PSC
MCID: CHL119
MIFTS: 62

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 58 77 54 13
Primary Sclerosing Cholangitis 12 77 54 26 60 38 30 15 74
Psc 58 26 60
Cholangitis, Sclerosing 45 74
Sclerosing Cholangitis 54 26
Cholangitis Primary Sclerosing 56

Characteristics:

Orphanet epidemiological data:

60
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: 60  
Rare hepatic diseases


Summaries for Cholangitis, Primary Sclerosing

NIH Rare Diseases : 54 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 Methylprednisolone hemisuccinate have been mentioned in the context of this disorder. Affiliated tissues include liver, thyroid and t cells, 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.

Genetics Home Reference : 26 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 : 58 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 : 77 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 296)
# Related Disease Score Top Affiliating Genes
1 cholangitis 31.8 ABCB4 CFTR DLAT GGT1 GPT
2 sclerosing cholangitis 31.3 ABCB4 BPI CFTR CTSG GGT1 HLA-C
3 cholestasis 31.0 ABCB4 GGT1 GPT NR1I2
4 bile duct disease 30.6 ABCB4 GGT1 GPT NR1I2
5 vasculitis 30.5 BPI CTSG MPO
6 bronchiectasis 30.5 BPI CFTR CTSG MPO
7 suppurative cholangitis 30.3 ABCB4 DLAT MPO
8 biliary tract disease 30.3 ABCB4 GGT1 NR1I2
9 cystic fibrosis 30.2 BPI CFTR CTSG MPO
10 liver disease 29.9 ABCB4 DLAT GGT1 GPT SEPSECS
11 autoimmune hepatitis 29.7 AIRE DLAT GGT1 GPT HLA-A SEPSECS
12 ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis 12.9
13 sclerosing cholangitis, neonatal 12.7
14 pancreatitis, sclerosing cholangitis, and sicca complex 12.4
15 isolated neonatal sclerosing cholangitis 12.3
16 bile duct cancer 11.7
17 immunodeficiency 14 11.3
18 intrahepatic cholangiocarcinoma 10.7
19 crohn's disease 10.6
20 autoimmune pancreatitis 10.5
21 hepatocellular carcinoma 10.4
22 cholecystolithiasis 10.4 ABCB4 GPR35
23 retroperitoneal fibrosis 10.4
24 autoimmune retinopathy 10.4 AIRE ENO1
25 leukemia 10.4
26 idiopathic bronchiectasis 10.3 CFTR HLA-A HLA-C
27 crescentic glomerulonephritis 10.3 BPI CTSG MPO
28 autoimmune disease 10.3
29 autoimmune disease 1 10.3
30 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.3
31 hemolytic anemia 10.3
32 inflammatory bowel disease 10.3
33 hepatic tuberculosis 10.3 GGT1 GPT
34 granulomatosis with polyangiitis 10.3 BPI CTSG MPO
35 pityriasis rosea 10.3 HLA-A HLA-C
36 ichthyosis 10.3
37 anemia, autoimmune hemolytic 10.3
38 degos 'en cocarde' erythrokeratoderma 10.3
39 igg4-related sclerosing cholangitis 10.3
40 cryptosporidiosis 10.3
41 extrahepatic cholestasis 10.2 ABCB4 GGT1 GPT MPO
42 human herpesvirus 8 10.2 HLA-A HLA-C
43 varicose veins 10.2
44 lymphoma 10.2
45 colitis 10.2
46 pouchitis 10.2
47 viral hepatitis 10.2 GGT1 GPT SEPSECS
48 acquired immunodeficiency syndrome 10.2
49 autoimmune disease of urogenital tract 10.2 ABCB4 DLAT GGT1 SEPSECS
50 acute proliferative glomerulonephritis 10.2 BPI MPO

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:

60 33 (show top 50) (show all 55)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 autoimmunity 60 33 hallmark (90%) Very frequent (99-80%) HP:0002960
2 splenomegaly 60 33 frequent (33%) Frequent (79-30%) HP:0001744
3 hepatomegaly 60 33 frequent (33%) Frequent (79-30%) HP:0002240
4 portal hypertension 60 33 frequent (33%) Frequent (79-30%) HP:0001409
5 fever 60 33 frequent (33%) Frequent (79-30%) HP:0001945
6 ascites 60 33 frequent (33%) Frequent (79-30%) HP:0001541
7 weight loss 60 33 frequent (33%) Frequent (79-30%) HP:0001824
8 elevated hepatic transaminase 60 33 frequent (33%) Frequent (79-30%) HP:0002910
9 hepatic fibrosis 60 33 frequent (33%) Frequent (79-30%) HP:0001395
10 cirrhosis 60 33 frequent (33%) Frequent (79-30%) HP:0001394
11 hepatosplenomegaly 60 33 frequent (33%) Frequent (79-30%) HP:0001433
12 elevated alkaline phosphatase of hepatic origin 60 33 frequent (33%) Frequent (79-30%) HP:0010638
13 spider hemangioma 60 33 frequent (33%) Frequent (79-30%) HP:0012522
14 abnormal large intestine physiology 60 33 frequent (33%) Frequent (79-30%) HP:0012700
15 dilated superficial abdominal veins 60 33 frequent (33%) Frequent (79-30%) HP:0030168
16 ulcerative colitis 60 33 frequent (33%) Frequent (79-30%) HP:0100279
17 palmar telangiectasia 60 33 frequent (33%) Frequent (79-30%) HP:0100869
18 depressivity 60 33 occasional (7.5%) Occasional (29-5%) HP:0000716
19 osteopenia 60 33 occasional (7.5%) Occasional (29-5%) HP:0000938
20 renal insufficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0000083
21 fatigue 60 33 occasional (7.5%) Occasional (29-5%) HP:0012378
22 type i diabetes mellitus 60 33 occasional (7.5%) Occasional (29-5%) HP:0100651
23 osteoporosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000939
24 hepatitis 60 33 occasional (7.5%) Occasional (29-5%) HP:0012115
25 abdominal pain 60 33 occasional (7.5%) Occasional (29-5%) HP:0002027
26 pruritus 60 33 occasional (7.5%) Occasional (29-5%) HP:0000989
27 congestive heart failure 60 33 occasional (7.5%) Occasional (29-5%) HP:0001635
28 celiac disease 60 33 occasional (7.5%) Occasional (29-5%) HP:0002608
29 thyroiditis 60 33 occasional (7.5%) Occasional (29-5%) HP:0100646
30 cholelithiasis 60 33 occasional (7.5%) Occasional (29-5%) HP:0001081
31 jaundice 60 33 occasional (7.5%) Occasional (29-5%) HP:0000952
32 pancreatitis 60 33 occasional (7.5%) Occasional (29-5%) HP:0001733
33 vitamin d deficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0100512
34 generalized amyotrophy 60 33 occasional (7.5%) Occasional (29-5%) HP:0003700
35 chronic hepatic failure 60 33 occasional (7.5%) Occasional (29-5%) HP:0100626
36 pleural effusion 60 33 occasional (7.5%) Occasional (29-5%) HP:0002202
37 prolonged prothrombin time 60 33 occasional (7.5%) Occasional (29-5%) HP:0008151
38 hypoalbuminemia 60 33 occasional (7.5%) Occasional (29-5%) HP:0003073
39 hepatocellular carcinoma 60 33 occasional (7.5%) Occasional (29-5%) HP:0001402
40 polyclonal elevation of igm 60 33 occasional (7.5%) Occasional (29-5%) HP:0003459
41 vitamin a deficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0004905
42 vitamin k deficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0011892
43 cholangiocarcinoma 60 33 occasional (7.5%) Occasional (29-5%) HP:0030153
44 adenocarcinoma of the large intestine 60 33 occasional (7.5%) Occasional (29-5%) HP:0040275
45 vitamin e deficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0100513
46 acute hepatic failure 60 33 very rare (1%) Very rare (<4-1%) HP:0006554
47 encephalopathy 60 33 very rare (1%) Very rare (<4-1%) HP:0001298
48 uveitis 60 33 very rare (1%) Very rare (<4-1%) HP:0000554
49 neoplasm of the gallbladder 60 33 very rare (1%) Very rare (<4-1%) HP:0100575
50 cholestasis 60 Very frequent (99-80%)

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:

27 (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

Drugs & Therapeutics for Cholangitis, Primary Sclerosing

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

(show top 50) (show all 104)
# 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
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
3
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
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
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
8
Ceftriaxone Approved Phase 4 73384-59-5 5479530 5361919
9
Ofloxacin Approved Phase 4 82419-36-1 4583
10
Levofloxacin Approved, Investigational Phase 4 100986-85-4 149096
11
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
12 Anti-Bacterial Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
13 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
14 Liver Extracts Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
15 Antibiotics, Antitubercular Phase 4,Phase 2,Not Applicable
16 glucocorticoids Phase 4,Phase 1
17 Immunoglobulin G Phase 4,Phase 1,Phase 2,Not Applicable
18 Hormone Antagonists Phase 4,Phase 1
19 Methylprednisolone Acetate Phase 4
20 Prednisolone acetate Phase 4
21 Antibodies Phase 4,Phase 2,Phase 1,Not Applicable
22 Anti-Inflammatory Agents Phase 4,Phase 1,Phase 2
23 Hormones Phase 4,Phase 2,Phase 1
24 Immunologic Factors Phase 4,Phase 2,Phase 1
25 Antineoplastic Agents, Hormonal Phase 4
26 Immunosuppressive Agents Phase 4,Phase 2
27 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1
28 Immunoglobulins Phase 4,Phase 2,Phase 1,Not Applicable
29 Anti-Infective Agents, Urinary Phase 4
30 Cytochrome P-450 Enzyme Inhibitors Phase 4
31 Topoisomerase Inhibitors Phase 4
32 Cytochrome P-450 CYP1A2 Inhibitors Phase 4
33 Antitubercular Agents Phase 4
34 Renal Agents Phase 4
35
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
36
Ursodeoxycholic acid Approved, Investigational Phase 2, Phase 3,Phase 1 128-13-2 31401
37 Hypolipidemic Agents Phase 3,Phase 1,Phase 2
38 Lipid Regulating Agents Phase 3,Phase 1,Phase 2
39 Antimetabolites Phase 3,Phase 1,Phase 2,Not Applicable
40 Gastrointestinal Agents Phase 2, Phase 3,Phase 3,Phase 1
41 Cholagogues and Choleretics Phase 2, Phase 3,Phase 1
42
Norepinephrine Approved Phase 2 51-41-2 439260
43
Dobutamine Approved Phase 2 34368-04-2 36811
44
Curcumin Approved, Experimental, Investigational Phase 1, Phase 2 458-37-7 969516
45
chenodeoxycholic acid Approved Phase 2 474-25-9 10133
46
Cladribine Approved, Investigational Phase 2 4291-63-8 20279
47
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
48
Sulfasalazine Approved Phase 2 599-79-1 5359476 5353980
49
Aldesleukin Approved Phase 2 110942-02-4, 85898-30-2
50
Fenofibrate Approved Phase 1, Phase 2 49562-28-9 3339

Interventional clinical trials:

(show top 50) (show all 104)
# 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 Leflunomide for Maintenance of Remission in IgG4 Related Disease Completed NCT02703194 Phase 4 Prednisone;Leflunomide
3 Antibiotics to Decrease Post ERCP Cholangitis Recruiting NCT03087656 Phase 4 Ceftriaxone;Levofloxacin
4 Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis Unknown status NCT01398917 Phase 3
5 Probiotics in Patients With Primary Sclerosing Cholangitis Unknown status NCT00161148 Phase 3 Probiotics
6 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
7 Primary Sclerosing Cholangitis With Oral Vancomycin by the Study of Its Antimicrobial and Immunomodulating Effects Completed NCT01802073 Phase 3 Oral Vancomycin
8 Trial of High-dose Urso in Primary Sclerosing Cholangitis Completed NCT00059202 Phase 2, Phase 3 Ursodeoxycholic Acid
9 Vancomycin for Primary Sclerosing Cholangitis Recruiting NCT03710122 Phase 2, Phase 3 Vancomycin
10 Safety, Tolerability, and Efficacy of Cilofexor in Non-Cirrhotic Adults With Primary Sclerosing Cholangitis (PSC-Phase 3) Recruiting NCT03890120 Phase 3 Cilofexor;Placebo
11 Norursodeoxycholic Acid vs Placebo in PSC Recruiting NCT03872921 Phase 3 Norursocholic Acid
12 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
13 Molecular Adsorbent Recirculating System (MARS®) in Hypoxic Hepatitis Unknown status NCT01690845 Phase 2
14 PERSEUS: Preliminary Efficacy and Safety of Cenicriviroc in Adult Participants With Primary Sclerosing Cholangitis Completed NCT02653625 Phase 2 Cenicriviroc 150 mg
15 Fecal Microbiota Transplantation for the Treatment of Primary Sclerosing Cholangitis. Completed NCT02424175 Phase 1, Phase 2
16 A Trial of BTT1023 in Patients With Primary Sclerosing Cholangitis Completed NCT02239211 Phase 2 BTT1023
17 A Study Evaluating the Safety and Efficacy of Curcumin in Patients With Primary Sclerosing Cholangitis Completed NCT02978339 Phase 1, Phase 2 Curcumin
18 Phase 2 Study of NGM282 in Patients With Primary Sclerosing Cholangitis Completed NCT02704364 Phase 2
19 Norursodeoxycholic Acid in the Treatment of Primary Sclerosing Cholangitis Completed NCT01755507 Phase 2 norUDCA;Placebo
20 Obeticholic Acid (OCA) in Primary Sclerosing Cholangitis (PSC) Completed NCT02177136 Phase 2 OCA;Placebo
21 Open Label Study to Evaluate Safety and Efficacy of LUM001 in Patients With Primary Sclerosing Cholangitis Completed NCT02061540 Phase 2 LUM001
22 Simtuzumab (GS-6624) in the Prevention of Progression of Liver Fibrosis in Subjects With Primary Sclerosing Cholangitis (PSC) Completed NCT01672853 Phase 2
23 Phase II Pilot Study of Cladribine (2-Chlorodeoxyadenosine; 2-CdA) for Early Stage Primary Sclerosing Cholangitis Completed NCT00004762 Phase 2 cladribine
24 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
25 Mitomycin C Therapy for Patients With Primary Sclerosing Cholangitis Recruiting NCT01688024 Phase 2 Mitomycin C;Normal saline
26 A Research Study to Evaluate Safety and Efficacy of DUR-928 in Subjects With Primary Sclerosing Cholangitis (PSC) Recruiting NCT03394781 Phase 2 DUR-928
27 Sulfasalazine for the Treatment of Primary Sclerosing Cholangitis Recruiting NCT03561584 Phase 2 Sulfasalazine;Placebo
28 A POC and Dose-Ranging Study of HTD1801 in PSC Patients Recruiting NCT03333928 Phase 2 HTD1801;Placebo
29 An Efficacy Trial of Low Dose All-trans Retinoic Acid in Patients With Primary Sclerosing Cholangitis Recruiting NCT03359174 Phase 2 All-trans retinoic acid
30 A Single-arm,Phase IIa,Safety and Efficacy Trial of Selected MSCs in the Treatment of Patients With PSC & AiH Recruiting NCT02997878 Phase 1, Phase 2
31 Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases Recruiting NCT01988506 Phase 2 Interleukin 2
32 Umbilical Cord Mesenchymal Stem Cells in Primary Sclerosing Cholangitis Active, not recruiting NCT03516006 Phase 1, Phase 2 UCMSC;UDCA
33 Safety, Tolerability, and Efficacy of Cilofexor in Adults With Primary Sclerosing Cholangitis Without Cirrhosis Active, not recruiting NCT02943460 Phase 2 Cilofexor;Placebo to match Cilofexor
34 Vidofludimus Calcium for Primary Sclerosing Cholangitis Not yet recruiting NCT03722576 Phase 2 Vidofludimus calcium
35 A Study of HTD1801 in Adolescents With Primary Sclerosing Cholangitis (PSC) Not yet recruiting NCT03678480 Phase 2 HTD1801;Ursodeoxycholic Acid
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 A Study of Oral Hymecromone to Treat Adults With Primary Sclerosing Cholangitis Withdrawn NCT02780752 Phase 1, Phase 2 hymecromone
39 The Value of CT Cholangiography in Primary Sclerosing Cholangitis Withdrawn NCT00588458 Phase 2
40 Gastrointestinal Microbiota in Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin Completed NCT01322386 Phase 1 Vancomycin
41 A Pilot Study to Characterize Bile Acid Metabolism and Dysbiosis in Primary Sclerosing Cholangitis Completed NCT02464020 Phase 1 Vancomycin
42 Erlotinib for Chemoprevention in Trisomy 7 Positive Primary Sclerosing Cholangitis (PSC) Completed NCT00955149 Phase 1 Erlotinib (Tarceva)
43 Safety and Efficacy Study of Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis Completed NCT01088607 Phase 1 ursodeoxycholic acid (UDCA)
44 A Pilot Study of Vancomycin or Metronidazole in Patients With Primary Sclerosing Cholangitis Completed NCT01085760 Phase 1 Vancomycin;Vancomycin;Metronidazole;Metronidazole;Vancomycin
45 Minocycline in Primary Sclerosing Cholangitis (PSC) Completed NCT00630942 Phase 1 Minocycline
46 Pilot Study of Budesonide for Patients With Primary Sclerosing Cholangitis Completed NCT00004842 Phase 1 budesonide
47 Evaluation of DHA for the Treatment of PSC Completed NCT00325013 Phase 1 Docosahexaenoic Acid (DHA)
48 A Pilot Study of Xifaxan to Treat Patients With PSC Completed NCT01695174 Phase 1 Xifaxan
49 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)
50 A Study 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 30

Anatomical Context for Cholangitis, Primary Sclerosing

MalaCards organs/tissues related to Cholangitis, Primary Sclerosing:

42
Liver, Thyroid, T Cells, Colon, Skin, Spleen, Bone

Publications for Cholangitis, Primary Sclerosing

Articles related to Cholangitis, Primary Sclerosing:

(show top 50) (show all 2038)
# Title Authors Year
1
Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan. ( 30932290 )
2019
2
Important problems in the diagnosis and treatment of primary sclerosing cholangitis (based on the Russian consensus on diagnosis and treatment autoimmune hepatitis. Moscow, 2018). ( 31094167 )
2019
3
Massive hemobilia following plastic stent removal in common bile duct cancer associated with primary sclerosing cholangitis (with video). ( 30056613 )
2019
4
Longitudinal analysis of CA19-9 reveals individualised normal range and early changes before development of biliary tract cancer in patients with primary sclerosing cholangitis. ( 30687954 )
2019
5
Cholangiocarcinoma is associated with a raised enhanced liver fibrosis (ELF) score independent of primary sclerosing cholangitis. ( 30762236 )
2019
6
Return to sender: Lymphocyte trafficking mechanisms as contributors to primary sclerosing cholangitis. ( 31108158 )
2019
7
The Nonsteroidal Farnesoid X Receptor Agonist Cilofexor (GS-9674) Improves Markers of Cholestasis and Liver Injury in Patients With Primary Sclerosing Cholangitis. ( 30661255 )
2019
8
A pilot study of fecal bile acid and microbiota profiles in inflammatory bowel disease and primary sclerosing cholangitis. ( 30666146 )
2019
9
Specific Features of Patients With Inflammatory Bowel Disease and Primary Sclerosing Cholangitis. ( 30700999 )
2019
10
Treatment of primary sclerosing cholangitis in children. ( 30705716 )
2019
11
Severe hypocalcemia following denosumab treatment in a patient with secondary osteoporosis associated with primary sclerosing cholangitis. ( 30713246 )
2019
12
Primary Sclerosing Cholangitis: A Concise Review of Diagnosis and Management. ( 30725292 )
2019
13
Fecal Microbiota Transplantation in Patients With Primary Sclerosing Cholangitis: A Pilot Clinical Trial. ( 30730351 )
2019
14
In vivo 1 H MRS of human gallbladder bile in understanding the pathophysiology of primary sclerosing cholangitis (PSC): Immune-mediated disease versus bile acid-induced injury. ( 30735273 )
2019
15
Systematic review with meta-analysis: risk factors for recurrent primary sclerosing cholangitis after liver transplantation. ( 30740723 )
2019
16
Predictive criteria of response to endoscopic treatment for severe strictures in primary sclerosing cholangitis. ( 30772328 )
2019
17
Evolving role of magnetic resonance techniques in primary sclerosing cholangitis. ( 30783369 )
2019
18
Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies. ( 30783370 )
2019
19
CCL21-expression and accumulation of CCR7+ NK cells in livers of patients with primary sclerosing cholangitis. ( 30785638 )
2019
20
Role of endoscopy in the management of primary sclerosing cholangitis. ( 30788027 )
2019
21
Primary sclerosing cholangitis and inflammatory bowel disease comorbidity: an update of the evidence. ( 30837784 )
2019
22
Oral Vancomycin Induces and Maintains Remission of Ulcerative Colitis in the Subset of Patients With Associated Primary Sclerosing Cholangitis. ( 30838381 )
2019
23
Intestinal dysbiosis augments liver disease progression via NLRP3 in a murine model of primary sclerosing cholangitis. ( 30872395 )
2019
24
Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis: Predictors of Gamma Glutamyltransferase Normalization and Favorable Clinical Course. ( 30878206 )
2019
25
Magnetic Resonance Elastography in Primary Sclerosing Cholangitis: Interobserver Agreement for Liver Stiffness Measurement with Manual and Automated Methods. ( 30878345 )
2019
26
Use of Fenofibrate for patients with primary Sclerosing Cholangitis. ( 30880099 )
2019
27
Simple Magnetic Resonance Scores Associate With Outcomes of Patients With Primary Sclerosing Cholangitis. ( 30880273 )
2019
28
Effect of scheduled endoscopic dilatation of dominant strictures on outcome in patients with primary sclerosing cholangitis. ( 30910856 )
2019
29
Manipulation of the Gut-Liver Axis Using Microbiome Restoration Therapy in Primary Sclerosing Cholangitis. ( 30920413 )
2019
30
Primary sclerosing cholangitis and inflammatory bowel disease: Intestine-liver interrelation. ( 30948141 )
2019
31
Duck circovirus induces a new pathogenetic characteristic, primary sclerosing cholangitis. ( 30961815 )
2019
32
Fungi participate in the dysbiosis of gut microbiota in patients with primary sclerosing cholangitis. ( 31003979 )
2019
33
The Use of Biologics in Patients with Inflammatory Bowel Disease and Primary Sclerosing Cholangitis. ( 31008013 )
2019
34
The gut-liver axis in PSC: Are pathobionts the missing link? ( 31013359 )
2019
35
The "pruned tree" appearance of primary sclerosing cholangitis. ( 31025068 )
2019
36
Microbiota as a cornerstone in the development of primary sclerosing cholangitis: paving the path for translational diagnostic and therapeutic approaches. ( 31028154 )
2019
37
Magnetic Resonance Imaging in Primary Sclerosing Cholangitis-Current State and Future Directions. ( 31041791 )
2019
38
The role of imaging in determining prognosis for primary sclerosing cholangitis: A systematic review. ( 31044745 )
2019
39
Vedolizumab therapy is ineffective for primary sclerosing cholangitis in patients with inflammatory bowel disease: A GETAID multicentre cohort study. ( 31056693 )
2019
40
Clinical characteristics, associated malignancies and management of primary sclerosing cholangitis in inflammatory bowel disease patients: A multicenter retrospective cohort study. ( 31063540 )
2019
41
Is Magnetic Resonance Cholangiopancreatography Worth a Thousand Words in Determining Prognoses of Patients With Primary Sclerosing Cholangitis? ( 31077831 )
2019
42
Precision Medicine in Primary Sclerosing Cholangitis. ( 31099965 )
2019
43
Effects of Vedolizumab in Patients with Primary Sclerosing Cholangitis and Inflammatory Bowel Diseases. ( 31100458 )
2019
44
Non-Invasive Prediction of High-Risk Varices in Patients with Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. ( 30315285 )
2019
45
High Serum sCD40 and a Distinct Colonic T Cell Profile in Ulcerative Colitis Associated With Primary Sclerosing Cholangitis. ( 30383225 )
2019
46
Effect of NGM282, an FGF19 analogue, in primary sclerosing cholangitis: A multicenter, randomized, double-blind, placebo-controlled phase II trial. ( 30414864 )
2019
47
Primary Sclerosing Cholangitis and Psychological Wellbeing: A Scoping Review. ( 30466122 )
2019
48
Diagnostic performance of a stepwise cytological algorithm for biliary malignancy in primary sclerosing cholangitis. ( 30507030 )
2019
49
Beaded bile ducts in primary sclerosing cholangitis. ( 30600377 )
2019
50
Rate of Spleen Length Progression is a Marker of Outcome in Patients with Primary Sclerosing Cholangitis. ( 30616025 )
2019

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 COSM521 KRAS biliary tract,gallbladder,other,inflammation c.35G>A p.G12D 12:25245350-25245350 0
2 COSM520 KRAS biliary tract,bile duct,other,primary sclerosing cholangitis c.35G>T p.G12V 12:25245350-25245350 0
3 COSM522 KRAS biliary tract,bile duct,other,primary sclerosing cholangitis c.35G>C p.G12A 12:25245350-25245350 0
4 COSM517 KRAS biliary tract,bile duct,other,primary sclerosing cholangitis c.34G>A p.G12S 12:25245351-25245351 0

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 10.66 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 extracellular space GO:0005615 9.92 BPI CTSG DMBT1 ENO1 GGT1 GPT
2 cell surface GO:0009986 9.8 CFTR CTSG ENO1 HLA-A HLA-C
3 azurophil granule lumen GO:0035578 9.54 BPI CTSG MPO
4 recycling endosome membrane GO:0055038 9.43 CFTR HLA-A HLA-C
5 extracellular exosome GO:0070062 9.32 ABCB4 BPI CTSG DMBT1 ENO1 GGT1
6 MHC class I protein complex GO:0042612 9.16 HLA-A HLA-C
7 phagocytic vesicle membrane GO:0030670 9.13 DMBT1 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 8.65 NR1I2
7 antigen processing and presentation of endogenous peptide antigen via MHC class Ib GO:0002476 8.62 HLA-A HLA-C

Sources for Cholangitis, Primary Sclerosing

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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