Liver diseases can range from mild fatty liver disease to complete decompensation and loss of liver function, Some are acquired by virus, some due to diet and alcohol use, some secondary from other body illnesses, and some are primary arising directly in the liver and bile ducts. Two such primary diseases of a very serious nature are primary sclerosing cholangitis and primary biliary cirrhosis.
Primary sclerosing cholangitis (PSC) is a somewhat uncommon disease that is characterized by a diffuse inflammation of the bile tract in the liver, leading to fibrosis, hardening and strictures of those tracts. It is more common in men than in women, mostly from the ages of 20 to 50. The disease can be associated with ulcerative colitis (a much more common gastrointestinal disease) and, in fact, nearly two thirds of people with PSC have ulcerative colitis. It is somewhat genetic in origin, as PSC is associated with several genetic loci that are identifiable. It also occurs after biliary surgery in some cases.
Most PSC patients have obstructive jaundice, which is progressive. Before symptoms, an elevated blood alkaline phosphatase of several times normal may be the first indication of disease. Late in the course, osteoporosis, malabsorption, and bleeding veins in the esophagus are ominous signs. The diagnosis is made by a procedure called magnetic resonance cholangiography, which is like an MRI of the bile ducts and structures. When smaller rather than larger bile ducts are involved, the life expectancy is longer. Treatments may include antibiotics when infection coexists, stents to bypass the closing ducts, and liver transplant when the disease has a more relentless course. Survival from the time of diagnosis averages about 15 years, but can be much shorter when a cancer called cholangiocarcinoma (occurring in as many as 20 percent of cases) is diagnosed.
Primary biliary cirrhosis (PBC) is a chronic disease of the liver where there is autoimmune destruction of liver bile ducts. It is slow in onset and mainly affects females ages 40 to 60. Like PSC, the first finding is an increase in blood alkaline phosphatase and, also like PSC, it is associated genetically with certain definable loci. Many times PBC is associated with other autoimmune diseases such as Sjogren syndrome, thyroid disease and Raynaud’s.
An enlarged liver is the first finding, followed by skin lesions, jaundice, and in late stages cognitive dysfunction. Blood tests may diagnose the disease, but a liver biopsy allows quantifying how far the disease has progressed. Survival in PBC generally averages between seven to 10 years, but a medication called ursodeoxycholic acid helps those who are younger live longer. Liver transplantation is again a necessary therapy, but disease may recur in the transplanted liver as well.
Both primary sclerosing cholangitis and primary biliary cirrhosis are progressive and often unremitting diseases. If the diagnosis is a proven one, generally even earlier stage patients are not eligible for life insurance coverage. Those with a successful liver transplant and a healthy course without disease progression for years afterwards are eligible for individual consideration, but no known medical therapies at this time have been able to cure these diseases.
