38 Cholagogues and Choleretics Phase 2, Phase 3,Phase 1
39 Hypolipidemic Agents Phase 3,Phase 1,Phase 2
40 Lipid Regulating Agents Phase 3,Phase 1,Phase 2
41
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
42
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
43
Curcumin Approved, Investigational Phase 1, Phase 2 458-37-7 969516
44
Aldesleukin Approved Phase 2 110942-02-4, 85898-30-2
45
Fenofibrate Approved Phase 1, Phase 2 49562-28-9 3339
46
Tretinoin Approved, Investigational, Nutraceutical Phase 2,Phase 1 302-79-4 5538
47 Anti-HIV Agents Phase 2
48 Anti-Retroviral Agents Phase 2
49 Antiviral Agents Phase 2
50 TAK-652 Phase 2

Interventional clinical trials:

(show top 50) (show all 86)

# 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 Trial of High-dose Urso in Primary Sclerosing Cholangitis Completed NCT00059202 Phase 2, Phase 3 Ursodeoxycholic Acid
8 The Effect of Bezafibrate on Cholestatic Itch Recruiting NCT02701166 Phase 3 Bezafibrate;Placebo
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 Norursodeoxycholic Acid in the Treatment of Primary Sclerosing Cholangitis Completed NCT01755507 Phase 2 norUDCA;Placebo
14 Open Label Study to Evaluate Safety and Efficacy of LUM001 in Patients With Primary Sclerosing Cholangitis Completed NCT02061540 Phase 2 LUM001
15 Simtuzumab (GS-6624) in the Prevention of Progression of Liver Fibrosis in Subjects With Primary Sclerosing Cholangitis (PSC) Completed NCT01672853 Phase 2
16 Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis Completed NCT00004762 Phase 2 cladribine
17 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
18 Mitomycin C Therapy for Patients With Primary Sclerosing Cholangitis Recruiting NCT01688024 Phase 2 Mitomycin C;Normal saline
19 A Trial of BTT1023 in Patients With Primary Sclerosing Cholangitis Recruiting NCT02239211 Phase 2 BTT1023
20 A Study Evaluating the Safety and Efficacy of Curcumin in Patients With Primary Sclerosing Cholangitis Recruiting NCT02978339 Phase 1, Phase 2 Curcumin
21 A Research Study to Evaluate Safety and Efficacy of DUR-928 in Subjects With Primary Sclerosing Cholangitis (PSC) Recruiting NCT03394781 Phase 2 DUR-928
22 A POC and Dose-Ranging Study of HTD1801 in PSC Patients Recruiting NCT03333928 Phase 2 HTD1801;Placebo
23 An Efficacy Trial of Low Dose All-trans Retinoic Acid in Patients With Primary Sclerosing Cholangitis Recruiting NCT03359174 Phase 2 All-trans retinoic acid
24 Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases Recruiting NCT01988506 Phase 2 Interleukin 2
25 Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis. Active, not recruiting NCT02424175 Phase 1, Phase 2
26 Obeticholic Acid (OCA) in Primary Sclerosing Cholangitis (PSC) Active, not recruiting NCT02177136 Phase 2 OCA;Placebo
27 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
28 A Study of Oral Hymecromone to Treat Adults With Primary Sclerosing Cholangitis Not yet recruiting NCT02780752 Phase 1, Phase 2 hymecromone
29 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
30 Thalidomide for the Treatment of Primary Sclerosing Cholangitis (PSC) Terminated NCT00953615 Phase 2 Thalidomide
31 Pilot Study of Fenofibrate for PSC Terminated NCT01142323 Phase 1, Phase 2 fenofibrate
32 The Value of CT Cholangiography in Primary Sclerosing Cholangitis Withdrawn NCT00588458 Phase 2
33 Erlotinib for Chemoprevention in Trisomy 7 Positive Primary Sclerosing Cholangitis (PSC) Completed NCT00955149 Phase 1 Erlotinib (Tarceva)
34 Gastrointestinal Microbiota in Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Completed NCT01322386 Phase 1 Vancomycin
35 Safety and Efficacy Study of Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis Completed NCT01088607 Phase 1 ursodeoxycholic acid (UDCA)
36 A Pilot Study of Vancomycin or Metronidazole in Patients With Primary Sclerosing Cholangitis Completed NCT01085760 Phase 1 Vancomycin;Vancomycin;Metronidazole;Metronidazole;Vancomycin
37 Minocycline in Primary Sclerosing Cholangitis (PSC) Completed NCT00630942 Phase 1 Minocycline
38 Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis Completed NCT00004842 Phase 1 budesonide
39 Evaluation of DHA for the Treatment of PSC Completed NCT00325013 Phase 1 Docosahexaenoic Acid (DHA)
40 A Pilot Study of Xifaxan to Treat Patients With PSC Completed NCT01695174 Phase 1 Xifaxan
41 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)
42 A Study of HTD1801 in Healthy Subjects Completed NCT03099603 Phase 1 HTD1801
43 Optical Frequency Domain Imaging (OFDI) for Biliary Stricture Imaging Completed NCT01439620 Phase 1
44 Biliary Excretion of Conjugated Bile Acids in Humans Measured by 11C-cholylsarcosine PET/CT Completed NCT01879735 Phase 1 11C-CSar;ICG
45 Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Recruiting NCT02137668 Phase 1 Oral Vancomycin
46 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
47 A Study Of Ursolic Acid For Primary Sclerosing Cholangitis Withdrawn NCT03216876 Phase 1 Ursolic acid
48 NPD Measurements in PSC Patients Unknown status NCT00476814
49 Telomere Length and Other Parameters of Telomere Dysfunction in PSC and Colitis Unknown status NCT02247622
50 Liver Transplantation for Hilar Cholangiocarcinoma in Association With Neoadjuvant Radio- and Chemo-therapy Unknown status NCT01549795 Capecitabine

Search NIH Clinical Center for Cholangitis, Primary Sclerosing

Genetic Tests for Cholangitis, Primary Sclerosing

Anatomical Context for Cholangitis, Primary Sclerosing

MalaCards organs/tissues related to Cholangitis, Primary Sclerosing:

38
Liver, Thyroid, Skin, Eye, Spleen, Small Intestine, T Cells

Publications for Cholangitis, Primary Sclerosing

Articles related to Cholangitis, Primary Sclerosing:

(show top 50) (show all 768)
# Title Authors Year
1
Reviewing the Risk of Colorectal Cancer in Inflammatory Bowel Disease After Liver Trasnplantation for Primary Sclerosing Cholangitis. ( 29361103 )
2018
2
High Risk of Advanced Colorectal Neoplasia in Patients With Primary Sclerosing Cholangitis Associated With Inflammatory Bowel Disease. ( 29378311 )
2018
3
Quality of life and primary sclerosing cholangitis: the business of defining what counts. ( 29315689 )
2018
4
Loss of tolerance to gut immunity protein, glycoprotein 2 (GP2) is associated with progressive disease course in primary sclerosing cholangitis. ( 29321484 )
2018
5
Editorial: the role of colonic inflammation in the progression of liver disease in patients with primary sclerosing cholangitis. ( 29314124 )
2018
6
Review article: the evidence that vancomycin is a therapeutic option for primary sclerosing cholangitis. ( 29411404 )
2018
7
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 )
2018
8
Crohn's Disease Behavior and Location is Altered when Associated with Primary Sclerosing Cholangitis. ( 29333790 )
2018
9
Cholestasis after TIPS placement in a patient with primary sclerosing cholangitis: an uncommon complication. ( 29278002 )
2018
10
Lack of Correlation of Liver Tests With Fibrosis Stage at Diagnosis in Pediatric Primary Sclerosing Cholangitis. ( 29356767 )
2018
11
Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation: a Nordic Multicentre Study. ( 29301479 )
2018
12
Letter: the effects of colectomy prior to the diagnosis of primary sclerosing cholangitis on prognosis may have been overestimated. ( 29314130 )
2018
13
The gut microbiota, bile acids and their correlation in primary sclerosing cholangitis associated with inflammatory bowel disease. ( 29435321 )
2018
14
Symptoms do not Correlate With Findings From Colonoscopy in Children with Inflammatory Bowel Disease and Primary Sclerosing Cholangitis. ( 29378308 )
2018
15
Peribiliary Cysts presenting as Primary Sclerosing Cholangitis. ( 29432923 )
2018
16
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 )
2018
17
Gut barrier failure biomarkers are associated with poor disease outcome in patients with primary sclerosing cholangitis. ( 28839442 )
2017
18
Knockout of the Primary Sclerosing Cholangitis-Risk Gene Fut2 Causes Liver Disease in Mice. ( 28056490 )
2017
19
Updates on primary sclerosing cholangitis. ( 28079751 )
2017
20
Successful Treatment With Vedolizumab in a Patient With Chronic Refractory Pouchitis and Primary Sclerosing Cholangitis. ( 29106505 )
2017
21
Impact of Crohn's Disease Duration on the Risk of Cholangiocarcinoma in Patients With Primary Sclerosing Cholangitis. ( 28050039 )
2017
22
Bile microbiota in primary sclerosing cholangitis: Impact on disease progression and development of biliary dysplasia. ( 28796833 )
2017
23
Acute-on-chronic hepatitis. A case report of autoimmune hepatitis/primary sclerosing cholangitis/ulcerative colitis overlap syndrome in a 15-year-old patient. ( 28856287 )
2017
24
New approach to decrease post-ERCP adverse events in patients with primary sclerosing cholangitis. ( 28791317 )
2017
25
Cholangiocytes in the pathogenesis of primary sclerosing cholangitis and development of cholangiocarcinoma. ( 28844951 )
2017
26
Inflammatory bowel disease with primary sclerosing cholangitis: A Danish population-based cohort study 1977-2011. ( 28796371 )
2017
27
Increased cholestatic enzymes in two patients with long-term history of ulcerative colitis: consider primary biliary cholangitis not always primary sclerosing cholangitis. ( 28951510 )
2017
28
Efficacy of oral vancomycin in recurrent primary sclerosing cholangitis following liver transplantation. ( 28951512 )
2017
29
Primary Hepatic Adenosquamous Carcinoma Associated with Primary Sclerosing Cholangitis. ( 29258074 )
2017
30
A novel approach to genome-wide association analysis identifies genetic associations with primary biliary cholangitis and primary sclerosing cholangitis in Polish patients. ( 28056976 )
2017
31
Endpoints in the design of clinical trials for primary sclerosing cholangitis. ( 28847513 )
2017
32
Pathological features of primary sclerosing cholangitis identified by bile proteomic analysis. ( 28943450 )
2017
33
Primary biliary cholangitis-primary sclerosing cholangitis in an evolving overlap syndrome: A case report. ( 28089354 )
2017
34
Carcinoembryonic Antigen Level in Primary Sclerosing Cholangitis Is Not Influenced by Dominant Strictures or Bacterial Cholangitis. ( 27943017 )
2017
35
Patients With Ulcerative Colitis and Primary Sclerosing Cholangitis Frequently Have Subclinical Inflammation in the Proximal Colon. ( 28756053 )
2017
36
Retraction notice to: PS124-Efficacy and Safety of Induction Dosing of Vedolizumab for Reducing Biliary Inflammation in Primary Sclerosing Cholangitis (PSC) in Individuals with Inflammatory Bowel Disease [J Hepatol 64 (2016) S199]. ( 28042931 )
2017
37
Primary sclerosing cholangitis - a comprehensive review. ( 28802875 )
2017
38
Development and characterization of cholangioids from normal and diseased human cholangiocytes as an in vitro model to study primary sclerosing cholangitis. ( 28892096 )
2017
39
Genetic association analysis identifies variants associated with disease progression in primary sclerosing cholangitis. ( 28779025 )
2017
40
Cholangiocarcinoma and its mimickers in primary sclerosing cholangitis. ( 28951947 )
2017
41
Complete resolution of extensive xanthomas associated with primary sclerosing cholangitis following liver transplantation. ( 28940652 )
2017
42
Genome-wide association study of primary sclerosing cholangitis identifies new risk loci and quantifies the genetic relationship with inflammatory bowel disease. ( 27992413 )
2016
43
Evaluation of Biliary Calprotectin as a Biomarker in Primary Sclerosing Cholangitis. ( 27124059 )
2016
44
Primary sclerosing cholangitis in the Swiss Inflammatory Bowel Disease Cohort Study: prevalence, risk factors, and long-term follow-up. ( 27622999 )
2016
45
Letter: impact of concomitant autoimmune diseases on the outcome of primary sclerosing cholangitis. ( 27137734 )
2016
46
Primary Sclerosing Cholangitis ( 28025879 )
2016
47
Intestinal microbiota in primary sclerosing cholangitis. ( 28030369 )
2016
48
Alkaline Phosphatase at Diagnosis of Primary Sclerosing Cholangitis and One Year Later: Evaluation of Prognostic Value. ( 26945698 )
2016
49
Antineutrophil antibodies define clinical and genetic subgroups in primary sclerosing cholangitis. ( 27558072 )
2016
50
The Microbiome and Primary Sclerosing Cholangitis. ( 27997974 )
2016

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 10.65 ABCB4 CFTR SULT2A1
2 10.54 ABCB4 SULT2A1
3 10.39 NR1I2 SULT2A1
4 10.13 MPO NR1I2

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 9.54 BPI CTSG MPO
2 phagocytic vesicle membrane GO:0030670 9.43 DMBT1 HLA-A HLA-C
3 cell surface GO:0009986 9.43 CFTR CTSG ENO1 EPCAM HLA-A HLA-C
4 extracellular exosome GO:0070062 9.4 ABCB4 BPI CFTR CTSG DMBT1 ENO1
5 recycling endosome membrane GO:0055038 9.33 CFTR HLA-A HLA-C
6 MHC class I protein complex GO:0042612 9.32 HLA-A HLA-C

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 9.72 AIRE BPI CTSG HLA-A HLA-C
2 defense response to Gram-negative bacterium GO:0050829 9.43 BPI CTSG DMBT1
3 negative regulation of growth of symbiont in host GO:0044130 9.37 CTSG MPO
4 xenobiotic transport GO:0042908 9.16 ABCB4 NR1I2
5 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent GO:0002480 8.96 HLA-A HLA-C
6 drug export GO:0046618 8.62 ABCB4 NR1I2

Sources for Cholangitis, Primary Sclerosing

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
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47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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