MCID: PRM236
MIFTS: 63

Primary Biliary Cholangitis

Categories: Genetic diseases, Immune diseases, Liver diseases, Rare diseases

Aliases & Classifications for Primary Biliary Cholangitis

MalaCards integrated aliases for Primary Biliary Cholangitis:

Name: Primary Biliary Cholangitis 54 60
Primary Biliary Cirrhosis 54 60 74
Familial Primary Biliary Cirrhosis 54 74
Pbc 54 60
Hanot's Cirrhosis 74
Hanot Syndrome 60

Characteristics:

Orphanet epidemiological data:

60
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: 60  
Rare hepatic diseases


Summaries for Primary Biliary Cholangitis

NIH Rare Diseases : 54 Primary biliary cholangitis (PBC) is a chronic, progressive liver disease in which the bile ducts become inflamed and damaged. This leads to the buildup of bile and causes liver problems such as scarring, cirrhosis (scarring and poor liver function), and eventual liver failure. PBC is more common in women. Many people do not have symptoms when they are first diagnosed and may not develop symptoms for several years. Early symptoms may include fatigue (the most common symptom), itchy skin (pruritus), and abdominal pain. As the disease progresses, people with PBC may develop weakness, nausea, diarrhea, swelling in the legs and feet (edema), bone and joint pain, jaundice, dark urine, and xanthomas. The symptoms of PBC can significantly impair quality of life. The diagnosis of PBC may involve blood tests, imaging studies (such as X-ray or ultrasound), and sometimes, a liver biopsy. Blood tests may include tests for anti-mitochondrial antibodies (which may confirm the diagnosis), liver function tests, and cholesterol tests. Abnormal blood test results commonly lead to the diagnosis in people with PBC who do not have symptoms. PBC is considered an autoimmune disease in which the immune system malfunctions and mistakenly attacks a person�??s healthy bile duct cells, causing the inflammation and damage. It is thought to be caused by a combination of genetic susceptibility and environmental triggers (multifactorial inheritance). The first treatment recommended for people with PBC is ursodiol, also called ursodeoxycholic acid (UDCA), which has been shown to slow disease progression and reduce the need for a liver transplant.Obeticholic acid (OCA) is available as a second-line treatment either in combination with UDCA (in those with an inadequate response to UDCA), or by itself (in those who are not able to tolerate UDCA). The symptoms of PBC typically do not improve with UDCA or OCA, so individual symptoms are treated separately. A liver transplant may be needed when PBC leads to liver failure. The rate of progression varies greatly among people with PBC, and the disease may progress over many decades before resulting in end-stage liver disease and its complications.

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

Wikipedia : 77 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 top 50) (show all 325)
# Related Disease Score Top Affiliating Genes
1 primary biliary cirrhosis 11.7
2 reynolds syndrome 11.6
3 cholangitis 11.2
4 biliary cirrhosis, primary, 2 11.2
5 biliary cirrhosis, primary, 3 11.2
6 biliary cirrhosis, primary, 4 11.2
7 biliary cirrhosis, primary, 5 11.2
8 nodular regenerative hyperplasia 11.0
9 hepatitis 10.5
10 cholangitis, primary sclerosing 10.5
11 autoimmune hepatitis 10.5
12 sclerosing cholangitis 10.5
13 polymyositis 10.4
14 lymphoma 10.4
15 osteomalacia 10.4
16 thyroiditis 10.4
17 membranous nephropathy 10.4
18 glomerulonephritis 10.4
19 purpura 10.4
20 hashimoto thyroiditis 10.3
21 hepatitis c virus 10.3
22 pulmonary hypertension 10.3
23 pneumonia 10.3
24 connective tissue disease 10.3
25 liver disease 10.3
26 multiple sclerosis 10.2
27 pernicious anemia 10.2
28 temporal arteritis 10.2
29 thrombocytopenic purpura, autoimmune 10.2
30 myeloma, multiple 10.2
31 alcoholic liver cirrhosis 10.2
32 bacteriuria 10.2
33 pancreatitis 10.2
34 lichen planus 10.2
35 biliary cirrhosis, primary, 1 10.2
36 hepatocellular carcinoma 10.2
37 myasthenia gravis 10.2
38 infantile liver failure syndrome 1 10.2
39 thrombocytopenia 10.2
40 inflammatory bowel disease 10.2
41 graves' disease 10.2
42 interstitial lung disease 10.2
43 myopathy 10.2
44 lung disease 10.2
45 vasculitis 10.2
46 neuropathy 10.2
47 amyloidosis 10.2
48 myasthenia gravis congenital 10.2
49 lichen sclerosus et atrophicus 10.2
50 helicobacter pylori infection 10.2

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:

60 33 (show all 35)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 cirrhosis 60 33 hallmark (90%) Very frequent (99-80%) HP:0001394
2 biliary cirrhosis 60 33 hallmark (90%) Very frequent (99-80%) HP:0002613
3 hyperpigmentation of the skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0000953
4 conjugated hyperbilirubinemia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002908
5 dermatographic urticaria 60 33 hallmark (90%) Very frequent (99-80%) HP:0011971
6 portal hypertension 60 33 frequent (33%) Frequent (79-30%) HP:0001409
7 pruritus 60 33 frequent (33%) Frequent (79-30%) HP:0000989
8 hepatic fibrosis 60 33 frequent (33%) Frequent (79-30%) HP:0001395
9 jaundice 60 33 frequent (33%) Frequent (79-30%) HP:0000952
10 hepatic failure 60 33 frequent (33%) Frequent (79-30%) HP:0001399
11 abnormality of the thyroid gland 60 33 frequent (33%) Frequent (79-30%) HP:0000820
12 orthostatic hypotension 60 33 frequent (33%) Frequent (79-30%) HP:0001278
13 hepatocellular carcinoma 60 33 frequent (33%) Frequent (79-30%) HP:0001402
14 antinuclear antibody positivity 60 33 frequent (33%) Frequent (79-30%) HP:0003493
15 elevated alkaline phosphatase 60 33 frequent (33%) Frequent (79-30%) HP:0003155
16 abnormality of the intrahepatic bile duct 60 33 frequent (33%) Frequent (79-30%) HP:0011040
17 onychomycosis 60 33 frequent (33%) Frequent (79-30%) HP:0012203
18 increased circulating igm level 33 frequent (33%) HP:0003496
19 abnormal circulating lipid concentration 33 frequent (33%) HP:0003119
20 fatigue 60 33 occasional (7.5%) Occasional (29-5%) HP:0012378
21 osteoporosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000939
22 ascites 60 33 occasional (7.5%) Occasional (29-5%) HP:0001541
23 hepatitis 60 33 occasional (7.5%) Occasional (29-5%) HP:0012115
24 celiac disease 60 33 occasional (7.5%) Occasional (29-5%) HP:0002608
25 abdominal distention 60 33 occasional (7.5%) Occasional (29-5%) HP:0003270
26 excessive daytime somnolence 60 33 occasional (7.5%) Occasional (29-5%) HP:0001262
27 hypoalbuminemia 60 33 occasional (7.5%) Occasional (29-5%) HP:0003073
28 increased circulating iga level 33 occasional (7.5%) HP:0003261
29 gastrointestinal inflammation 60 33 very rare (1%) Very rare (<4-1%) HP:0004386
30 sleep disturbance 60 Occasional (29-5%)
31 autoimmunity 60 Frequent (79-30%)
32 abnormality of lipid metabolism 60 Frequent (79-30%)
33 recurrent fungal infections 60 Frequent (79-30%)
34 increased igm level 60 Frequent (79-30%)
35 increased iga level 60 Occasional (29-5%)

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

27
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased vaccinia virus (VACV) infection GR00249-S 9.58 ABCA1 BCL2 CTLA4 IL12A IL12RB1 IL12RB2

MGI Mouse Phenotypes related to Primary Biliary Cholangitis:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.07 ABCA1 BCL2 CTLA4 IL12A IL12RB1 IL12RB2
2 endocrine/exocrine gland MP:0005379 9.92 ABCA1 BCL2 CTLA4 IL12RB2 NTN1 PIN1
3 digestive/alimentary MP:0005381 9.91 ABCA1 BCL2 CTLA4 IL12RB2 PTPN22 SPIB
4 homeostasis/metabolism MP:0005376 9.9 ABCA1 BCL2 CTLA4 IL12A IL12RB1 IL12RB2
5 immune system MP:0005387 9.77 ABCA1 BCL2 CTLA4 IL12A IL12RB1 IL12RB2
6 renal/urinary system MP:0005367 9.1 ABCA1 BCL2 IL12RB2 POU2AF1 PTPN22 TRAF3IP2

Drugs & Therapeutics for Primary Biliary Cholangitis

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

(show top 50) (show all 141)
# 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 Liver Extracts Phase 4,Phase 3,Phase 2,Not Applicable
6 Gastrointestinal Agents Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1,Not Applicable
7 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Phase 1
8 Cholagogues and Choleretics Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
9 Immunologic Factors Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
10 Cathartics Phase 4,Phase 3,Phase 2,Early Phase 1
11 Laxatives Phase 4,Phase 3,Phase 2,Early Phase 1
12 Immunosuppressive Agents Phase 4,Phase 3,Not Applicable
13
Fenofibrate Approved Phase 3,Phase 1,Phase 2 49562-28-9 3339
14
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
15
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
16
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
17
Molybdenum Approved Phase 3 7439-98-7 185498
18
Copper Approved, Investigational Phase 3 7440-50-8 27099
19
Atorvastatin Approved Phase 3 134523-00-5 60823
20
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
21
Colchicine Approved Phase 3 64-86-8 2833 6167
22
leucovorin Approved Phase 3 58-05-9 143 6006
23
Budesonide Approved Phase 3,Phase 1,Not Applicable 51333-22-3 5281004 63006
24
Metformin Approved Phase 2, Phase 3 657-24-9 14219 4091
25
Metronidazole Approved Phase 2, Phase 3 443-48-1 4173
26
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
27
Tauroursodeoxycholic acid Experimental, Investigational Phase 3 14605-22-2 12443252
28
Taurochenodeoxycholic acid Experimental Phase 3 516-35-8 387316
29
Tetrathiomolybdate Investigational Phase 3 16330-92-0
30 Antimetabolites Phase 3,Phase 1,Phase 2
31 Lipid Regulating Agents Phase 3,Phase 1,Phase 2
32 Hypolipidemic Agents Phase 3,Phase 1,Phase 2
33 Anti-HIV Agents Phase 2, Phase 3,Not Applicable
34 Lamivudine, zidovudine drug combination Phase 2, Phase 3
35 Anti-Retroviral Agents Phase 2, Phase 3,Not Applicable
36 Antiviral Agents Phase 2, Phase 3,Phase 3,Not Applicable
37 Reverse Transcriptase Inhibitors Phase 2, Phase 3,Not Applicable
38 Anti-Infective Agents Phase 2, Phase 3,Phase 3,Phase 1,Not Applicable
39 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3,Phase 3,Not Applicable
40 Angiogenesis Modulating Agents Phase 3
41 Nutrients Phase 3,Not Applicable
42 Chelating Agents Phase 3
43 Trace Elements Phase 3,Not Applicable
44 Micronutrients Phase 3,Not Applicable
45 Angiogenesis Inhibitors Phase 3
46 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 3
47 Anticholesteremic Agents Phase 3,Phase 1
48 Antimitotic Agents Phase 3
49 Antimetabolites, Antineoplastic Phase 3
50 Dermatologic Agents Phase 3,Phase 2

Interventional clinical trials:

(show top 50) (show all 118)
# Name Status NCT ID Phase Drugs
1 Effectiveness of S-adenosyl-L-methionine in Patients With Primary Biliary Cirrhosis Completed NCT02557360 Phase 4
2 Study of Abatacept (Orencia) to Treat Primary Biliary Cirrhosis Completed NCT02078882 Phase 4
3 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)
4 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
5 Phase 4 Study of Obeticholic Acid Evaluating Clinical Outcomes in Patients With Primary Biliary Cholangitis Recruiting NCT02308111 Phase 4 Obeticholic Acid (OCA);Placebo
6 Study of OCA Evaluating Pharmacokinetics and Safety in Patients With PBC and Hepatic Impairment Recruiting NCT03633227 Phase 4 Obeticholic Acid (OCA);Placebo
7 Fenofibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Unknown status NCT02823353 Phase 3 Fenofibrate;UDCA
8 Fuzhenghuayu in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Unknown status NCT02916290 Phase 3 Fuzhenghuayu;UDCA
9 Fenofibrate for Patients With Primary Biliary Cirrhosis Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02823366 Phase 3 Fenofibrate;UDCA
10 Fuzhenghuayu for Patients With PBC Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02916641 Phase 3 Fuzhenghuayu;UDCA
11 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
12 Effectiveness of Ursodeoxycholic Acid Use in the Prevention of Gallstone Formation After Sleeve Gastrectomy Unknown status NCT02721862 Phase 3 Ursodeoxycholic Acid;Placebo
13 Study of Combivir for Patients With Primary Biliary Cirrhosis Completed NCT00490620 Phase 2, Phase 3 Combination antiviral therapy;Placebo
14 Phase 3 Study of Obeticholic Acid in Patients With Primary Biliary Cirrhosis Completed NCT01473524 Phase 3 Obeticholic Acid (OCA);Placebo
15 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
16 Study Comparing Tetrathiomolybdate vs Standard Treatment in Primary Biliary Cirrhosis Completed NCT00805805 Phase 3 Tetrathiomolybdate
17 Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis Completed NCT00844402 Phase 3 Atorvastatin
18 Low-Dose Oral Methotrexate Versus Colchicine for Primary Biliary Cirrhosis Completed NCT00004748 Phase 3 colchicine;methotrexate;ursodiol
19 Phase 3 Study of Bezafibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Completed NCT01654731 Phase 3 Bezafibrate;placebo
20 Ursodiol-Methotrexate for Primary Biliary Cirrhosis Completed NCT00006168 Phase 3 Methotrexate
21 Phase III Randomized Study of Ursodiol With Vs Without Methotrexate for Primary Biliary Cirrhosis Completed NCT00004784 Phase 3 methotrexate;ursodiol
22 Efficacy and Safety Study of TUDCA Compare UDCA to Treatment Chronic Cholestatic Liver Disease-PBC Completed NCT01829698 Phase 3 tauroursodeoxycholic;ursodeoxycholic acid
23 ENHANCE: Seladelpar in Subjects With Primary Biliary Cholangitis (PBC) and an Inadequate Response to or an Intolerance to Ursodeoxycholic Acid (UDCA) Recruiting NCT03602560 Phase 3 seladelpar 5-10 mg;seladelpar 10 mg;Placebo
24 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
25 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)
26 Metformin in Intrahepatic Cholestasis of Pregnancy Not yet recruiting NCT03056274 Phase 2, Phase 3 Metformin;Ursodeoxycholic Acid
27 Ursodeoxycholic Acid Plus Budesonide Versus Ursodeoxycholic Acid Alone in Primary Biliary Cirrhosis (PBC) Terminated NCT00746486 Phase 3 budesonide;budesonide placebo
28 Umbilical Cord Mesenchymal Stem Cells for Patients With Primary Biliary Cirrhosis Unknown status NCT01662973 Phase 1, Phase 2
29 Pilot Study of FFP104 Dose Escalation in PBC Subjects Unknown status NCT02193360 Phase 1, Phase 2 FFP104
30 Efficacy and Safety of Fenofibrate Combined With UDCA in PBC Patients With an Incomplete Biochemical Response to UDCA Unknown status NCT02965911 Phase 1, Phase 2 Fenofibrate;UDCA
31 Study of Rituximab in Patients With Steroid-Refractory Chronic Graft-Versus-Host Disease Unknown status NCT00472225 Phase 2 Rituximab
32 Study to Evaluate the Efficacy and Safety of Elafibranor in Patients With Primary Biliary Cholangitis (PBC) and Inadequate Response to Ursodeoxycholic Acid Completed NCT03124108 Phase 2 Elafibranor 80 mg;Elafibranor 120 mg;Placebo
33 A Multi-part, Double Blind Study to Assess Safety, Tolerability and Efficacy of Tropifexor (LJN452) in PBC Patients Completed NCT02516605 Phase 2 Part 1: LJN452;Part 1: Placebo;Part 2: LJN452 Dose level 1;Part 2: Placebo;Part 2: LJN452 Dose level 2
34 Initial Study of Rituximab to Treat Primary Biliary Cirrhosis Completed NCT00364819 Phase 1, Phase 2 rituximab
35 Moexipril for Primary Biliary Cirrhosis Completed NCT00588302 Phase 2 Moexipril
36 Use of Fenofibrate for Primary Biliary Cirrhosis Completed NCT00575042 Phase 2 Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle)
37 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
38 Phase 2b Study of NGM282 Extended Treatment in Patients With Primary Biliary Cirrhosis Completed NCT02135536 Phase 2
39 Pentoxifylline for Primary Biliary Cirrhosis Completed NCT01249092 Phase 2 Pentoxifylline
40 Phase 2 Study of NGM282 in Patients With Primary Biliary Cirrhosis Completed NCT02026401 Phase 2
41 B-Cell Depleting Therapy (Rituximab) as a Treatment for Fatigue in Primary Biliary Cirrhosis Completed NCT02376335 Phase 2
42 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
43 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)
44 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
45 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
46 Study of INT-747 as Monotherapy in Patients With PBC Completed NCT00570765 Phase 2 Placebo;INT-747;INT-747
47 Treatment of Cholestatic Pruritus With Sertraline Completed NCT00058903 Phase 2 sertraline
48 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
49 A Study of Baricitinib (LY3009104) in Participants With Primary Biliary Cholangitis Who do Not Respond or Cannot Take UDCA Recruiting NCT03742973 Phase 2 Baricitinib;Placebo
50 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

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:

42
Bone, T Cells, Liver, Skin, Testes, Thyroid, B Cells

Publications for Primary Biliary Cholangitis

Articles related to Primary Biliary Cholangitis:

(show top 50) (show all 3585)
# 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
Treatment response in primary biliary cholangitis: The role of autoimmune hepatitis features. ( 31036497 )
2019
3
Autoimmune liver disease (primary biliary cholangitis/autoimmune hepatitis-overlap) associated with sarcoidosis (clinical cases and literature review). ( 31090378 )
2019
4
Increased Cthrc1 expression in primary biliary cholangitis. ( 31102333 )
2019
5
The extrahepatic events of Asian patients with primary biliary cholangitis: A 30-year cohort study. ( 31110209 )
2019
6
Hepatology Elsewhere. ( 30672600 )
2019
7
Baveno-VI-Guided Prediction of Esogastric Varices in Primary Biliary Cholangitis. ( 30676377 )
2019
8
Editorial: liver transplantation for primary biliary cholangitis-the need for timely and more effective treatments. Authors' reply. ( 30689255 )
2019
9
Editorial: liver transplantation for primary biliary cholangitis-the need for timely and more effective treatments. ( 30689256 )
2019
10
CD4+ T cells from patients with primary biliary cholangitis show T cell activation and differentially expressed T-cell receptor repertoires. ( 30690835 )
2019
11
Histologically proven AMA positive primary biliary cholangitis but normal serum alkaline phosphatase: Is alkaline phosphatase truly a surrogate marker? ( 30709684 )
2019
12
TCRβ repertoire of memory T cell reveals potential role for Escherichia coli in the pathogenesis of primary biliary cholangitis. ( 30721553 )
2019
13
Primary biliary cholangitis patients exhibit MRI changes in structure and function of interoceptive brain regions. ( 30735529 )
2019
14
Autotaxin, bile acid profile and effect of ileal bile acid transporter inhibition in primary biliary cholangitis patients with pruritus. ( 30735608 )
2019
15
Bezafibrate improves GLOBE and UK-PBC scores and long-term outcomes in patients with primary biliary cholangitis. ( 30737815 )
2019
16
Liver assessment using Gd-EOB-DTPA-enhanced magnetic resonance imaging in primary biliary cholangitis patients. ( 30798469 )
2019
17
Obeticholic acid in primary biliary cholangitis: where we stand. ( 30844895 )
2019
18
Mechanisms and Treatments of Pruritus in Primary Biliary Cholangitis. ( 30849783 )
2019
19
Genome-wide Association Studies of Specific Antinuclear Autoantibody Subphenotypes in Primary Biliary Cholangitis. ( 30854688 )
2019
20
Burden of Primary Biliary Cholangitis Among Inpatient Population in the United States. ( 30859148 )
2019
21
A Randomized, Controlled, Phase 2 Study of Maralixibat in the Treatment of Itching Associated With Primary Biliary Cholangitis. ( 30859149 )
2019
22
Recurrence of Primary Biliary Cholangitis After Liver Transplantation: Is Tacrolimus Really Worse Than Other Drugs? ( 30880019 )
2019
23
Immunological abnormalities in patients with primary biliary cholangitis. ( 30890652 )
2019
24
Comprehensive Analysis of Serum and Fecal Bile Acid Profiles and Interaction with Gut Microbiota in Primary Biliary Cholangitis. ( 30900136 )
2019
25
Pruritus Secondary to Primary Biliary Cholangitis: A Review of the Pathophysiology and Management with Phototherapy. ( 30920648 )
2019
26
Long-term outcomes with obeticholic acid in primary biliary cholangitis: reassuring, but still an itch we need to scratch. ( 30922872 )
2019
27
Long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis: 3-year results of an international open-label extension study. ( 30922873 )
2019
28
Lights and Shadows on Fibrates as Second-Line Therapy of Primary Biliary Cholangitis. ( 30926340 )
2019
29
Current and promising therapy for primary biliary cholangitis. ( 30931642 )
2019
30
Primary biliary cholangitis with contemporary presence of anti-mithocondrial and anti-rods and rings autoantibodies: literature first case. ( 30949324 )
2019
31
Ursodeoxycholic Acid Therapy and Liver Transplant-free Survival in Patients with Primary Biliary Cholangitis. ( 30980847 )
2019
32
The Prevalence of Anti-Hexokinase-1 and Anti-Kelch-Like 12 Peptide Antibodies in Patients With Primary Biliary Cholangitis Is Similar in Europe and North America: A Large International, Multi-Center Study. ( 31001269 )
2019
33
Proteomics in Primary Biliary Cholangitis. ( 31016654 )
2019
34
Identification of clinical risk factors for histological progression of primary biliary cholangitis. ( 31021038 )
2019
35
Therapeutic trials of biologics in primary biliary cholangitis: An open label study of abatacept and review of the literature. ( 31027870 )
2019
36
Novel biomarkers for Primary Biliary Cholangitis to improve diagnosis and understand underlying regulatory mechanisms. ( 31033124 )
2019
37
Reply to: "Recurrence of primary biliary cholangitis after liver transplantation: Is Tacrolimus really worse than other drugs?" and "Goodbye for good: Stepping away from recurrence". ( 31039341 )
2019
38
A Practical Review of Primary Biliary Cholangitis for the Gastroenterologist. ( 31061656 )
2019
39
Biochemical and Plasma Lipids Responses to Pemafibrate in Patients with Primary Biliary Cholangitis. ( 31077509 )
2019
40
Alteration of liver-infiltrated and peripheral blood double-negative T-cells in primary biliary cholangitis. ( 31087812 )
2019
41
Precision medicine in primary biliary cholangitis. ( 31099953 )
2019
42
Factors Associated With Recurrence of Primary Biliary Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival. ( 30296431 )
2019
43
Analysis of predictive response scores to treatment with ursodeoxycholic acid in patients with primary biliary cholangitis. ( 30297252 )
2019
44
Non-Invasive Prediction of High-Risk Varices in Patients with Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. ( 30315285 )
2019
45
Risk stratification and treatment of primary biliary cholangitis. ( 30338693 )
2019
46
Diagnosis and Management of Primary Biliary Cholangitis. ( 30429590 )
2019
47
Coronary flow reserve in patients with primary biliary cholangitis. ( 30448459 )
2019
48
NFKB1 and MANBA Confer Disease Susceptibility to Primary Biliary Cholangitis via Independent Putative Primary Functional Variants. ( 30528300 )
2019
49
Metabolic Signature of Primary Biliary Cholangitis and Its Comparison with Celiac Disease. ( 30539636 )
2019
50
Coronary flow reserve is an innovative tool for the early detection of cardiovascular dysfunction in primary biliary cholangitis patients. ( 30583996 )
2019

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:

(show all 14)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.49 BCL2 CTLA4 IL12A IL12RB1 IL12RB2 IRF5
2
Show member pathways
13.07 BCL2 IL12A IL12RB1 IL12RB2 PRKCB PTPN22
3
Show member pathways
12.91 BCL2 IL12A IL12RB1 IL12RB2 IRF5 PIN1
4 12.49 BCL2 IL12A IL12RB1 IL12RB2 PRKCB
5
Show member pathways
12.33 BCL2 IL12A IL12RB1 IL12RB2
6
Show member pathways
12.1 BCL2 IL12A IL12RB1 IL12RB2
7 11.87 CTLA4 IRF5 PTPN22 SPIB TNFSF15
8
Show member pathways
11.83 IL12A IL12RB1 IL12RB2
9
Show member pathways
11.71 IL12A IL12RB1 IL12RB2
10 11.66 BCL2 IRF5 TNFSF15
11 11.63 IL12A IL12RB1 IL12RB2
12 11.4 IL12A IL12RB1 IL12RB2
13 10.55 ABCA1 BCL2
14
Show member pathways
10.26 IL12A IL12RB1 IL12RB2

GO Terms for Primary Biliary Cholangitis

Cellular components related to Primary Biliary Cholangitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 8.92 ABCA1 CTLA4 IL12RB1 IL12RB2

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

# Name GO ID Score Top Affiliating Genes
1 B cell receptor signaling pathway GO:0050853 9.61 BCL2 CTLA4 PRKCB
2 positive regulation of defense response to virus by host GO:0002230 9.48 IL12RB1 TRAF3IP2
3 positive regulation of T cell mediated cytotoxicity GO:0001916 9.46 IL12A IL12RB1
4 humoral immune response GO:0006959 9.43 BCL2 POU2AF1 TRAF3IP2
5 interferon-gamma production GO:0032609 9.4 IL12A IL12RB2
6 interleukin-35-mediated signaling pathway GO:0070757 9.37 IL12A IL12RB2
7 interleukin-12-mediated signaling pathway GO:0035722 9.33 IL12A IL12RB1 IL12RB2
8 response to UV-B GO:0010224 9.32 BCL2 IL12A
9 positive regulation of interferon-gamma production GO:0032729 9.13 IL12A IL12RB1 IL12RB2
10 cytokine-mediated signaling pathway GO:0019221 9.02 BCL2 IL12A IL12RB1 IL12RB2 IRF5

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 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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