PBC
MCID: PRM236
MIFTS: 62

Primary Biliary Cholangitis (PBC)

Categories: Gastrointestinal diseases, 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 12 20 58 15 37
Primary Biliary Cirrhosis 12 73 20 58 36 15 17 70 32
Familial Primary Biliary Cirrhosis 20 70
Biliary Liver Cirrhosis 12 17
Pbc 20 58
Chronic Nonsuppurative Destructive Cholangitis 12
Biliary Cirrhosis Primary 54
Liver Cirrhosis, Biliary 44
Cholestatic Cirrhosis 12
Hanot's Cirrhosis 70
Biliary Cirrhosis 70
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 Cholangitis

GARD : 20 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 hepatocellular carcinoma and systemic lupus erythematosus. An important gene associated with Primary Biliary Cholangitis is MIR223 (MicroRNA 223), and among its related pathways/superpathways are MicroRNAs in cancer and Parkinsons Disease Pathway. The drugs Abatacept and Ursodeoxycholic acid 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 : 73 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 677)
# Related Disease Score Top Affiliating Genes
1 hepatocellular carcinoma 31.2 MIRLET7B MIR483 MIR324 MIR223 MIR212 MIR20A
2 systemic lupus erythematosus 31.1 MIR346 MIR324 MIR30D MIR296 MIR223 MIR197
3 peripheral nervous system disease 31.1 MIR483 MIR324 MIR17 MIR122
4 arteries, anomalies of 31.0 MIRLET7B MIR483 MIR296 MIR223 MIR212 MIR17
5 lipoprotein quantitative trait locus 30.9 MIR483 MIR296 MIR223 MIR17 MIR150
6 intestinal disease 30.8 MIRLET7B MIR483 MIR324 MIR223 MIR20A MIR17
7 gastrointestinal system disease 30.8 MIRLET7B MIR483 MIR324 MIR223 MIR20A MIR17
8 disease by infectious agent 30.8 MIR223 MIR17 MIR150 MIR122
9 psoriasis 30.8 MIR326 MIR20A MIR197 MIR17 MIR133B MIR122
10 muscular dystrophy, duchenne type 30.8 MIR361 MIR30D MIR299 MIR197 ENSG00000266919
11 non-alcoholic fatty liver disease 30.7 MIR451A MIR30D MIR17 MIR122
12 glioblastoma 30.7 MIR451A MIR328 MIR326 MIR296 MIR197 MIR133B
13 gallbladder disease 30.7 MIR483 MIR30D MIR17
14 myeloma, multiple 30.6 MIRLET7B MIR483 MIR361 MIR17
15 connective tissue disease 30.5 MIR483 MIR223 MIR17 MIR150 MIR122
16 cholangitis 11.4
17 primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome 11.3
18 reynolds syndrome 11.2
19 biliary cirrhosis, primary, 1 11.2
20 biliary cirrhosis, primary, 2 11.0
21 biliary cirrhosis, primary, 3 11.0
22 biliary cirrhosis, primary, 4 11.0
23 biliary cirrhosis, primary, 5 11.0
24 cholestasis 10.9
25 autoimmune hepatitis 10.8
26 autoimmune disease 10.8
27 cholangitis, primary sclerosing 10.7
28 sclerosing cholangitis 10.7
29 hepatitis 10.7
30 liver disease 10.7
31 liver cirrhosis 10.7
32 pleural disease 10.6 MIRLET7B MIR30D MIR223 MIR17
33 acquired metabolic disease 10.6 MIRLET7B MIR483 MIR223 MIR20A MIR17 MIR150
34 immune system disease 10.6 MIRLET7B MIR483 MIR223 MIR20A MIR17 MIR150
35 glucose metabolism disease 10.6 MIR483 MIR223 MIR20A MIR17 MIR150
36 nemaline myopathy 10.6 MIR361 MIR299 MIR223 ENSG00000266919
37 nasopharyngeal disease 10.6 MIR483 MIR324 MIR296 MIR223 MIR17
38 lymphatic system cancer 10.6 MIR483 MIR361 MIR223 MIR20A MIR17 MIR150
39 prostate disease 10.6 MIR483 MIR361 MIR223 MIR20A MIR17 MIR150
40 central nervous system disease 10.6 MIRLET7B MIR223 MIR212 MIR17 MIR150
41 pharynx cancer 10.6 MIR483 MIR324 MIR296 MIR223 MIR17
42 sensory system disease 10.6 MIRLET7B MIR483 MIR361 MIR223 MIR20A MIR17
43 oral squamous cell carcinoma 10.6 MIRLET7B MIR30D MIR296 MIR223 MIR197 MIR17
44 endocrine gland cancer 10.6 MIRLET7B MIR483 MIR324 MIR223 MIR20A MIR17
45 stomach disease 10.6 MIR483 MIR361 MIR223 MIR212 MIR20A MIR17
46 endocrine system disease 10.6 MIRLET7B MIR483 MIR324 MIR223 MIR20A MIR17
47 lymphatic system disease 10.6 MIR483 MIR361 MIR324 MIR223 MIR20A MIR17
48 gastrointestinal system cancer 10.6 MIRLET7B MIR483 MIR324 MIR223 MIR20A MIR17
49 overnutrition 10.6 MIR483 MIR223 MIR17 MIR122
50 thoracic cancer 10.6 MIRLET7B MIR483 MIR324 MIR296 MIR223 MIR20A

Comorbidity relations with Primary Biliary Cholangitis 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 Cholangitis:



Diseases related to Primary Biliary Cholangitis

Symptoms & Phenotypes for Primary Biliary Cholangitis

Human phenotypes related to Primary Biliary Cholangitis:

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 hepatic fibrosis 58 31 frequent (33%) Frequent (79-30%) HP:0001395
8 jaundice 58 31 frequent (33%) Frequent (79-30%) HP:0000952
9 hepatic failure 58 31 frequent (33%) Frequent (79-30%) HP:0001399
10 abnormality of the thyroid gland 58 31 frequent (33%) Frequent (79-30%) HP:0000820
11 pruritus 58 31 frequent (33%) Frequent (79-30%) HP:0000989
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 elevated alkaline phosphatase 58 31 frequent (33%) Frequent (79-30%) HP:0003155
17 onychomycosis 58 31 frequent (33%) Frequent (79-30%) HP:0012203
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 Cholangitis

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

(show top 50) (show all 131)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Abatacept Approved Phase 4 332348-12-6 10237
2
Ursodeoxycholic acid Approved, Investigational Phase 4 128-13-2 31401
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
5
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
6
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
7
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
8
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
9
Mycophenolic acid Approved Phase 4 24280-93-1 446541
10
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
11
Teicoplanin Approved, Investigational Phase 4 61036-62-2
12
Cefoperazone Approved, Investigational Phase 4 62893-19-0 44185
13
Tinidazole Approved, Investigational Phase 4 19387-91-8 5479
14
Meropenem Approved, Investigational Phase 4 119478-56-7, 96036-03-2 441130 64778
15
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
16 Antirheumatic Agents Phase 4
17 Immunosuppressive Agents Phase 4
18 Dermatologic Agents Phase 4
19 Immunologic Factors Phase 4
20 Anti-Infective Agents Phase 4
21 Anti-Bacterial Agents Phase 4
22 Antibiotics, Antitubercular Phase 4
23 Methylprednisolone Acetate Phase 4
24 Cyclosporins Phase 4
25 Antitubercular Agents Phase 4
26 Calcineurin Inhibitors Phase 4
27 Antifungal Agents Phase 4
28 Alkylating Agents Phase 4
29 Antiparasitic Agents Phase 4
30 Antiprotozoal Agents Phase 4
31 Sulperazone Phase 4
32
Metformin Approved Phase 2, Phase 3 657-24-9 4091 14219
33
Metronidazole Approved Phase 2, Phase 3 443-48-1 4173
34
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
35
Colchicine Approved Phase 3 64-86-8 6167 2833
36
Levoleucovorin Approved, Investigational Phase 3 68538-85-2 149436
37
Zidovudine Approved Phase 2, Phase 3 30516-87-1 35370
38
Lamivudine Approved, Investigational Phase 2, Phase 3 134678-17-4 60825
39
chenodeoxycholic acid Approved Phase 3 474-25-9 10133
40
Atorvastatin Approved Phase 3 134523-00-5 60823
41
Copper Approved, Investigational Phase 3 7440-50-8 27099
42
Bezafibrate Approved, Investigational Phase 3 41859-67-0 39042
43
Budesonide Approved Phase 3 51333-22-3 63006 5281004
44
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
45
Tauroursodeoxycholic acid Experimental, Investigational Phase 3 14605-22-2 12443252
46
Tetrathiomolybdate Investigational Phase 3 16330-92-0
47 Hypoglycemic Agents Phase 2, Phase 3
48 Tubulin Modulators Phase 3
49 Vitamin B9 Phase 3
50 Folic Acid Antagonists Phase 3

Interventional clinical trials:

(show top 50) (show all 127)
# 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 Effectiveness of S-adenosyl-L-methionine in Patients With Primary Biliary Cirrhosis Completed NCT02557360 Phase 4
3 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)
4 Abatacept For The Treatment Of Primary Biliary Cirrhosis With An Incomplete Biochemical Response To Ursodeoxycholic Acid Completed NCT02078882 Phase 4
5 Clinical Research of Reducing Medication Regimen for Ursodeoxycholic Acid in Treatment of Stable Primary Biliary Cholangitis Recruiting NCT04650243 Phase 4 ursodeoxycholic acid
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 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 Active, not recruiting NCT03633227 Phase 4 Obeticholic Acid (OCA);Placebo
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 Fenofibrate for Patients With Primary Biliary Cirrhosis Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02823366 Phase 3 Fenofibrate;UDCA
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 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)
14 Fuzhenghuayu for Patients With PBC Who Had An Inadequate Response to Ursodeoxycholic Acid Unknown status NCT02916641 Phase 3 Fuzhenghuayu;UDCA
15 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
16 METformin in Intrahepatic Cholestasis of Pregnancy (METRIC) Study Unknown status NCT03056274 Phase 2, Phase 3 Metformin;Ursodeoxycholic Acid
17 The Effect of Bezafibrate on Cholestatic Itch Unknown status NCT02701166 Phase 3 Bezafibrate;Placebo
18 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
19 Ursodiol-Methotrexate for Primary Biliary Cirrhosis Completed NCT00006168 Phase 3 Methotrexate
20 Phase III Randomized Study of Ursodiol With Vs Without Methotrexate for Primary Biliary Cirrhosis Completed NCT00004784 Phase 3 methotrexate;ursodiol
21 Randomized Controlled Pilot Study of Combivir for Patients With Primary Biliary Cirrhosis Completed NCT00490620 Phase 2, Phase 3 Combination antiviral therapy;Placebo
22 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
23 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
24 Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis Completed NCT00844402 Phase 3 Atorvastatin
25 Phase III Trial of Tetrathiomolybdate (TM) in Primary Biliary Cirrhosis Completed NCT00805805 Phase 3 Tetrathiomolybdate
26 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
27 A Double-blind, Randomized, Placebo-Controlled Study and Open-label Long Term Extension to Evaluate the Efficacy and Safety of Elafibranor 80 mg in Patients With Primary Biliary Cholangitis With Inadequate Response or Intolerance to Ursodeoxycholic Acid Recruiting NCT04526665 Phase 3 Elafibranor 80mg;Placebo
28 ASSURE: An Open Label Long-Term Study to Evaluate the Safety and Tolerability of Seladelpar in Subjects With Primary Biliary Cholangitis (PBC) Recruiting NCT03301506 Phase 3 Seladelpar 5 mg Capsule;Seladelpar 10 mg Capsule
29 RESPONSE: A Placebo-controlled, Randomized, Phase 3 Study to Evaluate the Efficacy and Safety of Seladelpar in Patients With Primary Biliary Cholangitis (PBC) and an Inadequate Response to or an Intolerance to Ursodeoxycholic Acid (UDCA) Recruiting NCT04620733 Phase 3 Seladelpar 10 mg;Placebo;Seladelpar 5 mg
30 Long-term Safety and Tolerability Study of Linerixibat for the Treatment of Cholestatic Pruritus in Participants With Primary Biliary Cholangitis Recruiting NCT04167358 Phase 3 Linerixibat
31 Safety and Efficacy of Bezafibrate Plus Ursodesoxicolic Acid in Patients With Primary Biliary Cholangitis Without Response Active, not recruiting NCT04751188 Phase 3 Bezafibrate 200 MG Oral Tablet;Placebo;Ursodeoxycholic Acid
32 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
33 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
34 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
35 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
36 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
37 A Pilot Study of Pentoxifylline for the Treatment of Primary Biliary Cirrhosis Completed NCT01249092 Phase 2 Pentoxifylline
38 Open-Label Pilot Investigation of Moexipril for the Treatment of Primary Biliary Cirrhosis (PBC) Completed NCT00588302 Phase 2 Moexipril
39 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
40 Treatment of Cholestatic Pruritus With Sertraline Completed NCT00058903 Phase 2 sertraline
41 A Randomized, Double-blind, Multi-dose, Placebo-controlled Study to Evaluate the Efficacy, Safety and Tolerability of GSK2330672 Administration for the Treatment of Pruritus in Patients With Primary Biliary Cholangitis (GLIMMER: GSK2330672 triaL of IBAT Inhibition With Multidose Measurement for Evaluation of Response) Completed NCT02966834 Phase 2 Placebo;GSK2330672
42 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) Completed NCT02955602 Phase 2 MBX-8025 2 mg Capsule;MBX-8025 5 mg Capsule;MBX-8025 10 mg Capsule
43 A Phase 2, Prospective, Multicenter, Randomized, Double-blind, Placebo-controlled Study to Evaluate Safety, Tolerability and Efficacy of Saroglitazar Magnesium in Patients With Primary Biliary Cholangitis (EPICS ) Completed NCT03112681 Phase 2 Saroglitazar magnesium 2 mg;Saroglitazar magnesium 4 mg;Placebo Oral Tablet
44 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
45 Effects of Rituximab (Rituxan) on B Cell and AMA Response in Patients With Primary Biliary Cirrhosis Completed NCT00364819 Phase 1, Phase 2 rituximab
46 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)
47 Pilot Study of Fenofibrate for Primary Biliary Cirrhosis Completed NCT00575042 Phase 2 Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle)
48 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
49 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
50 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

Search NIH Clinical Center for Primary Biliary Cholangitis

Inferred drug relations via UMLS 70 / NDF-RT 51 :


Colchicine
Penicillamine
Ursodiol

Cell-based therapeutics:


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

Cochrane evidence based reviews: liver cirrhosis, biliary

Genetic Tests for Primary Biliary Cholangitis

Anatomical Context for Primary Biliary Cholangitis

MalaCards organs/tissues related to Primary Biliary Cholangitis:

40
Liver, T Cells, Bone, Thyroid, Bone Marrow, Breast, Neutrophil
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

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

Publications for Primary Biliary Cholangitis

Articles related to Primary Biliary Cholangitis:

(show top 50) (show all 8636)
# Title Authors PMID Year
1
EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. 61 20
28427765 2017
2
Primary biliary cirrhosis is associated with altered hepatic microRNA expression. 61 47
19345069 2009
3
WITHDRAWN:MicroRNA profile in peripheral blood T cells of patients with primary biliary cirrhosis. 61 47
18631158 2008
4
LBR mutation and nuclear envelope defects in a patient affected with Reynolds syndrome. 61 54
20522425 2010
5
Catalytic domain of PDC-E2 contains epitopes recognized by antimitochondrial antibodies in primary biliary cirrhosis. 61 54
20180236 2010
6
Diagnostic utility of IgG and IgM immunohistochemistry in autoimmune liver disease. 61 54
20101770 2010
7
Mycoplasma antigens as a possible trigger for the induction of antimitochondrial antibodies in primary biliary cirrhosis. 54 61
19638108 2009
8
Vitamin D receptor polymorphisms are associated with increased susceptibility to primary biliary cirrhosis in Japanese and Italian populations. 54 61
19376604 2009
9
The pathogenesis of primary biliary cirrhosis. 54 61
19630465 2009
10
Vitamin D receptor polymorphisms in primary biliary cirrhosis: a functional connection? 61 54
19398232 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. 61 54
19245866 2009
14
Induction of autoimmune cholangitis in non-obese diabetic (NOD).1101 mice following a chemical xenobiotic immunization. 61 54
19094117 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
Apotopes and the biliary specificity of primary biliary cirrhosis. 61 54
19185000 2009
17
Decreased production of immunoglobulin M and A in autoimmune pancreatitis. 61 54
19626266 2009
18
Xenobiotic incorporation into pyruvate dehydrogenase complex can occur via the exogenous lipoylation pathway. 61 54
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. 61 54
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. 61 54
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. 54 61
18041714 2008
25
Clinical and serological features of patients with autoantibodies to GW/P bodies. 61 54
17870671 2007
26
Breakthrough of immune self-tolerance to calreticulin induced by CpG-oligodeoxynucleotides as adjuvant. 61 54
18402289 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. 54 61
17610472 2007
30
High aspartate to alanine aminotransferase ratio is an indicator of cirrhosis and poor outcome in patients with primary sclerosing cholangitis. 54 61
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. 54 61
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. 61 54
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. 54 61
17427759 2006
38
IL-2 receptor alpha(-/-) mice and the development of primary biliary cirrhosis. 61 54
17058261 2006
39
Cytokeratin-7, cytokeratin-19, and c-Kit: Immunoreaction during the evolution stages of primary biliary cirrhosis. 61 54
16963314 2006
40
Transgenic mice aberrantly expressing pyruvate dehydrogenase complex E2 component on biliary epithelial cells do not show primary biliary cirrhosis. 61 54
16792678 2006
41
Cholestasis and cholestatic syndromes. 54 61
16550034 2006
42
Altered expression of alkaline phosphatase (ALP) in the liver of primary biliary cirrhosis (PBC) patients. 54 61
16567122 2006
43
Anti-p97/VCP antibodies: an autoantibody marker for a subset of primary biliary cirrhosis patients with milder disease? 61 54
16640662 2006
44
A novel multidrug-resistance protein 2 gene mutation identifies a subgroup of patients with primary biliary cirrhosis and pruritus. 61 54
16628674 2006
45
PDC-E3BP is not a dominant T-cell autoantigen in primary biliary cirrhosis. 54 61
16629643 2006
46
Multidrug resistance 1 genotype and disposition of budesonide in early primary biliary cirrhosis. 54 61
16584389 2006
47
Expression of epithelial cellular adhesion molecule (Ep-CAM) in chronic (necro-)inflammatory liver diseases and hepatocellular carcinoma. 54 61
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 Cholangitis

Expression for Primary Biliary Cholangitis

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

Pathways for Primary Biliary Cholangitis

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

# Super pathways Score Top Affiliating Genes
1 11.89 MIRLET7B MIR483 MIR451A MIR326 MIR324 MIR30D
2 11.06 MIR212 MIR20A MIR17

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 extracellular exosome GO:0070062 9.76 MIRLET7B MIR451A MIR328 MIR30D MIR223 MIR197
2 extracellular vesicle GO:1903561 9.43 MIRLET7B MIR451A MIR30D MIR20A MIR17 MIR122
3 extracellular space GO:0005615 9.4 MIRLET7B MIR451A MIR361 MIR328 MIR30D MIR223

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.71 MIR451A MIR212 MIR20A MIR17
2 gene silencing by miRNA GO:0035195 9.58 MIRLET7B MIR483 MIR451A MIR361 MIR346 MIR328
3 negative regulation of cell migration involved in sprouting angiogenesis GO:0090051 9.5 MIR483 MIR361 MIR20A
4 negative regulation of blood vessel endothelial cell proliferation involved in sprouting angiogenesis GO:1903588 9.48 MIR483 MIR361
5 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.46 MIR451A MIR20A
6 negative regulation of amyloid precursor protein biosynthetic process GO:0042985 9.43 MIR20A MIR17
7 miRNA mediated inhibition of translation GO:0035278 9.43 MIR483 MIR299 MIR212 MIR20A MIR17 MIR133B
8 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.37 MIR20A MIR17
9 negative regulation of vascular endothelial growth factor production GO:1904046 9.33 MIR361 MIR20A MIR17
10 positive regulation of pulmonary blood vessel remodeling GO:1905111 9.26 MIR20A MIR17

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

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.4 MIRLET7B MIR483 MIR361 MIR328 MIR299 MIR296
2 mRNA 3'-UTR binding GO:0003730 9.26 MIR361 MIR20A MIR17 MIR133B

Sources for Primary Biliary Cholangitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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