MCID: PRM236
MIFTS: 55

Primary Biliary Cholangitis

Categories: Rare diseases, Liver diseases, Genetic diseases

Aliases & Classifications for Primary Biliary Cholangitis

MalaCards integrated aliases for Primary Biliary Cholangitis:

Name: Primary Biliary Cholangitis 53 59
Primary Biliary Cirrhosis 53 59 73
Familial Primary Biliary Cirrhosis 53 73
Pbc 53 59
Hanot's Cirrhosis 73
Hanot Syndrome 59

Characteristics:

Orphanet epidemiological data:

59
primary biliary cholangitis
Prevalence: 1-9/100000 (Europe),1-5/10000 (Worldwide),1-9/100000 (Worldwide),1-5/10000 (Europe),1-5/10000 (Spain),1-9/100000 (Iceland),1-5/10000 (Iceland),1-9/100000 (Netherlands),1-5/10000 (Netherlands),1-9/100000 (Denmark),1-5/10000 (Denmark),1-9/100000 (Finland),1-5/10000 (Finland),1-5/10000 (United Kingdom),1-9/100000 (United States),1-5/10000 (United States),1-9/100000 (Canada),1-5/10000 (Canada),1-9/100000 (Australia),1-9/100000 (Israel),1-9/100000 (China),1-9/100000 (Brunei Darussalam),1-5/10000 (Greece),1-9/100000 (Greece),1-9/100000 (United Kingdom); Age of onset: Adolescent,Adult,Elderly;

Classifications:

Orphanet: 59  
Rare hepatic diseases


Summaries for Primary Biliary Cholangitis

NIH Rare Diseases : 53 Primary biliary cholangitis (PBC) is a chronic disease that affects the bile ducts within the liver. The bile ducts carry a fluid called bile from the liver to the gallbladder, where it is stored. The bile ducts become inflamed and damaged, which causes bile to build up in the liver. This abnormal buildup destroys liver tissue and in later stages results in cirrhosis. Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men. The cause of this condition is unknown, but research suggests that it is an autoimmune disease. Ursodiol is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis.

MalaCards based summary : Primary Biliary Cholangitis, also known as primary biliary cirrhosis, is related to primary biliary cirrhosis and cholangitis. An important gene associated with Primary Biliary Cholangitis is TNFSF15 (TNF Superfamily Member 15), and among its related pathways/superpathways are PAK Pathway and Pathways in cancer. The drugs Ursodeoxycholic acid and chenodeoxycholic acid have been mentioned in the context of this disorder. Affiliated tissues include liver, t cells and bone, and related phenotypes are jaundice and pruritus

Wikipedia : 76 Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune... more...

Related Diseases for Primary Biliary Cholangitis

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

(show all 40)
# Related Disease Score Top Affiliating Genes
1 primary biliary cirrhosis 11.5
2 cholangitis 10.8
3 autoimmune addison disease 10.5 CTLA4 PTPN22
4 anterior uveitis 10.5 CTLA4 PTPN22
5 syringomyelia 10.4 NRAS VDR
6 malignant skin fibrous histiocytoma 10.4 CTLA4 NRAS
7 malignant dermis tumor 10.4 CTLA4 NRAS
8 autoimmune disease of endocrine system 10.2 CTLA4 PTPN22
9 hepatitis 10.1
10 sclerosing cholangitis 10.1
11 cholangitis, primary sclerosing 10.1
12 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1 CTLA4 PTPN22
13 autoimmune hepatitis 10.1
14 arthritis 9.9
15 mucositis 9.9
16 peritonitis 9.8
17 dermatitis herpetiformis 9.8
18 crohn's disease 9.8
19 depression 9.8
20 liver disease 9.8
21 encephalopathy 9.8
22 ulcerative colitis 9.8
23 colitis 9.8
24 biliary cirrhosis, primary, 1 9.8
25 crest syndrome 9.8
26 hepatocellular carcinoma 9.8
27 osteoporosis 9.8
28 rheumatoid arthritis 9.8
29 hypothyroidism 9.8
30 dermatitis herpetiformis, familial 9.8
31 hepatitis c 9.8
32 psoriatic arthritis 9.8
33 dermatitis 9.8
34 reynolds syndrome 9.8
35 cerebritis 9.8
36 thyroiditis 9.8
37 lupus erythematosus 9.8 CTLA4 IRF5 PTPN22
38 autoimmune disease 9.5 CTLA4 IRF5 PTPN22 VDR
39 diabetes mellitus, insulin-dependent 9.5 CTLA4 PTPN22 VDR
40 inflammatory bowel disease 8.7 CTLA4 IL12A IRF5 PTPN22 TNFSF15 VDR

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



Diseases related to Primary Biliary Cholangitis

Symptoms & Phenotypes for Primary Biliary Cholangitis

Human phenotypes related to Primary Biliary Cholangitis:

59 32 (show all 32)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 jaundice 59 32 frequent (33%) Frequent (79-30%) HP:0000952
2 pruritus 59 32 frequent (33%) Frequent (79-30%) HP:0000989
3 orthostatic hypotension 59 32 frequent (33%) Frequent (79-30%) HP:0001278
4 hepatic fibrosis 59 32 frequent (33%) Frequent (79-30%) HP:0001395
5 hepatic failure 59 32 frequent (33%) Frequent (79-30%) HP:0001399
6 hepatocellular carcinoma 59 32 frequent (33%) Frequent (79-30%) HP:0001402
7 portal hypertension 59 32 frequent (33%) Frequent (79-30%) HP:0001409
8 abnormality of lipid metabolism 59 32 frequent (33%) Frequent (79-30%) HP:0003119
9 elevated alkaline phosphatase 59 32 frequent (33%) Frequent (79-30%) HP:0003155
10 antinuclear antibody positivity 59 32 frequent (33%) Frequent (79-30%) HP:0003493
11 increased igm level 59 32 frequent (33%) Frequent (79-30%) HP:0003496
12 abnormality of the intrahepatic bile duct 59 32 frequent (33%) Frequent (79-30%) HP:0011040
13 onychomycosis 59 32 frequent (33%) Frequent (79-30%) HP:0012203
14 osteoporosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000939
15 excessive daytime somnolence 59 32 occasional (7.5%) Occasional (29-5%) HP:0001262
16 ascites 59 32 occasional (7.5%) Occasional (29-5%) HP:0001541
17 celiac disease 59 32 occasional (7.5%) Occasional (29-5%) HP:0002608
18 hypoalbuminemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003073
19 increased iga level 59 32 occasional (7.5%) Occasional (29-5%) HP:0003261
20 abdominal distention 59 32 occasional (7.5%) Occasional (29-5%) HP:0003270
21 hepatitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012115
22 fatigue 59 32 occasional (7.5%) Occasional (29-5%) HP:0012378
23 gastrointestinal inflammation 59 32 very rare (1%) Very rare (<4-1%) HP:0004386
24 hyperpigmentation of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0000953
25 cirrhosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001394
26 biliary cirrhosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0002613
27 conjugated hyperbilirubinemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002908
28 dermatographic urticaria 59 32 hallmark (90%) Very frequent (99-80%) HP:0011971
29 abnormality of the thyroid gland 59 32 frequent (33%) Frequent (79-30%) HP:0000820
30 recurrent fungal infections 59 Frequent (79-30%)
31 autoimmunity 59 Frequent (79-30%)
32 sleep disturbance 59 Occasional (29-5%)

MGI Mouse Phenotypes related to Primary Biliary Cholangitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 PRKCB PTPN22 SPIB TNFSF15 VDR CTLA4
2 immune system MP:0005387 9.36 PRKCB PTPN22 SPIB TNFSF15 VDR CTLA4

Drugs & Therapeutics for Primary Biliary Cholangitis

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

(show top 50) (show all 132)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ursodeoxycholic acid Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 128-13-2 31401
2
chenodeoxycholic acid Approved Phase 4,Phase 3,Phase 2,Early Phase 1 474-25-9 10133
3
Abatacept Approved Phase 4 332348-12-6 10237
4
Methionine Approved, Nutraceutical Phase 4,Phase 2 63-68-3 6137
5 Cholagogues and Choleretics Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
6 Gastrointestinal Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
7 Liver Extracts Phase 4,Phase 3,Phase 2,Not Applicable
8 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1
9 Cathartics Phase 4,Phase 3,Phase 2,Early Phase 1
10 Immunosuppressive Agents Phase 4,Phase 3,Not Applicable
11 Laxatives Phase 4,Phase 3,Phase 2,Early Phase 1
12
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
13
Fenofibrate Approved Phase 3,Phase 2,Phase 1 49562-28-9 3339
14
Copper Approved, Investigational Phase 3 7440-50-8 27099
15
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
16
Budesonide Approved Phase 3,Phase 1,Not Applicable 51333-22-3 63006 5281004
17
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
18
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
19
Colchicine Approved Phase 3 64-86-8 6167 2833
20
Molybdenum Approved Phase 3 7439-98-7 185498
21
Tauroursodeoxycholic acid Approved, Investigational Phase 3 14605-22-2 12443252
22
Metformin Approved Phase 2, Phase 3 657-24-9 14219 4091
23
Metronidazole Approved Phase 2, Phase 3 443-48-1 4173
24
leucovorin Approved, Nutraceutical Phase 3 58-05-9 143 6006
25
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
26 Hypolipidemic Agents Phase 3,Phase 2,Phase 1
27 Lipid Regulating Agents Phase 3,Phase 2,Phase 1
28 Antimetabolites Phase 3,Phase 2,Phase 1
29 Tetrathiomolybdate Phase 3
30 Atorvastatin Calcium Phase 3 134523-03-8
31 glucocorticoids Phase 3,Phase 1,Not Applicable
32 Respiratory System Agents Phase 3,Phase 1,Not Applicable
33 Reverse Transcriptase Inhibitors Phase 2, Phase 3,Not Applicable
34 Micronutrients Phase 3,Not Applicable
35 Chelating Agents Phase 3
36 Folic Acid Antagonists Phase 3
37 Taurochenodeoxycholic Acid Phase 3
38 Hormone Antagonists Phase 3,Phase 1,Not Applicable
39 Dermatologic Agents Phase 3,Phase 2
40 Trace Elements Phase 3,Not Applicable
41 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3,Phase 3,Not Applicable
42 Angiogenesis Inhibitors Phase 3
43 Hormones Phase 3,Phase 1,Not Applicable
44 Angiogenesis Modulating Agents Phase 3
45 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 1,Not Applicable
46 Anti-Asthmatic Agents Phase 3,Phase 1,Not Applicable
47 Peripheral Nervous System Agents Phase 3,Phase 1,Not Applicable
48 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 3
49 Vitamin B Complex Phase 3
50 Anticholesteremic Agents Phase 3,Phase 1

Interventional clinical trials:

(show top 50) (show all 103)
# Name Status NCT ID Phase Drugs
1 Effectiveness of S-adenosyl-L-methionine in Patients With Primary Biliary Cirrhosis Completed NCT02557360 Phase 4
2 Ursofalk Tablets (500 mg) Versus Ursofalk Capsules (250 mg) in the Treatment of Primary Biliary Cirrhosis Completed NCT01510860 Phase 4 UDCA (Ursodeoxycholic acid);UDCA (Ursodeoxycholic acid)
3 A Trial of 18-22mg/kg/d Ursodeoxycholic in Refractory Primary Biliary Cholangitis Recruiting NCT03345589 Phase 4 18-22mg/kg/d Ursodeoxycholic;13-15mg/kg/d Ursodeoxycholic
4 Phase 4 Study of Obeticholic Acid Evaluating Clinical Outcomes in Patients With Primary Biliary Cholangitis Recruiting NCT02308111 Phase 4 Obeticholic Acid (OCA);Placebo
5 Study of Abatacept (Orencia) to Treat Primary Biliary Cirrhosis Active, not recruiting NCT02078882 Phase 4
6 Fenofibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Unknown status NCT02823353 Phase 3 Fenofibrate;UDCA
7 Fenofibrate for Patients With Primary Biliary Cirrhosis Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02823366 Phase 3 Fenofibrate;UDCA
8 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
9 Study of Combivir for Patients With Primary Biliary Cirrhosis Completed NCT00490620 Phase 2, Phase 3 Combination antiviral therapy;Placebo
10 Safety and Efficacy of Tauroursodeoxycholic Acid Versus Ursofalk in the Treatment of Adult Primary Biliary Cirrhosis Completed NCT01857284 Phase 3 Tauroursodeoxycholic Acid Capsules;Ursodeoxycholic Acid Capsules
11 Study Comparing Tetrathiomolybdate vs Standard Treatment in Primary Biliary Cirrhosis Completed NCT00805805 Phase 3 Tetrathiomolybdate
12 Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis Completed NCT00844402 Phase 3 Atorvastatin
13 Low-Dose Oral Methotrexate Versus Colchicine for Primary Biliary Cirrhosis Completed NCT00004748 Phase 3 colchicine;methotrexate;ursodiol
14 Phase 3 Study of Bezafibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Completed NCT01654731 Phase 3 Bezafibrate;placebo
15 Ursodiol-Methotrexate for Primary Biliary Cirrhosis Completed NCT00006168 Phase 3 Methotrexate
16 Phase III Randomized Study of Ursodiol With Vs Without Methotrexate for Primary Biliary Cirrhosis Completed NCT00004784 Phase 3 methotrexate;ursodiol
17 Efficacy and Safety Study of TUDCA Compare UDCA to Treatment Chronic Cholestatic Liver Disease-PBC Completed NCT01829698 Phase 3 tauroursodeoxycholic;ursodeoxycholic acid
18 Seladelpar in Subjects With Primary Biliary Cholangitis (PBC) Recruiting NCT03301506 Phase 2, Phase 3 Seladelpar 2 mg Capsule;Seladelpar 5 mg Capsule;Seladelpar 10 mg Capsule
19 Use of Bezafibrate in Patients With Primary Biliary Cirrhosis to Archive Complete Biochemical Response in Non-responders Recruiting NCT02937012 Phase 3 Bezafibrate;Ursodeoxycholic Acid;Placebo (for Bezafibrate)
20 Fuzhenghuayu in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Recruiting NCT02916290 Phase 3 Fuzhenghuayu;UDCA
21 Fuzhenghuayu for Patients With PBC Who Had An Inadequate Response to Ursodeoxycholic Acid Recruiting NCT02916641 Phase 3 Fuzhenghuayu;UDCA
22 Effectiveness of Ursodeoxycholic Acid Use in the Prevention of Gallstone Formation After Sleeve Gastrectomy Recruiting NCT02721862 Phase 3 Ursodeoxycholic Acid;Placebo
23 Phase 3 Study of Obeticholic Acid in Patients With Primary Biliary Cirrhosis Active, not recruiting NCT01473524 Phase 3 Obeticholic Acid (OCA);Placebo
24 Metformin in Intrahepatic Cholestasis of Pregnancy Not yet recruiting NCT03056274 Phase 2, Phase 3 Metformin;Ursodeoxycholic Acid
25 Ursodeoxycholic Acid Plus Budesonide Versus Ursodeoxycholic Acid Alone in Primary Biliary Cirrhosis (PBC) Terminated NCT00746486 Phase 3 budesonide;budesonide placebo
26 Umbilical Cord Mesenchymal Stem Cells for Patients With Primary Biliary Cirrhosis Unknown status NCT01662973 Phase 1, Phase 2
27 Study of Rituximab in Patients With Steroid-Refractory Chronic Graft-Versus-Host Disease Unknown status NCT00472225 Phase 2 Rituximab
28 Initial Study of Rituximab to Treat Primary Biliary Cirrhosis Completed NCT00364819 Phase 1, Phase 2 rituximab
29 Moexipril for Primary Biliary Cirrhosis Completed NCT00588302 Phase 2 Moexipril
30 Use of Fenofibrate for Primary Biliary Cirrhosis Completed NCT00575042 Phase 2 Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle)
31 A Study of Efficacy and Safety of Ustekinumab in Patients With Primary Biliary Cirrhosis (PBC) Who Had an Inadequate Response to Ursodeoxycholic Acid Completed NCT01389973 Phase 2 ustekinumab 90 mg;ustekinumab 45 mg;ustekinumab 180 mg;Placebo
32 Phase 2b Study of NGM282 Extended Treatment in Patients With Primary Biliary Cirrhosis Completed NCT02135536 Phase 2
33 Phase 2 Study of NGM282 in Patients With Primary Biliary Cirrhosis Completed NCT02026401 Phase 2
34 Pentoxifylline for Primary Biliary Cirrhosis Completed NCT01249092 Phase 2 Pentoxifylline
35 B-Cell Depleting Therapy (Rituximab) as a Treatment for Fatigue in Primary Biliary Cirrhosis Completed NCT02376335 Phase 2
36 Phase 2 Study to Evaluate LUM001 in Combination With Ursodeoxycholic Acid in Patients With Primary Biliary Cirrhosis Completed NCT01904058 Phase 2 LUM001;Placebo;Ursodeoxycholic Acid
37 Phase 2 Study on Effects of Obeticholic Acid (OCA) on Lipoprotein Metabolism in Subjects With Primary Biliary Cirrhosis Completed NCT01865812 Phase 2 obeticholic acid (OCA)
38 A Study to Evaluate the Safety, Tolerability, Pharmacokinetics (PK) and Pharmacodynamics (PD) of Repeat Doses of GSK2330672 Administration in Subjects With Primary Biliary Cirrhosis (PBC) and Symptoms of Pruritus Completed NCT01899703 Phase 2 GSK2330672;Placebo;Ursodeoxycholic acid
39 Study of INT 747 in Combination With URSO in Patients With Primay Biliary Cirrhosis (PBC) Completed NCT00550862 Phase 2 INT-747;Ursodeoxycholic Acid (URSO);Placebo
40 Study of INT-747 as Monotherapy in Patients With PBC Completed NCT00570765 Phase 2 Placebo;INT-747;INT-747
41 Treatment of Cholestatic Pruritus With Sertraline Completed NCT00058903 Phase 2 sertraline
42 A Study to Evaluate Safety, Tolerability and Efficacy of Saroglitazar Magnesium in Patients With Primary Biliary Cholangitis (EPICS) Recruiting NCT03112681 Phase 2 Saroglitazar magnesium 2 mg;Saroglitazar magnesium 4 mg;Placebo Oral Tablet
43 Study of E6011 in Japanese Subjects With Primary Biliary Cholangitis Inadequately Responding to Ursodeoxycholic Acid Recruiting NCT03092765 Phase 2 E6011;Placebo
44 Safety, Tolerability, and Efficacy of Etrasimod (APD334) in Patients With Primary Biliary Cholangitis Recruiting NCT03155932 Phase 2 APD334
45 A Study to Assess the Safety, Tolerability, Pharmacokinetics and Efficacy of EDP-305 in Subjects With Primary Biliary Cholangitis Recruiting NCT03394924 Phase 2 EDP-305 Dose 1;EDP-305 Dose 2;Placebo
46 Study to Evaluate the Efficacy and Safety of Elafibranor in Patients With Primary Biliary Cholangitis (PBC) and Inadequate Response to Ursodeoxycholic Acid Recruiting NCT03124108 Phase 2 Elafibranor 80 mg;Elafibranor 120 mg;Placebo
47 A Multi-part, Double Blind Study to Assess Safety, Tolerability and Efficacy of Tropifexor (LJN452) in PBC Patients Recruiting NCT02516605 Phase 2 Part 1: LJN452;Part 1: Placebo;Part 2: LJN452 Dose level 1;Part 2: Placebo;Part 2: LJN452 Dose level 2
48 Study to Assess Safety and Efficacy of GKT137831 in Patients With Primary Biliary Cholangitis Receiving Ursodeoxycholic Acid. Recruiting NCT03226067 Phase 2 GKT137831;Placebo oral capsule
49 Non-comparative Study of BCD-085 in Combination With UDCA in Patients With Primary Biliary Cholangitis Recruiting NCT03476993 Phase 2
50 Dose Response Study of GSK2330672 for the Treatment of Pruritus in Patients With Primary Biliary Cholangitis Recruiting NCT02966834 Phase 2 Placebo;GSK2330672

Search NIH Clinical Center for Primary Biliary Cholangitis

Genetic Tests for Primary Biliary Cholangitis

Anatomical Context for Primary Biliary Cholangitis

MalaCards organs/tissues related to Primary Biliary Cholangitis:

41
Liver, T Cells, Bone, B Cells, Thyroid, Lung, Testes

Publications for Primary Biliary Cholangitis

Articles related to Primary Biliary Cholangitis:

(show top 50) (show all 1573)
# Title Authors Year
1
Serum levels of a cell death biomarker predict the development of cirrhosis-related conditions in primary biliary cholangitis. ( 29380060 )
2018
2
Improvement of primary biliary cholangitis (PBC) under treatment with sulfasalazine and abatacept. ( 29754140 )
2018
3
Rituximab therapy in rheumatoid arthritis and primary biliary cholangitis. ( 29338925 )
2018
4
Influence factors and a predictive scoring model for measuring the biochemical response of primary biliary cholangitis to ursodeoxycholic acid treatment. ( 29889683 )
2018
5
Primary Biliary Cholangitis and Autoimmune Hepatitis. ( 29751878 )
2018
6
IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary SjAPgren's syndrome: A case report. ( 29953016 )
2018
7
Patients with primary biliary cholangitis and fatigue present with depressive symptoms and selected cognitive deficits, but with normal attention performance and brain structure. ( 29320524 )
2018
8
Rituximab is Ineffective for Treatment of Fatigue in Primary Biliary Cholangitis: A phase-2 Randomised Controlled Trial. ( 29790196 )
2018
9
Lower Plasma Levels of IL-35 in Patients with Primary Biliary Cirrhosis. ( 29445068 )
2018
10
Palmoplantar keratoderma as a presenting sign of primary biliary cirrhosis. ( 29270458 )
2018
11
Progress in Primary Biliary Cholangitis. ( 29874531 )
2018
12
Primary biliary cholangitis triggered by DAAs-induced HCV clearance: a biological proof of concept. ( 29854373 )
2018
13
A Placebo-Controlled Trial of Bezafibrate in Primary Biliary Cholangitis. ( 29874528 )
2018
14
Increased risk of osteoporosis in patients with primary biliary cirrhosis. ( 29543880 )
2018
15
NELFCD and CTSZ loci are associated with jaundice-stage progression in primary biliary cholangitis in the Japanese population. ( 29795304 )
2018
16
Magnetic resonance imaging evidence of hippocampal structural changes in patients with primary biliary cholangitis. ( 29977030 )
2018
17
External validation of the United Kingdom-primary biliary cholangitis risk scores of patients with primary biliary cholangitis treated with ursodeoxycholic acid. ( 29881819 )
2018
18
Primary Biliary Cholangitis-A Autoimmune Hepatitis Overlap Syndrome. ( 29423670 )
2018
19
Effect of deferred or no treatment with ursodeoxycholic acid in patients with early primary biliary cholangitis. ( 29405510 )
2018
20
Platelet count to spleen thickness ratio is related to histologic severity of primary biliary cholangitis. ( 29443746 )
2018
21
Biomarkers for primary biliary cholangitis: current perspectives. ( 29950909 )
2018
22
Clinical application of the GLOBE and United Kingdom-primary biliary cholangitis risk scores in a trial cohort of patients with primary biliary cholangitis. ( 29881820 )
2018
23
A real-world observational cohort of patients with primary biliary cholangitis: TARGET-primary biliary cholangitis study design and rationale. ( 29761165 )
2018
24
Increased Waitlist Mortality and Lower Rate for Liver Transplantation in Hispanic Patients with Primary Biliary Cholangitis. ( 29427734 )
2018
25
Deviations in Peripheral Blood Cell Populations are Associated with the Stage of Primary Biliary Cholangitis and Presence of Itching. ( 29951695 )
2018
26
Risk of Cardiovascular Events in Patients with Primary Biliary Cholangitis - Systematic Review. ( 29951355 )
2018
27
A case series evaluating the impact of Hepatitis C eradication using direct acting antivirals on primary biliary cholangitis-associated autoimmunity. ( 29940867 )
2018
28
Serum Autotaxin Is a Useful Disease Progression Marker in Patients with Primary Biliary Cholangitis. ( 29802350 )
2018
29
Primary myelofibrosis but not autoimmune myelofibrosis accompanied by SjAPgren's syndrome and primary biliary cirrhosis in a patient with trisomy 8 mosaic: a case report and literature review. ( 29745886 )
2018
30
Environmental basis of primary biliary cholangitis. ( 29307284 )
2018
31
Primary Biliary Cholangitis, DNA and Beyond: The Relative Contribution of Genes. ( 29331078 )
2018
32
Genome-wide haplotype association analysis of primary biliary cholangitis risk in Japanese. ( 29773854 )
2018
33
Primary Biliary Cholangitis in British Columbia First Nations: Clinical features and discovery of novel genetic susceptibility loci. ( 29297981 )
2018
34
Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and SjAPgren syndrome: A case report. ( 29465536 )
2018
35
Implication of increased serum stromal cell-derived factor-1 for primary biliary cholangitis. ( 29414663 )
2018
36
NI-0801, an anti-chemokine (C-X-C motif) ligand 10 antibody, in patients with primary biliary cholangitis and an incomplete response to ursodeoxycholic acid. ( 29761166 )
2018
37
Association between STAT4 polymorphisms and risk of primary biliary cholangitis: a meta-analysis. ( 29956222 )
2018
38
The immunobiology of mucosal-associated invariant T cell (MAIT) function in primary biliary cholangitis: Regulation by cholic acid-induced Interleukin-7. ( 29429758 )
2018
39
Update on New Drugs and Those in Development for the Treatment of Primary Biliary Cholangitis. ( 29928160 )
2018
40
MicroRNA-223 and microRNA-21 in peripheral blood B cells associated with progression of primary biliary cholangitis patients. ( 28886078 )
2017
41
Regulatory T cells with a defect in inhibition on co-stimulation deteriorated primary biliary cholangitis. ( 29312539 )
2017
42
Primary Biliary Cholangitis (Primary Biliary Cirrhosis) ( 29083627 )
2017
43
Increased cholestatic enzymes in two patients with long-term history of ulcerative colitis: consider primary biliary cholangitis not always primary sclerosing cholangitis. ( 28951510 )
2017
44
Genetic Risk Factors for Autoimmune Thyroid Disease might Affect the Susceptibility to and Modulate the Progression of Primary Biliary Cholangitis. ( 28922436 )
2017
45
Interstitial granulomatous dermatitis associated with primary biliary cirrhosis. ( 28225148 )
2017
46
Coexistent Primary Biliary Cholangitis with CREST Syndrome (Reynolds Syndrome). ( 28602872 )
2017
47
A brief review on prognostic models of primary biliary cholangitis. ( 28913620 )
2017
48
Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study. ( 28187915 )
2017
49
New developments in the treatment of primary biliary cholangitis - role of obeticholic acid. ( 28860789 )
2017
50
Association between primary biliary cholangitis and osteoporosis: meta-analysis. ( 28948408 )
2017

Variations for Primary Biliary Cholangitis

Expression for Primary Biliary Cholangitis

Search GEO for disease gene expression data for Primary Biliary Cholangitis.

Pathways for Primary Biliary Cholangitis

Pathways related to Primary Biliary Cholangitis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.83 IL12A IL12RB1 NRAS PRKCB PTPN22
2 12.28 IL12A IL12RB1 NRAS PRKCB
3 11.54 CTLA4 IRF5 PTPN22 SPIB TNFSF15
4 11.08 IL12A IL12RB1 NRAS
5 10.87 IL12A PRKCB
6
Show member pathways
10.62 IL12A IL12RB1

GO Terms for Primary Biliary Cholangitis

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

# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.26 CTLA4 IRF5 PRKCB PTPN22
2 positive regulation of T cell mediated cytotoxicity GO:0001916 8.62 IL12A IL12RB1

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

# Name GO ID Score Top Affiliating Genes
1 interleukin-12 receptor binding GO:0005143 8.62 IL12A IL12RB1

Sources for Primary Biliary Cholangitis

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
46 MGI
49 NCI
50 NCIt
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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