PBC
MCID: PRM006
MIFTS: 62

Primary Biliary Cirrhosis (PBC)

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

Aliases & Classifications for Primary Biliary Cirrhosis

MalaCards integrated aliases for Primary Biliary Cirrhosis:

Name: Primary Biliary Cirrhosis 12 74 52 58 36 15 37 17 71 32
Primary Biliary Cholangitis 52 58
Biliary Liver Cirrhosis 12 17
Pbc 52 58
Chronic Nonsuppurative Destructive Cholangitis 12
Familial Primary Biliary Cirrhosis 52
Biliary Cirrhosis Primary 54
Liver Cirrhosis, Biliary 43
Cholestatic Cirrhosis 12
Biliary Cirrhosis 71
Hanot Syndrome 58

Characteristics:

Orphanet epidemiological data:

58
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: 58  
Rare hepatic diseases


Summaries for Primary Biliary Cirrhosis

NIH Rare Diseases : 52 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 Cirrhosis, also known as primary biliary cholangitis, is related to connective tissue disease and non-alcoholic fatty liver disease. An important gene associated with Primary Biliary Cirrhosis is DLAT (Dihydrolipoamide S-Acetyltransferase), and among its related pathways/superpathways are MicroRNAs in cancer and Parkinsons Disease Pathway. The drugs Abatacept and Sorafenib have been mentioned in the context of this disorder. Affiliated tissues include Umbilical Cord, liver and t cells, and related phenotypes are cirrhosis and biliary cirrhosis

Disease Ontology : 12 A liver cirrhosis characterized by chronic and slow progressive destruction of intrahepatic bile ducts.

KEGG : 36 Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic liver disease characterised by destruction of small intrahepatic bile ducts, eventually leading to fibrosis and potential cirrhosis. Patients are usually middle-aged women and presents in the fifth or sixth decade of life. The most common symptoms are pruritus. It is not clear what causes PBC. It may be relate to problems in the autoimmune mechanism. The increased risk of genetic predisposition and environmental factors (infectious or toxic agents) trigger the disease. Ursodeoxycholic acid is currently the only medicine approved for the treatment of the disease.

Wikipedia : 74 Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune... more...

Related Diseases for Primary Biliary Cirrhosis

Diseases in the Primary Biliary Cirrhosis family:

Biliary Cirrhosis, Primary, 1 Biliary Cirrhosis, Primary, 2
Biliary Cirrhosis, Primary, 3 Biliary Cirrhosis, Primary, 4
Biliary Cirrhosis, Primary, 5

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

(show top 50) (show all 640)
# Related Disease Score Top Affiliating Genes
1 connective tissue disease 30.8 MIR223 MIR17 MIR150 MIR122
2 non-alcoholic fatty liver disease 30.4 MIR451A MIR30D MIR17 MIR122
3 arteries, anomalies of 30.2 MIR296 MIR223 MIR17 MIR150 MIR122
4 systemic lupus erythematosus 30.1 MIR346 MIR324 MIR30D MIR296 MIR223 MIR197
5 glioma susceptibility 1 29.9 MIR451A MIR326 MIR296 MIR197
6 intestinal disease 29.9 MIRLET7B MIR324 MIR20A MIR17 MIR150
7 muscular dystrophy, duchenne type 29.8 MIR361 MIR30D MIR299 MIR197 ENSG00000266919
8 psoriasis 29.7 MIR326 MIR20A MIR197 MIR17 MIR133B MIR122
9 gastrointestinal system disease 29.5 MIRLET7B MIR324 MIR223 MIR20A MIR17 MIR150
10 reynolds syndrome 12.7
11 biliary cirrhosis, primary, 1 12.2
12 biliary cirrhosis, primary, 4 11.8
13 biliary cirrhosis, primary, 5 11.8
14 biliary cirrhosis, primary, 2 11.8
15 biliary cirrhosis, primary, 3 11.8
16 liver cirrhosis 11.7
17 primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome 11.3
18 cholangitis 11.2
19 autoimmune disease 11.1
20 liver disease 11.1
21 cholestasis 11.1
22 autoimmune hepatitis 11.1
23 sclerosing cholangitis 11.0
24 cholangitis, primary sclerosing 11.0
25 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.9
26 varicose veins 10.9
27 portal hypertension 10.9
28 systemic scleroderma 10.8
29 viral hepatitis 10.8
30 scleroderma, familial progressive 10.8
31 esophageal varix 10.7
32 raynaud phenomenon 10.7
33 osteoporosis 10.7
34 thyroiditis 10.7
35 sjogren syndrome 10.7
36 crest syndrome 10.7
37 sarcoidosis 1 10.7
38 bone disease 10.7
39 hepatocellular carcinoma 10.7
40 bone mineral density quantitative trait locus 8 10.7
41 bone mineral density quantitative trait locus 15 10.7
42 polymyositis 10.6
43 hypercholesterolemia, familial, 1 10.6
44 osteomalacia 10.6
45 hepatitis 10.6
46 deficiency anemia 10.5
47 hemolytic anemia 10.5
48 anemia, x-linked, with or without neutropenia and/or platelet abnormalities 10.5
49 graft-versus-host disease 10.5
50 telangiectasis 10.5

Comorbidity relations with Primary Biliary Cirrhosis via Phenotypic Disease Network (PDN):


Active Peptic Ulcer Disease Acute Cystitis
Cholangitis Deficiency Anemia
Hepatic Encephalopathy Hypothyroidism
Portal Hypertension

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



Diseases related to Primary Biliary Cirrhosis

Symptoms & Phenotypes for Primary Biliary Cirrhosis

Human phenotypes related to Primary Biliary Cirrhosis:

58 31 (show all 33)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 cirrhosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0001394
2 biliary cirrhosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002613
3 conjugated hyperbilirubinemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002908
4 hyperpigmentation of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0000953
5 dermatographic urticaria 58 31 hallmark (90%) Very frequent (99-80%) HP:0011971
6 portal hypertension 58 31 frequent (33%) Frequent (79-30%) HP:0001409
7 pruritus 58 31 frequent (33%) Frequent (79-30%) HP:0000989
8 hepatic fibrosis 58 31 frequent (33%) Frequent (79-30%) HP:0001395
9 jaundice 58 31 frequent (33%) Frequent (79-30%) HP:0000952
10 hepatic failure 58 31 frequent (33%) Frequent (79-30%) HP:0001399
11 abnormality of the thyroid gland 58 31 frequent (33%) Frequent (79-30%) HP:0000820
12 orthostatic hypotension 58 31 frequent (33%) Frequent (79-30%) HP:0001278
13 hepatocellular carcinoma 58 31 frequent (33%) Frequent (79-30%) HP:0001402
14 increased circulating igm level 58 31 frequent (33%) Frequent (79-30%) HP:0003496
15 antinuclear antibody positivity 58 31 frequent (33%) Frequent (79-30%) HP:0003493
16 onychomycosis 58 31 frequent (33%) Frequent (79-30%) HP:0012203
17 elevated alkaline phosphatase 58 31 frequent (33%) Frequent (79-30%) HP:0003155
18 abnormality of the intrahepatic bile duct 58 31 frequent (33%) Frequent (79-30%) HP:0011040
19 abnormal circulating lipid concentration 31 frequent (33%) HP:0003119
20 fatigue 58 31 occasional (7.5%) Occasional (29-5%) HP:0012378
21 ascites 58 31 occasional (7.5%) Occasional (29-5%) HP:0001541
22 hepatitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012115
23 osteoporosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000939
24 celiac disease 58 31 occasional (7.5%) Occasional (29-5%) HP:0002608
25 hypoalbuminemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003073
26 abdominal distention 58 31 occasional (7.5%) Occasional (29-5%) HP:0003270
27 excessive daytime somnolence 58 31 occasional (7.5%) Occasional (29-5%) HP:0001262
28 increased circulating iga level 58 31 occasional (7.5%) Occasional (29-5%) HP:0003261
29 gastrointestinal inflammation 58 31 very rare (1%) Very rare (<4-1%) HP:0004386
30 sleep disturbance 58 Occasional (29-5%)
31 autoimmunity 58 Frequent (79-30%)
32 abnormality of lipid metabolism 58 Frequent (79-30%)
33 recurrent fungal infections 58 Frequent (79-30%)

Drugs & Therapeutics for Primary Biliary Cirrhosis

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

(show top 50) (show all 155)
# 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
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Mycophenolic acid Approved Phase 4 24280-93-1 446541
5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
6
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
7
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
8
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
9 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
10
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
11
Cefoperazone Approved, Investigational Phase 4 62893-19-0 44185
12
Meropenem Approved, Investigational Phase 4 96036-03-2, 119478-56-7 441130 64778
13
Teicoplanin Approved, Investigational Phase 4 61036-62-2
14
Tinidazole Approved, Investigational Phase 4 19387-91-8 5479
15
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
16 Protein Kinase Inhibitors Phase 4
17 Anti-Bacterial Agents Phase 4
18 Anti-Infective Agents Phase 4
19 Immunologic Factors Phase 4
20 Dermatologic Agents Phase 4
21 Immunosuppressive Agents Phase 4
22 Antirheumatic Agents Phase 4
23 Antitubercular Agents Phase 4
24 Methylprednisolone Acetate Phase 4
25 Cyclosporins Phase 4
26 Antifungal Agents Phase 4
27 Calcineurin Inhibitors Phase 4
28 Antibiotics, Antitubercular Phase 4
29 Alkylating Agents Phase 4
30 Antiprotozoal Agents Phase 4
31 Antiparasitic Agents Phase 4
32 Sulperazone Phase 4
33
Fenofibrate Approved Phase 3 49562-28-9 3339
34
Metformin Approved Phase 2, Phase 3 657-24-9 14219 4091
35
Metronidazole Approved Phase 2, Phase 3 443-48-1 4173
36
chenodeoxycholic acid Approved Phase 3 474-25-9 10133
37
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
38
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
39
leucovorin Approved Phase 3 58-05-9 6006 143
40
Colchicine Approved Phase 3 64-86-8 2833 6167
41
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
42
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
43
Copper Approved, Investigational Phase 3 7440-50-8 27099
44
Atorvastatin Approved Phase 3 134523-00-5 60823
45
Ursodeoxycholic acid Approved, Investigational Phase 3 128-13-2 31401
46
Budesonide Approved Phase 3 51333-22-3 63006 5281004
47
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
48
Tauroursodeoxycholic acid Experimental, Investigational Phase 3 14605-22-2 12443252
49
Tetrathiomolybdate Investigational Phase 3 16330-92-0
50 Hypoglycemic Agents Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 154)
# Name Status NCT ID Phase Drugs
1 A Randomized Controlled Trial of 18-22mg/kg/d Ursodeoxycholic in Refractory Primary Biliary Cholangitis Unknown status NCT03345589 Phase 4 18-22mg/kg/d Ursodeoxycholic;13-15mg/kg/d Ursodeoxycholic
2 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)
3 Abatacept For The Treatment Of Primary Biliary Cirrhosis With An Incomplete Biochemical Response To Ursodeoxycholic Acid Completed NCT02078882 Phase 4
4 Effectiveness of S-adenosyl-L-methionine in Patients With Primary Biliary Cirrhosis Completed NCT02557360 Phase 4
5 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
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 Mycophenolate Mofetil Versus Cyclosporin A in the Treatment of Primary Biliary Cholangitis-autoimmune Hepatitis Overlap Syndrome Duo to Nonresponse to Standard Therapy Not yet recruiting NCT04376528 Phase 4 Cyclosporin A;Mycophenolate Mofetil
9 Antibiotics Treatment of Cholangitis Post-Kasai Portoenterostomy Not yet recruiting NCT04370145 Phase 4 Sulperazon;Teicoplanin;Meropenem Injection
10 Efficacy and Security of Bezafibrate in Patients With Primary Biliary Cirrhosis Without Biochemical Response to Ursodeoxycholic Acid: A Randomized, Double-blind, Placebo-controlled Trial Unknown status NCT02937012 Phase 3 Bezafibrate;Ursodeoxycholic Acid;Placebo (for Bezafibrate)
11 Fenofibrate in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis: a Randomized Control Study Unknown status NCT02823353 Phase 3 Fenofibrate;UDCA
12 Fuzhenghuayu in Combination With Ursodeoxycholic Acid in Primary Biliary Cirrhosis Unknown status NCT02916290 Phase 3 Fuzhenghuayu;UDCA
13 Fenofibrate for Patients With Primary Biliary Cirrhosis Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02823366 Phase 3 Fenofibrate;UDCA
14 Fuzhenghuayu for Patients With PBC Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02916641 Phase 3 Fuzhenghuayu;UDCA
15 METformin in Intrahepatic Cholestasis of Pregnancy (METRIC) Study Unknown status NCT03056274 Phase 2, Phase 3 Metformin;Ursodeoxycholic Acid
16 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
17 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
18 Probiotics in Patients With Primary Sclerosing Cholangitis and Inflammatory Bowel Disease- a Randomized Placebo-Controlled Cross-Over Trial Unknown status NCT00161148 Phase 3 Probiotics
19 Multicenter Randomized Trial Comparing Short-term Stenting Versus Balloon Dilatation for Dominant Strictures in Primary Sclerosing Cholangitis Unknown status NCT01398917 Phase 3
20 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
21 Phase III Randomized Study of Ursodiol With Vs Without Methotrexate for Primary Biliary Cirrhosis Completed NCT00004784 Phase 3 methotrexate;ursodiol
22 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
23 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
24 Randomized Controlled Pilot Study of Combivir for Patients With Primary Biliary Cirrhosis Completed NCT00490620 Phase 2, Phase 3 Combination antiviral therapy;Placebo
25 Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis Completed NCT00844402 Phase 3 Atorvastatin
26 Ursodiol-Methotrexate for Primary Biliary Cirrhosis Completed NCT00006168 Phase 3 Methotrexate
27 Phase III Trial of Tetrathiomolybdate (TM) in Primary Biliary Cirrhosis Completed NCT00805805 Phase 3 Tetrathiomolybdate
28 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
29 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
30 A Phase 3 Study to Evaluate the Place of Budesonide in the Treatment of GVHD Completed NCT00128739 Phase 3 Budesonide
31 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
32 Long-term Safety and Tolerability Study of Linerixibat for the Treatment of Cholestatic Pruritus in Participants With Primary Biliary Cholangitis Not yet recruiting NCT04167358 Phase 3 Linerixibat
33 An Open Label Long-Term Study to Evaluate the Safety and Tolerability of Seladelpar in Subjects With Primary Biliary Cholangitis (PBC) Suspended NCT03301506 Phase 2, Phase 3 Seladelpar 2 mg Capsule;Seladelpar 5 mg Capsule;Seladelpar 10 mg Capsule
34 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) Suspended NCT03602560 Phase 3 seladelpar 5-10 mg;seladelpar 10 mg;Placebo
35 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
36 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
37 An 8-week, Dose Ranging, Open Label, Randomized, Phase 2 Study With a 44-week Extension, to Evaluate the Safety and Efficacy of MBX-8025 in Subjects With Primary Biliary Cholangitis (PBC) and an Inadequate Response to or Intolerance to Ursodeoxycholic Acid (UDCA) Unknown status NCT02955602 Phase 2 MBX-8025 2 mg Capsule;MBX-8025 5 mg Capsule;MBX-8025 10 mg Capsule
38 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
39 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
40 Open-Labelled, Multicenter Phase II Study of Rituximab in Patients With Steroid-Refractory Chronic Graft-Versus-Host Disease Unknown status NCT00472225 Phase 2 Rituximab
41 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
42 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
43 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
44 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
45 A Study of INT-747 (6-ECDCA) Monotherapy in Patients With Primary Biliary Cirrhosis Completed NCT00570765 Phase 2 Placebo;INT-747;INT-747
46 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
47 Treatment of Cholestatic Pruritus With Sertraline Completed NCT00058903 Phase 2 sertraline
48 A Double-Blind, Randomized, Placebo-Controlled Clinical Trial to Assess the Efficacy and Safety of Oral GKT137831 in Patients With Primary Biliary Cholangitis Receiving Ursodeoxycholic Acid and With Persistently Elevated Alkaline Phosphatase Completed NCT03226067 Phase 2 GKT137831;Placebo oral capsule
49 Pilot Study of Fenofibrate for Primary Biliary Cirrhosis Completed NCT00575042 Phase 2 Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle)
50 A Pilot Study of Pentoxifylline for the Treatment of Primary Biliary Cirrhosis Completed NCT01249092 Phase 2 Pentoxifylline

Search NIH Clinical Center for Primary Biliary Cirrhosis

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Colchicine
Penicillamine
Ursodiol

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Primary Biliary Cirrhosis cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: liver cirrhosis, biliary

Genetic Tests for Primary Biliary Cirrhosis

Anatomical Context for Primary Biliary Cirrhosis

MalaCards organs/tissues related to Primary Biliary Cirrhosis:

40
Liver, T Cells, Bone, Testes, B Cells, Thyroid, Skin
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Primary Biliary Cirrhosis:
# Tissue Anatomical CompartmentCell Relevance
1 Umbilical Cord Wharton's Jelly Mesenchymal Stem Cells Potential therapeutic candidate

Publications for Primary Biliary Cirrhosis

Articles related to Primary Biliary Cirrhosis:

(show top 50) (show all 8390)
# Title Authors PMID Year
1
EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. 52 61
28427765 2017
2
Primary biliary cirrhosis is associated with altered hepatic microRNA expression. 61 46
19345069 2009
3
WITHDRAWN:MicroRNA profile in peripheral blood T cells of patients with primary biliary cirrhosis. 61 46
18631158 2008
4
LBR mutation and nuclear envelope defects in a patient affected with Reynolds syndrome. 54 61
20522425 2010
5
Catalytic domain of PDC-E2 contains epitopes recognized by antimitochondrial antibodies in primary biliary cirrhosis. 54 61
20180236 2010
6
Diagnostic utility of IgG and IgM immunohistochemistry in autoimmune liver disease. 54 61
20101770 2010
7
Mycoplasma antigens as a possible trigger for the induction of antimitochondrial antibodies in primary biliary cirrhosis. 61 54
19638108 2009
8
The pathogenesis of primary biliary cirrhosis. 61 54
19630465 2009
9
Vitamin D receptor polymorphisms in primary biliary cirrhosis: a functional connection? 61 54
19398232 2009
10
Vitamin D receptor polymorphisms are associated with increased susceptibility to primary biliary cirrhosis in Japanese and Italian populations. 54 61
19376604 2009
11
Farnesoid X receptor agonists in biliary tract disease. 61 54
19300246 2009
12
CTLA4/ICOS gene variants and haplotypes are associated with rheumatoid arthritis and primary biliary cirrhosis in the Canadian population. 54 61
19333938 2009
13
Bile salts control the antimicrobial peptide cathelicidin through nuclear receptors in the human biliary epithelium. 54 61
19245866 2009
14
Apotopes and the biliary specificity of primary biliary cirrhosis. 54 61
19185000 2009
15
When lightning strikes twice: the plot thickens for a dual role of the anion exchanger 2 (AE2/SLC4A2) in the pathogenesis and treatment of primary biliary cirrhosis. 61 54
19157624 2009
16
Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization. 54 61
19094117 2009
17
Decreased production of immunoglobulin M and A in autoimmune pancreatitis. 54 61
19626266 2009
18
Xenobiotic incorporation into pyruvate dehydrogenase complex can occur via the exogenous lipoylation pathway. 54 61
19003917 2008
19
Primary biliary cirrhosis is associated with a genetic variant in the 3' flanking region of the CTLA4 gene. 54 61
18778710 2008
20
Primary biliary cirrhosis following Lactobacillus vaccination for recurrent vaginitis. 54 61
18644655 2008
21
Isoform of fibronectin mediates bone loss in patients with primary biliary cirrhosis by suppressing bone formation. 54 61
18348696 2008
22
Increased expression of Toll-like receptor 3 in intrahepatic biliary epithelial cells at sites of ductular reaction in diseased livers. 54 61
19669308 2008
23
Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis. 54 61
18471521 2008
24
Interacting alleles of the coinhibitory immunoreceptor genes cytotoxic T-lymphocyte antigen 4 and programmed cell-death 1 influence risk and features of primary biliary cirrhosis. 61 54
18041714 2008
25
Breakthrough of immune self-tolerance to calreticulin induced by CpG-oligodeoxynucleotides as adjuvant. 54 61
18402289 2007
26
Clinical and serological features of patients with autoantibodies to GW/P bodies. 61 54
17870671 2007
27
Association of CTLA4 single nucleotide polymorphisms with viral but not autoimmune liver disease. 54 61
18049163 2007
28
Nuclear envelope protein autoantigens in primary biliary cirrhosis. 54 61
17931195 2007
29
Intrahepatic expression of the co-stimulatory molecules programmed death-1, and its ligands in autoimmune liver disease. 61 54
17610472 2007
30
High aspartate to alanine aminotransferase ratio is an indicator of cirrhosis and poor outcome in patients with primary sclerosing cholangitis. 61 54
17498256 2007
31
Increased serum anti-carbonic anhydrase II antibodies in patients with Graves' disease. 54 61
17516290 2007
32
Differential epitope mapping of antibodies to PDC-E2 in patients with hematologic malignancies after allogeneic hematopoietic stem cell transplantation and primary biliary cirrhosis. 61 54
17068145 2007
33
Pathogenesis of autoimmune hepatitis: from break of tolerance to immune-mediated hepatocyte apoptosis. 61 54
17320796 2007
34
AMA production in primary biliary cirrhosis is promoted by the TLR9 ligand CpG and suppressed by potassium channel blockers. 54 61
17256753 2007
35
MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes. 54 61
17295178 2007
36
Primary biliary cirrhosis in female subjects with sicca-associated antibodies. 61 54
18084701 2007
37
Possible association of cytotoxic T lymphocyte antigen-4 genetic polymorphism with liver damage of primary biliary cirrhosis in Japan. 61 54
17427759 2006
38
Cytokeratin-7, cytokeratin-19, and c-Kit: Immunoreaction during the evolution stages of primary biliary cirrhosis. 54 61
16963314 2006
39
IL-2 receptor alpha(-/-) mice and the development of primary biliary cirrhosis. 54 61
17058261 2006
40
Transgenic mice aberrantly expressing pyruvate dehydrogenase complex E2 component on biliary epithelial cells do not show primary biliary cirrhosis. 54 61
16792678 2006
41
Altered expression of alkaline phosphatase (ALP) in the liver of primary biliary cirrhosis (PBC) patients. 54 61
16567122 2006
42
PDC-E3BP is not a dominant T-cell autoantigen in primary biliary cirrhosis. 61 54
16629643 2006
43
Cholestasis and cholestatic syndromes. 61 54
16550034 2006
44
Anti-p97/VCP antibodies: an autoantibody marker for a subset of primary biliary cirrhosis patients with milder disease? 61 54
16640662 2006
45
A novel multidrug-resistance protein 2 gene mutation identifies a subgroup of patients with primary biliary cirrhosis and pruritus. 61 54
16628674 2006
46
Multidrug resistance 1 genotype and disposition of budesonide in early primary biliary cirrhosis. 61 54
16584389 2006
47
Expression of epithelial cellular adhesion molecule (Ep-CAM) in chronic (necro-)inflammatory liver diseases and hepatocellular carcinoma. 61 54
16364680 2006
48
Tubulointerstitial nephritis and Fanconi syndrome in primary biliary cirrhosis. 61 54
16129198 2005
49
Primary biliary cirrhosis is characterized by IgG3 antibodies cross-reactive with the major mitochondrial autoepitope and its Lactobacillus mimic. 61 54
16025495 2005
50
Enhanced expression of type I interferon and toll-like receptor-3 in primary biliary cirrhosis. 54 61
15856047 2005

Variations for Primary Biliary Cirrhosis

Expression for Primary Biliary Cirrhosis

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

Pathways for Primary Biliary Cirrhosis

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

# Super pathways Score Top Affiliating Genes
1 11.85 MIRLET7B MIR451A MIR326 MIR324 MIR30D MIR223
2 11.06 MIR212 MIR20A MIR17

GO Terms for Primary Biliary Cirrhosis

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

# Name GO ID Score Top Affiliating Genes
1 extracellular vesicle GO:1903561 9.43 MIRLET7B MIR451A MIR30D MIR20A MIR17 MIR122
2 extracellular space GO:0005615 9.4 MIRLET7B MIR451A MIR361 MIR328 MIR30D MIR223

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.67 MIR451A MIR212 MIR20A MIR17
2 gene silencing by miRNA GO:0035195 9.55 MIRLET7B MIR451A MIR361 MIR346 MIR328 MIR326
3 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.4 MIR451A MIR20A
4 miRNA mediated inhibition of translation GO:0035278 9.35 MIR299 MIR212 MIR20A MIR17 MIR133B
5 negative regulation of vascular endothelial growth factor production GO:1904046 9.33 MIR361 MIR20A MIR17
6 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.32 MIR20A MIR17
7 positive regulation of pulmonary blood vessel remodeling GO:1905111 9.26 MIR20A MIR17

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

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.36 MIRLET7B MIR361 MIR328 MIR299 MIR296 MIR223

Sources for Primary Biliary Cirrhosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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