1. BRÍD MCHUGH C12355236 DT204/2 R&D ASSIGNMENT Primary Biliary Cirrhosis

2. What is Primary Biliary Cirrhosis? It is a disease which affects the liver. The bile ducts in the liver are slowly destroyed. Bile ducts transport bile from the liver. Damage to the ducts causes a build up of bile in the liver. This leads to damage of the liver tissue.

3. What is Primary Biliary Cirrhosis? This disease is thought to be an autoimmune disease. The causes of the disease are not clear. The disease is slow developing. The progression of the disease can be delayed by the use of medication, if caught early enough.

5. What are the Symptoms? Much of the time, the patient is symptom-less for years. Some symptoms include:  Fatigue  Itchy skin  Dry eyes  Dry mouth  Jaundice  Accumulation of fluid in abdomen

6. Development of the Disease Inflammation of PBC begins when T cells start gathering in the liver. In PBC T cells invade and destroy the cells lining the small bile ducts in the liver. The inflammation in the small ducts spreads in time and destroys nearby liver cells. As the cells are destroyed they are replaced by scar tissue and this contributes to cirrhosis.

7. Risk Factors Factors which may increase chances of developing primary biliary cirrhosis:  Sex  Age  Infection

8. Epidemiology 12.9 per 100000 population in the UK About 90% of these are women More common among northern Europeans The prevalence appears to be rising since the 1980s. This might be due to increased survival and also increased awareness of the disease.

9. Complications Complications which may arise due to the disease include:  Portal hypertension  Enlarged veins  Liver cancer  Vitamin deficiencies

10. Tests and Diagnosis Tests used to diagnose primary biliary cirrhosis include:  Blood tests to check liver function (ALP)  Blood tests to check for signs of autoimmune disease  Imaging tests to examine the liver  Liver biopsy for testing

11. Treatment There is no cure for PBC. Treatment is to prevent liver damage as much as possible and relieve symptoms. Self-help Ursodeoxycholic acid Liver transplant Drugs used to inhibit the autoimmune response

12. Ursodeoxycholic Acid UDCA is used to prevent liver damage by changing the composition of the bile and reduce inflammation of the liver It reduces the levels of liver enzymes It reduces the need for a liver transplant If responding well to UDCA it is likely that PBC will remain under control and will not shorten life span.

13. Liver Transplant This is recommended if the disease may become life threatening. This may lead to further complications, much like all organ transplant operations. There is a rather high success rate for these operations.

14. Drugs used to inhibit the Autoimmune Response Methotrexate is used within clinical trials. It is however thought to increase mortality Steroids may help but osteoporosis is a concern Ciclosporin may be benificial also but should be used in clinical trials as it has a high rate of complications e.g renal dysfunction

15. Staging Portal stage Periportal stage Septal stage Cirrhotic stage

16. Lifestyle Changes Eat more sodium-reduced foods Exercise more often Reduce alcohol consumption

17. References National Digestive Diseases, 2012, available at: digestive.niddk.nih.gov www.patient.co.uk/doctor/primary-biliary-cirrhosis Primary Biliary Cirrhosis, Mayo Clinic Staff, 2013, available at http://www.mayoclinic.org/diseases-conditions/primary-biliary-cirrhosis Primary Biliary Cirrhosis, Mayo Clinic Staff, 2013, available at http://www.mayoclinic.org/diseases-conditions/primary-biliary-cirrhosis PBC Treatment, 2012, available at: http://www.nhs.uk/Conditions/Primary-biliary-cirrhosis PBC Treatment, 2012, available at: http://www.nhs.uk/Conditions/Primary-biliary-cirrhosis Primary biliary cirrhosis in 2014, Flores, A., 2014, Accessed from PubMed, available at http://www.ncbi.nlm.nih.gov/pubmed/24671010http://www.ncbi.nlm.nih.gov/pubmed/24671010 Primary Biliary Cirrhosis, 2014, available at http://en.wikipedia.org/wiki/Primary_biliary_cirrhosis http://en.wikipedia.org/wiki/Primary_biliary_cirrhosis Primary Biliary Cirrhosis (PBC) - Topic Overview, 2012, available at http://www.webmd.com/digestive-disorders/tc/primary-biliary-cirrhosis- pbc-topic-overview http://www.webmd.com/digestive-disorders/tc/primary-biliary-cirrhosis- pbc-topic-overview PBC, 2011, American Liver Foundation, available at http://www.liverfoundation.org/abouttheliver/info/pbc/ http://www.liverfoundation.org/abouttheliver/info/pbc/ PBC, 2012, Canadian Liver Foundation, Available at http://www.liver.ca/liver-disease/types/primary-biliary-cirrhosis.aspx http://www.liver.ca/liver-disease/types/primary-biliary-cirrhosis.aspx