PBC
MCID: PRM236
MIFTS: 57

Primary Biliary Cholangitis (PBC)

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 53 59
Primary Biliary Cirrhosis 53 59 72
Familial Primary Biliary Cirrhosis 53 72
Pbc 53 59
Hanot's Cirrhosis 72
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


External Ids:

ICD10 via Orphanet 34 K74.3
UMLS via Orphanet 73 C0008312 C0859942
Orphanet 59 ORPHA186
UMLS 72 C0008312 C0859942 C2931878

Summaries for Primary Biliary Cholangitis

NIH Rare Diseases : 53 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 biliary cirrhosis, primary, 1. An important gene associated with Primary Biliary Cholangitis is TNFSF15 (TNF Superfamily Member 15), and among its related pathways/superpathways are PEDF Induced Signaling and TGF-Beta Pathway. The drugs Abatacept and Sorafenib have been mentioned in the context of this disorder. Affiliated tissues include liver, t cells and bone, and related phenotypes are cirrhosis and biliary cirrhosis

Wikipedia : 75 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 576)
# Related Disease Score Top Affiliating Genes
1 primary biliary cirrhosis 11.8
2 biliary cirrhosis, primary, 1 11.8
3 reynolds syndrome 11.8
4 biliary cirrhosis, primary, 2 11.3
5 biliary cirrhosis, primary, 3 11.3
6 biliary cirrhosis, primary, 4 11.3
7 biliary cirrhosis, primary, 5 11.3
8 cholangitis 11.3
9 nodular regenerative hyperplasia 11.2
10 cholestasis 10.8
11 autoimmune disease 10.7
12 liver disease 10.7
13 autoimmune hepatitis 10.7
14 cholangitis, primary sclerosing 10.6
15 sclerosing cholangitis 10.6
16 liver cirrhosis 10.5
17 glomerulonephritis 10.5
18 hepatitis c 10.5
19 lupus erythematosus 10.5
20 hashimoto thyroiditis 10.5
21 vasculitis 10.5
22 neuropathy 10.5
23 47,xyy 10.5
24 autonomic dysfunction 10.5
25 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.5
26 portal hypertension 10.5
27 colitis 10.5
28 hepatitis c virus 10.4
29 membranous nephropathy 10.4
30 keratoconjunctivitis sicca 10.4
31 thrombocytopenia 10.4
32 type 1 diabetes mellitus 13 10.4 CTLA4 ABCA1
33 pulmonary hypertension 10.4
34 hepatitis a 10.4
35 gastritis 10.4
36 keratoconjunctivitis 10.4
37 pernicious anemia 10.4
38 exanthem 10.4
39 lymphoma 10.4
40 bacteriuria 10.4
41 temporal arteritis 10.4
42 myasthenia gravis 10.4
43 myeloma, multiple 10.4
44 esophagitis, eosinophilic, 1 10.4 CCL26 CCL11
45 cholelithiasis 10.3
46 steatorrhea 10.3
47 extrahepatic cholestasis 10.3
48 alcoholic liver cirrhosis 10.3
49 chronic cholangitis 10.3
50 gallbladder disease 1 10.3

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 35)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 cirrhosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001394
2 biliary cirrhosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0002613
3 hyperpigmentation of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0000953
4 conjugated hyperbilirubinemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002908
5 dermatographic urticaria 59 32 hallmark (90%) Very frequent (99-80%) HP:0011971
6 portal hypertension 59 32 frequent (33%) Frequent (79-30%) HP:0001409
7 pruritus 59 32 frequent (33%) Frequent (79-30%) HP:0000989
8 hepatic fibrosis 59 32 frequent (33%) Frequent (79-30%) HP:0001395
9 jaundice 59 32 frequent (33%) Frequent (79-30%) HP:0000952
10 hepatic failure 59 32 frequent (33%) Frequent (79-30%) HP:0001399
11 abnormality of the thyroid gland 59 32 frequent (33%) Frequent (79-30%) HP:0000820
12 orthostatic hypotension 59 32 frequent (33%) Frequent (79-30%) HP:0001278
13 hepatocellular carcinoma 59 32 frequent (33%) Frequent (79-30%) HP:0001402
14 antinuclear antibody positivity 59 32 frequent (33%) Frequent (79-30%) HP:0003493
15 elevated alkaline phosphatase 59 32 frequent (33%) Frequent (79-30%) HP:0003155
16 abnormality of the intrahepatic bile duct 59 32 frequent (33%) Frequent (79-30%) HP:0011040
17 onychomycosis 59 32 frequent (33%) Frequent (79-30%) HP:0012203
18 increased circulating igm level 32 frequent (33%) HP:0003496
19 abnormal circulating lipid concentration 32 frequent (33%) HP:0003119
20 fatigue 59 32 occasional (7.5%) Occasional (29-5%) HP:0012378
21 osteoporosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000939
22 ascites 59 32 occasional (7.5%) Occasional (29-5%) HP:0001541
23 hepatitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012115
24 celiac disease 59 32 occasional (7.5%) Occasional (29-5%) HP:0002608
25 abdominal distention 59 32 occasional (7.5%) Occasional (29-5%) HP:0003270
26 excessive daytime somnolence 59 32 occasional (7.5%) Occasional (29-5%) HP:0001262
27 hypoalbuminemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003073
28 increased circulating iga level 32 occasional (7.5%) HP:0003261
29 gastrointestinal inflammation 59 32 very rare (1%) Very rare (<4-1%) HP:0004386
30 sleep disturbance 59 Occasional (29-5%)
31 autoimmunity 59 Frequent (79-30%)
32 abnormality of lipid metabolism 59 Frequent (79-30%)
33 recurrent fungal infections 59 Frequent (79-30%)
34 increased igm level 59 Frequent (79-30%)
35 increased iga level 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 9.77 ABCA1 BCL2 CCL11 CTLA4 CXCL12 DNMT1
2 digestive/alimentary MP:0005381 9.7 ABCA1 BCL2 CTLA4 DNMT1 IL12RB2 KEAP1
3 immune system MP:0005387 9.44 ABCA1 BCL2 CCL11 CTLA4 CXCL12 DNMT1

Drugs & Therapeutics for Primary Biliary Cholangitis

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

(show top 50) (show all 150)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Abatacept Approved Phase 4 332348-12-6 10237
2
Sorafenib Approved, Investigational Phase 4 284461-73-0 216239 406563
3 Immunologic Factors Phase 4
4 Antirheumatic Agents Phase 4
5 Immunosuppressive Agents Phase 4
6 Liver Extracts Phase 4
7 Protein Kinase Inhibitors Phase 4
8
Fenofibrate Approved Phase 3 49562-28-9 3339
9
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
10
leucovorin Approved Phase 3 58-05-9 143 6006
11
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
12
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
13
Colchicine Approved Phase 3 64-86-8 6167 2833
14
Molybdenum Approved Phase 3 7439-98-7 185498
15
Copper Approved, Investigational Phase 3 7440-50-8 27099
16
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
17
Atorvastatin Approved Phase 3 134523-00-5 60823
18
Metformin Approved Phase 2, Phase 3 657-24-9 4091 14219
19
Metronidazole Approved Phase 2, Phase 3 443-48-1 4173
20
Ursodeoxycholic acid Approved, Investigational Phase 3 128-13-2 31401
21
Budesonide Approved Phase 3 51333-22-3 63006 5281004
22
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
23
Taurochenodeoxycholic acid Experimental Phase 3 516-35-8 387316
24
Tauroursodeoxycholic acid Experimental, Investigational Phase 3 14605-22-2 12443252
25
Tetrathiomolybdate Investigational Phase 3 16330-92-0
26 Anti-Infective Agents Phase 2, Phase 3
27 Folic Acid Antagonists Phase 3
28 Folate Phase 3
29 Vitamin B Complex Phase 3
30 Vitamin B9 Phase 3
31 Dermatologic Agents Phase 3
32 Nucleic Acid Synthesis Inhibitors Phase 2, Phase 3
33 Antimetabolites, Antineoplastic Phase 3
34 Reverse Transcriptase Inhibitors Phase 2, Phase 3
35 Anti-HIV Agents Phase 2, Phase 3
36 Lamivudine, zidovudine drug combination Phase 2, Phase 3
37 Tubulin Modulators Phase 3
38 Antimitotic Agents Phase 3
39 Angiogenesis Inhibitors Phase 3
40 Angiogenesis Modulating Agents Phase 3
41 Chelating Agents Phase 3
42 Lipid Regulating Agents Phase 3
43 Hypolipidemic Agents Phase 3
44 Antimetabolites Phase 3
45 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 3
46 Anticholesteremic Agents Phase 3
47 carnitine Phase 3
48 Hypoglycemic Agents Phase 2, Phase 3
49 Gastrointestinal Agents Phase 3
50 Cholagogues and Choleretics Phase 3

Interventional clinical trials:

(show top 50) (show all 128)
# Name Status NCT ID Phase Drugs
1 Double-blind, Double-dummy, Randomised, Crossover, Multicentre Phase IV Clinical Study Comparing the Effect of Ursofalk 500 mg Tablets od Versus Ursofalk 250 mg Capsules od on Liver Enzymes in the Treatment of Primary Biliary Cirrhosis Completed NCT01510860 Phase 4 UDCA (Ursodeoxycholic acid);UDCA (Ursodeoxycholic acid)
2 Abatacept For The Treatment Of Primary Biliary Cirrhosis With An Incomplete Biochemical Response To Ursodeoxycholic Acid Completed NCT02078882 Phase 4
3 Effectiveness of S-adenosyl-L-methionine in Patients With Primary Biliary Cirrhosis Completed NCT02557360 Phase 4
4 A Phase 4, Double-Blind, Randomized, Placebo-Controlled Study Evaluating the Pharmacokinetics and Safety of Obeticholic Acid in Patients With Primary Biliary Cholangitis and Moderate to Severe Hepatic Impairment Recruiting NCT03633227 Phase 4 Obeticholic Acid (OCA);Placebo
5 A Randomized Controlled 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
6 A Phase 4, Double-Blind, Randomized, Placebo-Controlled, Multicenter Study Evaluating the Effect of Obeticholic Acid on Clinical Outcomes in Patients With Primary Biliary Cholangitis Recruiting NCT02308111 Phase 4 Obeticholic Acid (OCA);Placebo
7 Mechanism of Sorafenib Resistance in Patients With Advanced Hepatocellular Carcinoma Recruiting NCT02733809 Phase 4 Sorafenib
8 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
9 Fenofibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis: a Randomized Control Study Unknown status NCT02823353 Phase 3 Fenofibrate;UDCA
10 Fenofibrate for Patients With Primary Biliary Cirrhosis Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02823366 Phase 3 Fenofibrate;UDCA
11 Fuzhenghuayu in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Unknown status NCT02916290 Phase 3 Fuzhenghuayu;UDCA
12 Fuzhenghuayu for Patients With PBC Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02916641 Phase 3 Fuzhenghuayu;UDCA
13 Effectiveness of Ursodeoxycholic Acid Use in the Prevention of Gallstone Formation After Sleeve Gastrectomy: A Prospective Randomized Multicenter Placebo-Controlled Trial Unknown status NCT02721862 Phase 3 Ursodeoxycholic Acid;Placebo
14 A Phase 3, Double Blind, Placebo Controlled Trial and Long Term Safety Extension of Obeticholic Acid in Patients With Primary Biliary Cirrhosis Completed NCT01473524 Phase 3 Obeticholic Acid (OCA);Placebo
15 Phase III Randomized Study of Ursodiol With Vs Without Methotrexate for Primary Biliary Cirrhosis Completed NCT00004784 Phase 3 methotrexate;ursodiol
16 A Randomized, Double-blinded, Double-dummy, Parallel-controlled and Multicenter Clinical Trial to Investigate Safety and Efficacy of Tauroursodeoxycholic Acid Capsules in Treatment of Adult Primary Biliary Cirrhosis Completed NCT01857284 Phase 3 Tauroursodeoxycholic Acid Capsules;Ursodeoxycholic Acid Capsules
17 Randomized Controlled Pilot Study of Combivir for Patients With Primary Biliary Cirrhosis Completed NCT00490620 Phase 2, Phase 3 Combination antiviral therapy;Placebo
18 Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis Completed NCT00844402 Phase 3 Atorvastatin
19 Phase III Randomized, Double-Blind, Placebo-Controlled Study of Low-Dose Oral Methotrexate Versus Colchicine for Primary Biliary Cirrhosis Completed NCT00004748 Phase 3 colchicine;methotrexate;ursodiol
20 Evaluate the Efficacy And Safety Of TUDCA Compare UDCA In The Treatment Of Cholestatic Liver Disease-PBC by A Randomized,Double-Blind,Double Dummy,Parallel-Controlled,Multicenter Trial and The Consecutive Treatment By TUDCA Completed NCT01829698 Phase 3 tauroursodeoxycholic;ursodeoxycholic acid
21 Ursodiol-Methotrexate for Primary Biliary Cirrhosis Completed NCT00006168 Phase 3 Methotrexate
22 Phase III Trial of Tetrathiomolybdate (TM) in Primary Biliary Cirrhosis Completed NCT00805805 Phase 3 Tetrathiomolybdate
23 Multicenter, Randomized, Double-blind Placebo Controlled Trial of Bezafibrate for the Treatment of Primary Biliary Cirrhosis in Patients With Incomplete Response to Ursodesoxycholic Acid Therapy. Completed NCT01654731 Phase 3 Bezafibrate;placebo
24 A Phase 3 Study to Evaluate the Place of Budesonide in the Treatment of GVHD Completed NCT00128739 Phase 3 Budesonide
25 An Open Label Long-Term Study to Evaluate the Safety and Tolerability of 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
26 Efficacy and Security of Bezafibrate in Patients With Primary Biliary Cirrhosis Without Biochemical Response to Ursodeoxycholic Acid: A Randomized, Double-blind, Placebo-controlled Trial Recruiting NCT02937012 Phase 3 Bezafibrate;Ursodeoxycholic Acid;Placebo (for Bezafibrate)
27 A 52-week, Placebo-controlled, Randomized, Phase 3 Study to Evaluate the Safety and Efficacy of 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
28 Estudio clínico Fase III Para Evaluar la Eficacia terapéutica en Pacientes Mexicanos Con Dislipidemia Mediante el Uso vía Oral de L-Carnitina + Atorvastatina Comparado Con Atorvastatina Active, not recruiting NCT03696940 Phase 3 L-Carnitine 500Mg Oral Tablet + Atorvastatin 10 mg;Atorvastatin 10mg
29 METformin in Intrahepatic Cholestasis of Pregnancy (METRIC) Study Not yet recruiting NCT03056274 Phase 2, Phase 3 Metformin;Ursodeoxycholic Acid
30 Double-blind, Randomised, Placebo-controlled, Multi-centre Phase III Clinical Study Comparing the Combination of Ursodeoxycholic Acid Capsules Plus Budesonide Capsules to Ursodeoxycholic Acid Capsules Plus Placebo in the Treatment of Primary Biliary Cirrhosis Terminated NCT00746486 Phase 3 budesonide;budesonide placebo
31 A Randomized Controlled Clinical Trial on the Efficacy and Safety of Fenofibrate Combined With Ursodeoxycholic Acid in PBC Patients With an Incomplete Biochemical Response to UDCA Unknown status NCT02965911 Phase 1, Phase 2 Fenofibrate;UDCA
32 A Phase I/II, Open Label, Multicenter, Pilot Dose Escalation Study to Evaluate the Safety, Tolerability and Pharmacodynamics of FFP104 in Subjects Previously Diagnosed With Primary Biliary Cirrhosis (PBC) Unknown status NCT02193360 Phase 1, Phase 2 FFP104
33 Phase 1/2 Study of UC-MSC Treatment for Evaluation the Efficacy and Safety in Patients With Primary Biliary Cirrhosis Unknown status NCT01662973 Phase 1, Phase 2
34 Open-Labelled, Multicenter Phase II Study of Rituximab in Patients With Steroid-Refractory Chronic Graft-Versus-Host Disease Unknown status NCT00472225 Phase 2 Rituximab
35 A Multi-part, Randomized, Double-blind, Placebo-controlled Study to Assess the Safety, Tolerability and Efficacy of Tropifexor (LJN452) in Patients With Primary Biliary Cholangitis 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
36 A Phase 2, Randomized, Double Blind, Placebo Controlled, Parallel Group, Multiple Center Study to Evaluate the Safety, Tolerability, and Pharmacodynamic Activity of NGM282 in Combination With Ursodeoxycholic Acid (UDCA) Administered for 28 Days in Patients With Primary Biliary Cirrhosis Completed NCT02026401 Phase 2
37 A Phase 2, Multi-Center Study to Evaluate Three Doses of NGM282 Administered for 24 Weeks in Patients With Primary Biliary Cirrhosis (PBC) Who Have Completed Study 13-0103 Completed NCT02135536 Phase 2
38 A Multicenter, Randomized, Double-Blind, Placebo-Controlled, Phase 2 Study to Evaluate the Efficacy and Safety of Elafibranor at Doses of 80 mg and 120mg After 12 Weeks of Treatment in Patients With Primary Biliary Cholangitis and Inadequate Response to Ursodeoxycholic Acid Completed NCT03124108 Phase 2 Elafibranor 80 mg;Elafibranor 120 mg;Placebo
39 A Study of INT 747 (6α-ethyl Chenodeoxycholic Acid (6-ECDCA)) in Combination With Ursodeoxycholic Acid (URSO®, UDCA) in Patients With Primary Biliary Cirrhosis Completed NCT00550862 Phase 2 INT-747;Ursodeoxycholic Acid (URSO);Placebo
40 A Phase 2, Randomized, Double-blind, Placebo-controlled Study to Evaluate LUM001, an Apical Sodium-dependent Bile Acid Transporter Inhibitor (ASBTi) in Combination With Ursodeoxycholic Acid (UDCA) in Patients With Primary Biliary Cirrhosis Completed NCT01904058 Phase 2 LUM001;Placebo;Ursodeoxycholic Acid
41 Treatment of Cholestatic Pruritus With Sertraline Completed NCT00058903 Phase 2 sertraline
42 Pilot Study of Fenofibrate for Primary Biliary Cirrhosis Completed NCT00575042 Phase 2 Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle)
43 A Study of INT-747 (6-ECDCA) Monotherapy in Patients With Primary Biliary Cirrhosis Completed NCT00570765 Phase 2 Placebo;INT-747;INT-747
44 A Pilot Study of Pentoxifylline for the Treatment of Primary Biliary Cirrhosis Completed NCT01249092 Phase 2 Pentoxifylline
45 Open-Label Pilot Investigation of Moexipril for the Treatment of Primary Biliary Cirrhosis (PBC) Completed NCT00588302 Phase 2 Moexipril
46 Effects of Rituximab (Rituxan) on B Cell and AMA Response in Patients With Primary Biliary Cirrhosis Completed NCT00364819 Phase 1, Phase 2 rituximab
47 A Phase 2 Clinical Trial Investigating the Effects of Obeticholic Acid on Lipoprotein Metabolism in Subjects With Primary Biliary Cirrhosis Completed NCT01865812 Phase 2 obeticholic acid (OCA)
48 A Phase 2, Multi-center, Randomized, Double-blind, Placebo-controlled, Parallel-group Study Evaluating the Efficacy and Safety of Ustekinumab in Subjects With Primary Biliary Cirrhosis Who Had an Inadequate Response to Ursodeoxycholic Acid (UDCA) Completed NCT01389973 Phase 2 ustekinumab 90 mg;ustekinumab 45 mg;ustekinumab 180 mg;Placebo
49 A Randomised, Double-blind, Placebo-controlled Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Repeat Doses of GSK2330672 Administration in Patients With Primary Biliary Cirrhosis (PBC) and Symptoms of Pruritus Completed NCT01899703 Phase 2 GSK2330672;Placebo;Ursodeoxycholic acid
50 B-Cell Depleting Therapy (Rituximab) as a Treatment for Fatigue in Primary Biliary Cirrhosis Completed NCT02376335 Phase 2

Search NIH Clinical Center for Primary Biliary Cholangitis

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Obeticholic acid
Ursodeoxycholic acid

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, Testes, B Cells, Thyroid, Skin

Publications for Primary Biliary Cholangitis

Articles related to Primary Biliary Cholangitis:

(show top 50) (show all 8141)
# Title Authors PMID Year
1
EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. 38 6
28427765 2017
2
Red cell distribution width to platelet ratio for liver fibrosis: a systematic review and meta-analysis of diagnostic accuracy. 38
31389726 2019
3
UGT-dependent regioselective glucuronidation of ursodeoxycholic acid and obeticholic acid and selective transport of the consequent acyl glucuronides by OATP1B1 and 1B3. 38
31299240 2019
4
Liver biopsy in primary biliary cholangitis: is sinusoidal fibrosis the missing key? 38
31371396 2019
5
The Potential Effects of Diabetes Mellitus on Liver Fibrosis in Patients with Primary Biliary Cholangitis. 38
31420961 2019
6
Pharmacokinetics of Ursodeoxycholic Acid in Elderly Volunteers Compared With Younger Adults in a Korean Population. 38
30882914 2019
7
Efficacy and safety of 3-year denosumab therapy for osteoporosis in patients with autoimmune liver diseases. 38
31429969 2019
8
Variant and Specific Forms of Autoimmune Cholestatic Liver Diseases. 38
31165900 2019
9
Ursodeoxycholic acid accelerates bile acid enterohepatic circulation. 38
31077342 2019
10
Semisynthetic bile acids: a new therapeutic option for metabolic syndrome. 38
31254667 2019
11
Hepatic artery reconstruction in living donor liver transplantation: strategy of the extension of graft or recipient artery. 38
30929554 2019
12
Curcumin mitigates the epithelial-to-mesenchymal transition in biliary epithelial cells through upregulating CD109 expression. 38
31403228 2019
13
High occurrence of small intestinal bacterial overgrowth in primary biliary cholangitis. 38
31373141 2019
14
RANK/RANKL Acts as a Protective Factor by Targeting Cholangiocytes in Primary Biliary Cholangitis. 38
31377883 2019
15
A Brief Reflection on Continuous vs Binary Risk Indicators in Primary Biliary Cholangitis. 38
31425151 2019
16
Prediction of Transplant-Free Survival through Albumin-Bilirubin Score in Primary Biliary Cholangitis. 38
31430975 2019
17
Factors Associated With Progression and Outcomes of Early-stage Primary Biliary Cholangitis. 38
31419573 2019
18
Treatment of Fatigue in Primary Biliary Cholangitis: A Systematic Review and Meta-Analysis. 38
30632051 2019
19
Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis. 38
30980847 2019
20
Incidence, prevalence, and outcome of primary biliary cholangitis in a nationwide Swedish population-based cohort. 38
31395896 2019
21
Therapeutic trials of biologics in primary biliary cholangitis: An open label study of abatacept and review of the literature. 38
31027870 2019
22
Prevalence of autoimmune diseases and clinical significance of autoantibody profile: Data from National Institute of Hygiene in Rabat, Morocco. 38
30807792 2019
23
Polyphenols in the treatment of autoimmune diseases. 38
31059841 2019
24
Autoimmune Diseases and Gastric Cancer Risk: A Systematic Review and Meta-Analysis. 38
31048663 2019
25
Anti-centrosome antibodies: Prevalence and disease association in Chinese population. 38
31267615 2019
26
Fatigue in chronic hepatitis B patients is significant and associates with autonomic dysfunction. 38
31345232 2019
27
Reactive lymphoid hyperplasia of the liver: A case report. 38
31348258 2019
28
High Prevalence of Autoimmune Gastritis in Patients with Nonalcoholic Steatohepatitis. 38
31292380 2019
29
Ursodeoxycholic acid and cancer: From chemoprevention to chemotherapy. 38
31356908 2019
30
Autoantibodies in patients with interleukin 12 receptor beta 1 deficiency. 38
31111679 2019
31
CTHRC1 expression in primary biliary cholangitis. 38
31102333 2019
32
The gut microbial influence on cholestatic liver disease. 38
31125502 2019
33
Bile ductular reactions in the liver: similarities are only skin deep. 38
30883752 2019
34
Muscle performance and fatigue in compensated chronic liver disease. 38
31287334 2019
35
Human biodistribution, dosimetry, radiosynthesis and quality control of the bile acid PET tracer [N-methyl-11C]cholylsarcosine. 38
31330413 2019
36
Discovery of a Novel Selective Dual Peroxisome Proliferator-Activated Receptor α/δ Agonist for the Treatment of Primary Biliary Cirrhosis. 38
31312410 2019
37
Genome-wide Association Studies of Specific Antinuclear Autoantibody Subphenotypes in Primary Biliary Cholangitis. 38
30854688 2019
38
Prognosis of 732 ursodeoxycholic acid-treated patients with primary biliary cholangitis: A single center follow-up study from China. 38
30365184 2019
39
Treatment of Pruritus Secondary to Liver Disease. 38
31367993 2019
40
Guideline review: British Society of Gastroenterology/UK-PBC Primary Biliary Cholangitis treatment and management guidelines. 38
31281627 2019
41
Florid biliary duct lesions in an AMA -positive patient in absence of cholestatic liver biochemistry. 38
30975574 2019
42
Long-Standing Unresolved Primary Biliary Cholangitis Leading to Systemic Amyloid A Amyloidosis With Predominant Liver Involvement: A Death Case. 38
31365351 2019
43
Checkpoint inhibitor-induced liver injury: A novel form of liver disease emerging in the era of cancer immunotherapy. 38
31358424 2019
44
Pediatric-onset primary biliary cholangitis. 38
31155491 2019
45
Precision medicine in primary biliary cholangitis. 38
31099953 2019
46
Xanthomas and xanthelasmas in primary biliary cholangitis. 38
30470445 2019
47
Validation of Risk Scoring Systems in Ursodeoxycholic Acid-Treated Patients With Primary Biliary Cholangitis. 38
31241547 2019
48
A case of primary biliary cholangitis overlapping with type 2 autoimmune hepatitis. 38
31286423 2019
49
An ultra-performance liquid chromatography-tandem mass spectrometry method for the determination of obeticholic acid in rat plasma and its application in preclinical pharmacokinetic studies. 38
31129439 2019
50
Acute Adrenal Insufficiency Precipitated by the Discontinuation of a Betamethasone and Dextrochlorpheniramine Combination: The Diagnostic Utility of an Echocardiographic Assessment of Systemic Vascular Resistance. 38
30996179 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 24)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.51 TNFSF15 IL12RB2 IL12RB1 IL12A CXCL12 CCL26
2
Show member pathways
13.4 IL12RB2 IL12RB1 IL12A CXCL12 CCL26 CCL11
3
Show member pathways
13.22 IL12RB2 IL12RB1 IL12A CXCL12 CCL26 CCL11
4
Show member pathways
13.13 TNFSF15 IRF5 IL12RB2 IL12RB1 IL12A CCL11
5
Show member pathways
12.65 IL12RB2 IL12RB1 IL12A CXCL12 CTLA4 CCL11
6
Show member pathways
12.62 KEAP1 IL12RB2 IL12RB1 IL12A CXCL12 CCL26
7
Show member pathways
12.44 IL12RB2 IL12RB1 IL12A BCL2
8 12.36 KEAP1 IL12RB2 IL12RB1 IL12A CXCL12 BCL2
9 12.32 TNFSF15 SPIB IRF5 CXCL12 CTLA4
10
Show member pathways
12.1 CXCL12 CCL26 CCL11
11
Show member pathways
12.09 IL12RB2 IL12RB1 IL12A CXCL12 BCL2
12
Show member pathways
11.9 IL12RB2 IL12RB1 IL12A
13
Show member pathways
11.87 IL12A CXCL12 CCL26 CCL11 BCL2
14 11.82 IL12A CCL11 BCL2
15
Show member pathways
11.81 IL12RB2 IL12RB1 IL12A
16 11.76 TNFSF15 IRF5 BCL2
17 11.61 IL12RB2 IL12RB1 IL12A CXCL12
18 11.59 IL12RB2 IL12RB1 IL12A
19 11.52 IL12A CCL26 CCL11
20 10.95 DNMT1 BCL2
21
Show member pathways
10.9 IL12RB2 IL12RB1 IL12A
22 10.84 IL12A CXCL12 CCL26 CCL11 BCL2
23 10.69 BCL2 ABCA1
24 10.25 IL12A CXCL12 CCL26 CCL11 BCL2

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 9.02 IL12RB2 IL12RB1 CXCL12 CTLA4 ABCA1

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 chemotaxis GO:0006935 9.73 CXCL12 CCL26 CCL11
2 response to virus GO:0009615 9.7 IL12A CXCL12 CCL11
3 cellular calcium ion homeostasis GO:0006874 9.69 CXCL12 CCL11 BCL2
4 cellular response to interferon-gamma GO:0071346 9.67 IL12RB1 CCL26 CCL11
5 positive regulation of endothelial cell proliferation GO:0001938 9.58 CXCL12 CCL26 CCL11
6 regulation of calcium ion transport GO:0051924 9.57 CXCL12 BCL2
7 positive regulation of T cell mediated cytotoxicity GO:0001916 9.55 IL12RB1 IL12A
8 eosinophil chemotaxis GO:0048245 9.54 CCL26 CCL11
9 chemokine-mediated signaling pathway GO:0070098 9.54 CXCL12 CCL26 CCL11
10 interleukin-12-mediated signaling pathway GO:0035722 9.5 IL12RB2 IL12RB1 IL12A
11 response to UV-B GO:0010224 9.46 IL12A BCL2
12 interleukin-35-mediated signaling pathway GO:0070757 9.43 IL12RB2 IL12A
13 positive regulation of interferon-gamma production GO:0032729 9.43 IL12RB2 IL12RB1 IL12A
14 immune response GO:0006955 9.43 TNFSF15 IL12A CXCL12 CTLA4 CCL26 CCL11
15 response to radiation GO:0009314 9.33 CXCL12 CCL11 BCL2
16 interferon-gamma production GO:0032609 9.18 IL12RB2
17 cytokine-mediated signaling pathway GO:0019221 9.1 IRF5 IL12RB2 IL12RB1 IL12A CCL11 BCL2

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

# Name GO ID Score Top Affiliating Genes
1 chemokine activity GO:0008009 9.33 CXCL12 CCL26 CCL11
2 interleukin-12 receptor binding GO:0005143 9.26 IL12RB1 IL12A
3 cytokine activity GO:0005125 9.02 TNFSF15 IL12A CXCL12 CCL26 CCL11
4 CCR3 chemokine receptor binding GO:0031728 8.96 CCL26 CCL11

Sources for Primary Biliary Cholangitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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