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Primary Biliary Cholangitis (PBC)

> women account for about 90 percent of cases. > often diagnosed between the age of 35 and 60. > chronic disease that slowly destroys the medium- sized bile ducts within the liver. (Bile is a digestive liquid made in the liver - it travels through bile ducts to the small intestine, where it helps digest fats and fatty vitamins). > in PBC, the bile ducts are destroyed by inflammation => bile remains in the liver, where gradual injury damages liver cells and causes cirrhosis, or scarring of the liver. > as cirrhosis progresses and scar tissue in the liver increases, liver function reduces. > advances slowly. Many patients lead active lives for > 15 years after diagnosis, and those with no symptoms at the time of diagnosis often remain symptom-free for years. > if left untreated, the persisting damage of bile ducts leads to increasing cholestasis with jaundice, pruritus and hypercholesterinemia --> biliary cirrhosis --> liver transplantation.

PBC has four stages: Stage 1 = inflammation and damage to the walls of medium-sized bile ducts. Stage 2 = blockage of small bile ducts. Stage 3 = beginning of scarring. Stage 4 = cirrhosis develops => permanent, severe scarring & liver damage. How is PBC diagnosed? > often no symptoms => discovered through abnormal routine liver blood tests. > diagnosis = [1] presence of antimitochondrial antibodies (AMA) in the blood, and [2] ultrasound exams and a liver biopsy may also be performed. Signs and Symptoms > often no symptoms- especially in the early stages. > first symptoms include fatigue (up to 80%), dry mouth/eyes, and itchy skin (arms, legs and back – up to 70%). > later symptoms include: [1] belly pain, [2] darkening of skin, [3] fatty deposits in the skin around eye, [4] nausea, [5] appetite and weight loss, [6] joint, muscle, or bone pain, [7] yellowing of the skin and eyes (jaundice), [8] swollen belly (fluid buildup), [9] swelling in the legs & ankles, [10] diarrhoea, and [11] fractures (weakened bones > jaundice is an indication of how far the disease has progressed. > other disorders can also occur, including [1] arthritis, [2] thyroid issues, [3] renal stones, and [4] gallstones. What causes PBC? > precise cause unknown. > considered autoimmune disease => your immune system mistakes tissue in your liver for a foreign invader and attacks it, damaging cells in the bile ducts. > unknown exactly what causes this immune system attack, but probably triggered by both genetic and environmental causes. > suspected environmental causes include several bacteria (UTIs - E. coli), viruses (EBV), toxins (nail polish, cigarettes, xenobiotics), and drugs (HRT). > geographic clustering: 200-251/million in South Wales and North East England, under 25/million in Canada and Australia, and almost zero in Sub-Saharan Africa and India. > more common among siblings and in families where one member is affected. > faulty mitochondria trigger oxidative stress and promote the presentation of mitochondrial antigens to the immune system.

Conventional Treatment options include: 1/ Urursodiol (urseodeoxycholic acid), a bile acid [1] improves liver function (helps move bile from the liver into the small intestine), [2] extends life expectancy, and [3] may delay the need for a liver transplant. Side effects include weight gain, diarrhoea, and hair loss. 2/ Obeticholic acid (Ocaliva) is a newer drug which lowers the amount of bile in the liver by reducing bile production and helping push bile out of the liver. Complications include: 1/ Progressive liver damage (cirrhosis) affects the liver’s ability to function. 2/ Portal hypertension - high pressure in the portal vein can cause fluid buildup in the abdomen, enlarged spleen, low platelet levels, and enlarged veins in the GI tract. 3/ Cholestasis (bile flow stops) - within the liver, causing intestinal and gallbladder issues. 4/ Bone disease - about 30% get mild changes in bone density (osteopenia). Another 10% experience significant bone density losses -> osteoporosis. 5/ Increases in blood lipids - 85% experience high cholesterol & triglyceride levels. 6/ Autoimmune disorders – up to 70% have another autoimmune disease, including Raynaud’s disease and keratoconjunctivitis sicca (dry eye). 7/ Gallstones - people with cirrhosis are twice as likely to have gallstones. 8/ Jaundice - bile does not flow out of the liver and into the intestine. Instead, it seeps into the bloodstream, causing a yellow hue in the eyes and skin. Your naturopathic nutritionist may advise: Reduce 1/ trans & saturated fats: cakes, pies, fried foods, sausages, bacon, cured meats, cheese. 2/ salt intake & ready meals/convenience foods - sodium is used to extend shelf life. 3/ alcohol and cigarettes (ideally stop altogether) 4/ stress Increase 1/ hydration – aim for 3L daily. 2/ increase fruit and vegetables – avocado, berries, celery, onions, kale, spinach and garlic. 3/ fish – especially salmon, mackerel, sardines, and herring. 4/ nuts, seeds and pulses. 5/ exercise, particularly walking Supplementation advice may include: Vitamins A, D, E, and K Calcium Magnesium Glutathione Milk Thistle Probiotics (Adapted from: [1] https://journals.lww.com/co-gastroenterology/Fulltext/2021/03000/Primary_biliary_cholangitis__pathogenic_mechanisms.5.aspx, [2] www.ncbi. nlm.nih.gov/books/NBK459209/, [3] https://bnf.nice.org.uk/treatment-summaries/primary-biliary-cholangitis/, [4] www.niddk.nih.gov /health-information/liver-disease/primary-biliary-cholangitis#:~:text=Primary%20biliary%20cholangitis%20is%20a,up%20and%20causes%20liver%20 damage, [5] https://gut.bmj.com/content/47/4/586, and [6] Articles in: www.liverfoundation.org; www.sciencedirect.com; and www.healthline.com)

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