PSC
MCID: CHL119
MIFTS: 63

Cholangitis, Primary Sclerosing (PSC)

Categories: Cancer diseases, Gastrointestinal diseases, Liver diseases, Rare diseases

Aliases & Classifications for Cholangitis, Primary Sclerosing

MalaCards integrated aliases for Cholangitis, Primary Sclerosing:

Name: Cholangitis, Primary Sclerosing 57 76 53 13
Primary Sclerosing Cholangitis 12 76 53 25 59 37 15 73
Psc 57 25 59
Cholangitis, Sclerosing 44 73
Sclerosing Cholangitis 53 25
Cholangitis Primary Sclerosing 55

Characteristics:

Orphanet epidemiological data:

59
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: 59  
Rare hepatic diseases


Summaries for Cholangitis, Primary Sclerosing

NIH Rare Diseases : 53 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).

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 Bile secretion and Farnesoid X Receptor Pathway. The drugs Vancomycin and Ofloxacin have been mentioned in the context of this disorder. Affiliated tissues include liver, thyroid and spleen, and related phenotypes are depressivity and osteopenia

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

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.

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)

Wikipedia : 76 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 255)
# Related Disease Score Top Affiliating Genes
1 cholangitis 31.4 ABCB4 CFTR DLAT GGT1
2 sclerosing cholangitis 31.0 ABCB4 BPI CFTR CTSG GGT1 HLA-C
3 cholestasis 30.8 ABCB4 GGT1 NR1I2
4 cystic fibrosis 30.6 BPI CFTR CTSG MPO
5 bile duct disease 30.5 ABCB4 GGT1 NR1I2
6 suppurative cholangitis 30.1 ABCB4 DLAT MPO
7 biliary tract disease 30.1 ABCB4 GGT1 NR1I2
8 bronchiectasis 30.1 BPI CFTR CTSG MPO
9 inflammatory bowel disease 30.0 BPI CTSG MPO MST1 NR1I2
10 primary biliary cirrhosis 29.9 ABCB4 DLAT GGT1 SEPSECS
11 liver disease 29.9 ABCB4 DLAT GGT1 SEPSECS
12 autoimmune hepatitis 29.8 AIRE DLAT GGT1 HLA-A SEPSECS
13 ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis 12.9
14 sclerosing cholangitis, neonatal 12.6
15 pancreatitis, sclerosing cholangitis, and sicca complex 12.4
16 isolated neonatal sclerosing cholangitis 12.2
17 bile duct cancer 11.7
18 crohn's disease 10.6
19 colorectal cancer 10.5
20 autoimmune pancreatitis 10.4
21 intrahepatic cholangiocarcinoma 10.4
22 retroperitoneal fibrosis 10.3
23 hepatocellular carcinoma 10.3
24 hemolytic anemia 10.3
25 leukemia 10.3
26 ichthyosis 10.3
27 anemia, autoimmune hemolytic 10.3
28 igg4-related sclerosing cholangitis 10.3
29 cholecystolithiasis 10.2 ABCB4 GPR35
30 autoimmune retinopathy 10.2 AIRE ENO1
31 idiopathic bronchiectasis 10.2 CFTR HLA-A HLA-C
32 crescentic glomerulonephritis 10.2 BPI CTSG MPO
33 autoimmune disease 10.2
34 degos 'en cocarde' erythrokeratoderma 10.2
35 pouchitis 10.2
36 cryptosporidiosis 10.2
37 irritable bowel syndrome 10.2
38 granulomatosis with polyangiitis 10.2 BPI CTSG MPO
39 pityriasis rosea 10.2 HLA-A HLA-C
40 extrahepatic cholestasis 10.2 ABCB4 GGT1 MPO
41 vasculitis 10.2 BPI CTSG MPO
42 pancreatitis 10.2
43 human herpesvirus 8 10.2 HLA-A HLA-C
44 dermatitis herpetiformis, familial 10.2
45 autoimmune disease 1 10.2
46 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
47 lymphoma 10.2
48 gallbladder disease 10.2
49 dermatitis 10.2
50 gallbladder cancer 10.2

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:

59 32 (show top 50) (show all 56)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 depressivity 59 32 occasional (7.5%) Occasional (29-5%) HP:0000716
2 osteopenia 59 32 occasional (7.5%) Occasional (29-5%) HP:0000938
3 splenomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0001744
4 hepatomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0002240
5 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
6 portal hypertension 59 32 frequent (33%) Frequent (79-30%) HP:0001409
7 fever 59 32 frequent (33%) Frequent (79-30%) HP:0001945
8 fatigue 59 32 occasional (7.5%) Occasional (29-5%) HP:0012378
9 type i diabetes mellitus 59 32 occasional (7.5%) Occasional (29-5%) HP:0100651
10 osteoporosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000939
11 ascites 59 32 frequent (33%) Frequent (79-30%) HP:0001541
12 hepatitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012115
13 abdominal pain 59 32 occasional (7.5%) Occasional (29-5%) HP:0002027
14 pruritus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000989
15 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
16 congestive heart failure 59 32 occasional (7.5%) Occasional (29-5%) HP:0001635
17 hepatic fibrosis 59 32 frequent (33%) Frequent (79-30%) HP:0001395
18 celiac disease 59 32 occasional (7.5%) Occasional (29-5%) HP:0002608
19 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
20 thyroiditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100646
21 cirrhosis 59 32 frequent (33%) Frequent (79-30%) HP:0001394
22 cholelithiasis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001081
23 jaundice 59 32 occasional (7.5%) Occasional (29-5%) HP:0000952
24 pancreatitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001733
25 vitamin d deficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0100512
26 acute hepatic failure 59 32 very rare (1%) Very rare (<4-1%) HP:0006554
27 encephalopathy 59 32 very rare (1%) Very rare (<4-1%) HP:0001298
28 generalized amyotrophy 59 32 occasional (7.5%) Occasional (29-5%) HP:0003700
29 uveitis 59 32 very rare (1%) Very rare (<4-1%) HP:0000554
30 pleural effusion 59 32 occasional (7.5%) Occasional (29-5%) HP:0002202
31 hepatosplenomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0001433
32 prolonged prothrombin time 59 32 occasional (7.5%) Occasional (29-5%) HP:0008151
33 elevated alkaline phosphatase of hepatic origin 59 32 frequent (33%) Frequent (79-30%) HP:0010638
34 spider hemangioma 59 32 frequent (33%) Frequent (79-30%) HP:0012522
35 abnormal large intestine physiology 59 32 frequent (33%) Frequent (79-30%) HP:0012700
36 dilated superficial abdominal veins 59 32 frequent (33%) Frequent (79-30%) HP:0030168
37 ulcerative colitis 59 32 frequent (33%) Frequent (79-30%) HP:0100279
38 palmar telangiectasia 59 32 frequent (33%) Frequent (79-30%) HP:0100869
39 hepatocellular carcinoma 59 32 occasional (7.5%) Occasional (29-5%) HP:0001402
40 hypoalbuminemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003073
41 polyclonal elevation of igm 59 32 occasional (7.5%) Occasional (29-5%) HP:0003459
42 vitamin a deficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0004905
43 vitamin k deficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0011892
44 cholangiocarcinoma 59 32 occasional (7.5%) Occasional (29-5%) HP:0030153
45 adenocarcinoma of the large intestine 59 32 occasional (7.5%) Occasional (29-5%) HP:0040275
46 vitamin e deficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0100513
47 chronic hepatic failure 59 32 occasional (7.5%) Occasional (29-5%) HP:0100626
48 neoplasm of the gallbladder 59 32 very rare (1%) Very rare (<4-1%) HP:0100575
49 elevated hepatic transaminases 59 Frequent (79-30%)
50 cholestasis 59 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:

26 (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 HLA-A HLA-C CTSG GPR35
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-A HLA-C
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 GGT1 GPR35
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-A HLA-C
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-A HLA-C
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-A HLA-C
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-A HLA-C
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 GGT1 HLA-A HLA-C 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-A HLA-C GPR35
36 Increased shRNA abundance (Z-score > 2) GR00366-A-93 10.21 HLA-C

MGI Mouse Phenotypes related to Cholangitis, Primary Sclerosing:

46
# 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 103)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vancomycin Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 1404-90-6 14969 441141
2
Ofloxacin Approved Phase 4 82419-36-1 4583
3
Levofloxacin Approved, Investigational Phase 4 100986-85-4 149096
4
Ceftriaxone Approved Phase 4 73384-59-5 5479530 5361919
5
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
6
Prednisone Approved, Vet_approved Phase 4,Not Applicable 53-03-2 5865
7
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
8
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
9
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
10
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
11
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
12 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
13 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
14 Antibiotics, Antitubercular Phase 4,Phase 2,Not Applicable
15 Liver Extracts Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
16 Immunoglobulins Phase 4,Phase 2,Phase 1,Not Applicable
17 Immunosuppressive Agents Phase 4,Phase 2,Phase 1
18 Immunologic Factors Phase 4,Phase 2,Phase 1
19 Hormone Antagonists Phase 4,Phase 1
20 Immunoglobulin G Phase 4,Phase 1,Phase 2,Not Applicable
21 Antibodies Phase 4,Phase 2,Phase 1,Not Applicable
22 Antirheumatic Agents Phase 4,Phase 1,Phase 2
23 Hormones Phase 4,Phase 1
24 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 1
25 glucocorticoids Phase 4,Phase 1
26 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1
27 Bile Acids and Salts Phase 4,Phase 2,Phase 1
28 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Phase 1
29 Topoisomerase Inhibitors Phase 4
30 Cytochrome P-450 CYP1A2 Inhibitors Phase 4
31 Renal Agents Phase 4
32 Cytochrome P-450 Enzyme Inhibitors Phase 4
33 Anti-Infective Agents, Urinary Phase 4
34 Antitubercular Agents Phase 4
35 Methylprednisolone acetate Phase 4
36 Antineoplastic Agents, Hormonal Phase 4
37 Prednisolone acetate Phase 4
38
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
39
Ursodeoxycholic acid Approved, Investigational Phase 2, Phase 3,Phase 1 128-13-2 31401
40 Lipid Regulating Agents Phase 3,Phase 1,Phase 2
41 Antimetabolites Phase 3,Phase 1,Phase 2,Not Applicable
42 Hypolipidemic Agents Phase 3,Phase 1,Phase 2
43 Cholagogues and Choleretics Phase 2, Phase 3,Phase 1
44
Dobutamine Approved Phase 2 34368-04-2 36811
45
Norepinephrine Approved Phase 2 51-41-2 439260
46
chenodeoxycholic acid Approved Phase 2 474-25-9 10133
47
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
48
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
49
Sulfasalazine Approved Phase 2 599-79-1 5353980 5359476
50
Aldesleukin Approved Phase 2 85898-30-2, 110942-02-4

Interventional clinical trials:

(show top 50) (show all 99)
# Name Status NCT ID Phase Drugs
1 Effect and Safety of Oral Vancomycin in Primary Sclerosing Cholangitis Patients Unknown status NCT02605213 Phase 4 Vancomycin;Placebo
2 Antibiotics to Decrease Post ERCP Cholangitis Recruiting NCT03087656 Phase 4 Ceftriaxone;Levofloxacin
3 Leflunomide for Maintenance of Remission in IgG4 Related Disease Recruiting NCT02703194 Phase 4 Prednisone;Leflunomide
4 A Pilot Study to Characterize Bile Acid Metabolism and Dysbiosis in Primary Sclerosing Cholangitis Active, not recruiting NCT02464020 Phase 4 Vancomycin
5 Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis Unknown status NCT01398917 Phase 3
6 Probiotics in Patients With Primary Sclerosing Cholangitis Unknown status NCT00161148 Phase 3 Probiotics
7 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
8 Primary Sclerosing Cholangitis With Oral Vancomycin by the Study of Its Antimicrobial and Immunomodulating Effects Completed NCT01802073 Phase 3 Oral Vancomycin
9 Trial of High-dose Urso in Primary Sclerosing Cholangitis Completed NCT00059202 Phase 2, Phase 3 Ursodeoxycholic Acid
10 Vancomycin for Primary Sclerosing Cholangitis Recruiting NCT03710122 Phase 2, Phase 3 Vancomycin
11 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
12 Molecular Adsorbent Recirculating System (MARS®) in Hypoxic Hepatitis Unknown status NCT01690845 Phase 2
13 PERSEUS: Preliminary Efficacy and Safety of Cenicriviroc in Adult Participants With Primary Sclerosing Cholangitis Completed NCT02653625 Phase 2 Cenicriviroc 150 mg
14 Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis. Completed NCT02424175 Phase 1, Phase 2
15 Phase 2 Study of NGM282 in Patients With Primary Sclerosing Cholangitis Completed NCT02704364 Phase 2
16 Norursodeoxycholic Acid in the Treatment of Primary Sclerosing Cholangitis Completed NCT01755507 Phase 2 norUDCA;Placebo
17 Obeticholic Acid (OCA) in Primary Sclerosing Cholangitis (PSC) Completed NCT02177136 Phase 2 OCA;Placebo
18 Open Label Study to Evaluate Safety and Efficacy of LUM001 in Patients With Primary Sclerosing Cholangitis Completed NCT02061540 Phase 2 LUM001
19 Simtuzumab (GS-6624) in the Prevention of Progression of Liver Fibrosis in Subjects With Primary Sclerosing Cholangitis (PSC) Completed NCT01672853 Phase 2
20 Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis Completed NCT00004762 Phase 2 cladribine
21 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
22 Mitomycin C Therapy for Patients With Primary Sclerosing Cholangitis Recruiting NCT01688024 Phase 2 Mitomycin C;Normal saline
23 A Trial of BTT1023 in Patients With Primary Sclerosing Cholangitis Recruiting NCT02239211 Phase 2 BTT1023
24 A Research Study to Evaluate Safety and Efficacy of DUR-928 in Subjects With Primary Sclerosing Cholangitis (PSC) Recruiting NCT03394781 Phase 2 DUR-928
25 Sulfasalazine for the Treatment of Primary Sclerosing Cholangitis Recruiting NCT03561584 Phase 2 Sulfasalazine;Placebo
26 A POC and Dose-Ranging Study of HTD1801 in PSC Patients Recruiting NCT03333928 Phase 2 HTD1801;Placebo
27 An Efficacy Trial of Low Dose All-trans Retinoic Acid in Patients With Primary Sclerosing Cholangitis Recruiting NCT03359174 Phase 2 All-trans retinoic acid
28 Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases Recruiting NCT01988506 Phase 2 Interleukin 2
29 A Study Evaluating the Safety and Efficacy of Curcumin in Patients With Primary Sclerosing Cholangitis Active, not recruiting NCT02978339 Phase 1, Phase 2 Curcumin
30 Umbilical Cord Mesenchymal Stem Cells in Primary Sclerosing Cholangitis Active, not recruiting NCT03516006 Phase 1, Phase 2 UCMSC;UDCA
31 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
32 A Study of Oral Hymecromone to Treat Adults With Primary Sclerosing Cholangitis Not yet recruiting NCT02780752 Phase 1, Phase 2 hymecromone
33 Vidofludimus Calcium for Primary Sclerosing Cholangitis Not yet recruiting NCT03722576 Phase 2 Vidofludimus calcium
34 A Study of HTD1801 in Adolescents With Primary Sclerosing Cholangitis (PSC) Not yet recruiting NCT03678480 Phase 2 HTD1801;Ursodeoxycholic Acid
35 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
36 Thalidomide for the Treatment of Primary Sclerosing Cholangitis (PSC) Terminated NCT00953615 Phase 2 Thalidomide
37 Pilot Study of Fenofibrate for PSC Terminated NCT01142323 Phase 1, Phase 2 fenofibrate
38 The Value of CT Cholangiography in Primary Sclerosing Cholangitis Withdrawn NCT00588458 Phase 2
39 Gastrointestinal Microbiota in Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Completed NCT01322386 Phase 1 Vancomycin
40 Erlotinib for Chemoprevention in Trisomy 7 Positive Primary Sclerosing Cholangitis (PSC) Completed NCT00955149 Phase 1 Erlotinib (Tarceva)
41 Safety and Efficacy Study of Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis Completed NCT01088607 Phase 1 ursodeoxycholic acid (UDCA)
42 A Pilot Study of Vancomycin or Metronidazole in Patients With Primary Sclerosing Cholangitis Completed NCT01085760 Phase 1 Vancomycin;Vancomycin;Metronidazole;Metronidazole;Vancomycin
43 Minocycline in Primary Sclerosing Cholangitis (PSC) Completed NCT00630942 Phase 1 Minocycline
44 Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis Completed NCT00004842 Phase 1 budesonide
45 Evaluation of DHA for the Treatment of PSC Completed NCT00325013 Phase 1 Docosahexaenoic Acid (DHA)
46 A Pilot Study of Xifaxan to Treat Patients With PSC Completed NCT01695174 Phase 1 Xifaxan
47 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)
48 A Study of HTD1801 in Healthy Subjects Completed NCT03099603 Phase 1 HTD1801
49 Biliary Excretion of Conjugated Bile Acids in Humans Measured by 11C-cholylsarcosine PET/CT Completed NCT01879735 Phase 1 11C-CSar;ICG
50 Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Recruiting NCT02137668 Phase 1 Oral Vancomycin

Search NIH Clinical Center for Cholangitis, Primary Sclerosing

Cochrane evidence based reviews: cholangitis, sclerosing

Genetic Tests for Cholangitis, Primary Sclerosing

Anatomical Context for Cholangitis, Primary Sclerosing

MalaCards organs/tissues related to Cholangitis, Primary Sclerosing:

41
Liver, Thyroid, Spleen, Skin, Eye, Colon, Small Intestine

Publications for Cholangitis, Primary Sclerosing

Articles related to Cholangitis, Primary Sclerosing:

(show top 50) (show all 1217)
# Title Authors Year
1
Clinical guidelines for primary sclerosing cholangitis 2017. ( 29951926 )
2018
2
The gut microbiota, bile acids and their correlation in primary sclerosing cholangitis associated with inflammatory bowel disease. ( 29435321 )
2018
3
Symptoms do not Correlate With Findings From Colonoscopy in Children with Inflammatory Bowel Disease and Primary Sclerosing Cholangitis. ( 29378308 )
2018
4
Tumor Necrosis Factor Alpha Inhibition for Inflammatory Bowel Disease after Liver Transplant for Primary Sclerosing Cholangitis. ( 29862092 )
2018
5
Serum miRNA-122 is an independent biomarker of survival in patients with primary sclerosing cholangitis. ( 29922759 )
2018
6
Vedolizumab in patients with concurrent primary sclerosing cholangitis and inflammatory bowel disease does not improve liver biochemistry but is safe and effective for the bowel disease. ( 29377235 )
2018
7
Editorial: the role of colonic inflammation in the progression of liver disease in primary sclerosing cholangitis-Authors' reply and Letter: the effects of colectomy prior to the diagnosis of primary sclerosing cholangitis on prognosis may have been overestimated. ( 29314129 )
2018
8
Lack of Correlation of Liver Tests With Fibrosis Stage at Diagnosis in Pediatric Primary Sclerosing Cholangitis. ( 29356767 )
2018
9
The role of MRI and ERCP in the evaluation of disease activity and severity in primary sclerosing cholangitis. ( 29901259 )
2018
10
Peribiliary Cysts presenting as Primary Sclerosing Cholangitis. ( 29432923 )
2018
11
Letter: the effects of colectomy prior to the diagnosis of primary sclerosing cholangitis on prognosis may have been overestimated. ( 29314130 )
2018
12
Reviewing the Risk of Colorectal Cancer in Inflammatory Bowel Disease After Liver Trasnplantation for Primary Sclerosing Cholangitis. ( 29361103 )
2018
13
Endoscopic management of primary sclerosing cholangitis. ( 29883229 )
2018
14
Effects of vedolizumab, adalimumab and infliximab on biliary inflammation in individuals with primary sclerosing cholangitis and inflammatory bowel disease. ( 29808485 )
2018
15
Kindler syndrome in a patient with colitis and primary sclerosing cholangitis: coincidence or association? ( 29634879 )
2018
16
Diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. ( 29781738 )
2018
17
Serological markers of extracellular matrix remodeling predict transplant-free survival in primary sclerosing cholangitis. ( 29851098 )
2018
18
Update in the Care and Management of Patients with Primary Sclerosing Cholangitis. ( 29886518 )
2018
19
Patient-reported outcome measures used in patients with primary sclerosing cholangitis: a systematic review. ( 29976215 )
2018
20
Aberrant expression of redox regulatory proteins in patients with concomitant primary Sclerosing cholangitis/inflammatory bowel disease. ( 29852184 )
2018
21
Right Sided Colonic Juvenile Polyp (polyps) in Pediatric Primary Sclerosing Cholangitis Patients. ( 29931365 )
2018
22
Cholestasis after TIPS placement in a patient with primary sclerosing cholangitis: an uncommon complication. ( 29278002 )
2018
23
Review article: the evidence that vancomycin is a therapeutic option for primary sclerosing cholangitis. ( 29411404 )
2018
24
Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation: a Nordic Multicentre Study. ( 29301479 )
2018
25
An Imaging Biomarker for Assessing Hepatic Function in Patients with Primary Sclerosing Cholangitis. ( 29775791 )
2018
26
Primary sclerosing cholangitis is associated with abnormalities in CFTR. ( 29807875 )
2018
27
Crohn's Disease Behavior and Location is Altered when Associated with Primary Sclerosing Cholangitis. ( 29333790 )
2018
28
High Risk of Advanced Colorectal Neoplasia in Patients With Primary Sclerosing Cholangitis Associated With Inflammatory Bowel Disease. ( 29378311 )
2018
29
Association between smoking and risk of primary sclerosing cholangitis: A systematic review and meta-analysis. ( 29881604 )
2018
30
The impact of ileal pouch-anal anastomosis on graft survival following liver transplantation for primary sclerosing cholangitis. ( 29882252 )
2018
31
Editorial: the role of colonic inflammation in the progression of liver disease in patients with primary sclerosing cholangitis. ( 29314124 )
2018
32
Loss of tolerance to gut immunity protein, glycoprotein 2 (GP2) is associated with progressive disease course in primary sclerosing cholangitis. ( 29321484 )
2018
33
No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients With Primary Sclerosing Cholangitis. ( 29803836 )
2018
34
Quality of life and primary sclerosing cholangitis: the business of defining what counts. ( 29315689 )
2018
35
The impact of human leukocyte antigen donor and recipient serotyping and matching on liver transplant graft failure in primary sclerosing cholangitis, autoimmune hepatitis, and primary biliary cholangitis. ( 30136315 )
2018
36
Massive hemobilia following plastic stent removal in common bile duct cancer associated with primary sclerosing cholangitis (with video). ( 30056613 )
2018
37
Serum metabolites as diagnostic biomarkers for cholangiocarcinoma, hepatocellular carcinoma and primary sclerosing cholangitis. ( 30325540 )
2018
38
Recent developments in the research on biomarkers of cholangiocarcinoma in primary sclerosing cholangitis. ( 30266579 )
2018
39
Neoplastic transformation of peribiliary stem cell niche in cholangiocarcinoma arisen in primary sclerosing cholangitis. ( 30102768 )
2018
40
Identification of a serum biomarker panel for the differential diagnosis of cholangiocarcinoma and primary sclerosing cholangitis. ( 29707118 )
2018
41
Circulating markers of gut barrier function associated with disease severity in primary sclerosing cholangitis. ( 30269440 )
2018
42
Should we set-up routine screening for primary sclerosing cholangitis in all IBD patients? ( 30274792 )
2018
43
Non-invasive Prediction of High-risk Varices in Patients with Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. ( 30315285 )
2018
44
Metabolomic Profiling of Portal Blood and Bile Reveals Metabolic Signatures of Primary Sclerosing Cholangitis. ( 30332763 )
2018
45
Unique patterns of glycosylation in immunoglobulin subclass G4-related disease and primary sclerosing cholangitis. ( 30345709 )
2018
46
High serum sCD40 and a distinct colonic T-cell profile in ulcerative colitis associated with primary sclerosing cholangitis. ( 30383225 )
2018
47
Correlation between quantitative liver and spleen volumes and disease severity in primary sclerosing cholangitis as determined by Mayo risk score. ( 30396665 )
2018
48
Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis. ( 30411083 )
2018
49
Effect of NGM282, a FGF19 analogue, in Primary Sclerosing Cholangitis: a Multicentre, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial. ( 30414864 )
2018
50
Does colectomy affect the progression of primary sclerosing cholangitis? A systematic review and meta-analysis. ( 30425805 )
2018

Variations for Cholangitis, Primary Sclerosing

Cosmic variations for Cholangitis, Primary Sclerosing:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM520 KRAS biliary tract,bile duct,other,primary sclerosing cholangitis c.35G>T p.G12V 12:25245350-25245350 14
2 COSM522 KRAS biliary tract,bile duct,other,primary sclerosing cholangitis c.35G>C p.G12A 12:25245350-25245350 14
3 COSM517 KRAS biliary tract,bile duct,other,primary sclerosing cholangitis c.34G>A p.G12S 12:25245351-25245351 14
4 COSM521 KRAS biliary tract,gallbladder,other,inflammation c.35G>A p.G12D 12:25245350-25245350 9

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 recycling endosome membrane GO:0055038 9.5 CFTR HLA-A HLA-C
3 cell surface GO:0009986 9.43 CFTR CTSG ENO1 EPCAM HLA-A HLA-C
4 phagocytic vesicle membrane GO:0030670 9.33 DMBT1 HLA-A HLA-C
5 extracellular exosome GO:0070062 9.32 ABCB4 BPI CTSG DMBT1 ENO1 EPCAM
6 MHC class I protein complex GO:0042612 9.26 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 9.65 AIRE BPI CTSG HLA-A HLA-C
2 defense response to Gram-negative bacterium GO:0050829 9.5 BPI CTSG DMBT1
3 positive regulation of T cell mediated cytotoxicity GO:0001916 9.4 HLA-A HLA-C
4 negative regulation of growth of symbiont in host GO:0044130 9.37 CTSG MPO
5 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent GO:0002480 9.16 HLA-A HLA-C
6 xenobiotic transport GO:0042908 8.96 ABCB4 NR1I2
7 antigen processing and presentation of endogenous peptide antigen via MHC class Ib GO:0002476 8.62 HLA-A HLA-C

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.53 ABCB4 AIRE CFTR CTSG DLAT DMBT1

Sources for Cholangitis, Primary Sclerosing

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
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49 NCI
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51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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