$Unique_ID{BRK03593} $Pretitle{} $Title{Cholangitis, Primary Sclerosing} $Subject{Cholangitis, Primary Sclerosing Stenosing Cholangitis Fibrosing Cholangitis Chronic Obliterative Cholangitis Primary Biliary Cirrhosis} $Volume{} $Log{} Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc. 418: Cholangitis, Primary Sclerosing ** IMPORTANT ** It is possible the main title of the article (Primary Sclerosing Cholangitis) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Stenosing Cholangitis Fibrosing Cholangitis Chronic Obliterative Cholangitis Information on the following disorder can be found in the Related Disorders section of this report: Primary Biliary Cirrhosis General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Primary Sclerosing Cholangitis is a rare collagen disorder involving inflammation and blockage of the bile duct, liver ducts and gallbladder. Episodes of pain and discomfort of the right upper section of the abdomen may gradually become prolonged. This disorder predominately affects males. Symptoms The initial pain and discomfort of Primary Sclerosing Cholangitis affects the upper right section of the abdomen and is associated with inflammation of the common bile ducts. Episodes of pain gradually become prolonged. Loss of appetite, nausea, or vomiting may occur, accompanied by weight loss. The liver may become enlarged and noticeably sensitive. When collagen tissue increases, an irregular pattern of thickening in the walls of the common bile ducts blocks the transport of bile. When bile is blocked, it may be absorbed into the blood in abnormal amounts causing a yellow discoloration of the skin (jaundice) accompanied by chills, fever, or itching of the skin. Fat soluble vitamins are poorly absorbed from the intestine. Blockages often associated with inflammation or infection can also occur in the gall bladder and the liver. Bacterial infections resulting from ulcerative colitis, Crohn's Disease, retroperitoneal fibrosis, generalized vasculitis, thyroiditis, or orbital pseudotumors may be associated with bile duct blockages of Primary Sclerosing Cholangitis. For more information, choose "ulcerative colitis" and "Crohn" as your search terms in the Rare Disease Database. Causes The exact cause of Primary Sclerosing Cholangitis is not known. Researchers believe an allergic or hypersensitivity reaction or a widespread disturbance of collagen-containing tissues may be responsible for symptoms. Affected Population Primary Sclerosing Cholangitis is a rare disorder predominately affecting middle-aged males, although it may affect males of any age. Related Disorders Symptoms of the following disorders can be similar to Primary Sclerosing Cholangitis. Comparisons may be useful for a differential diagnosis: Primary Biliary Cirrhosis, also known as Hanot's Cirrhosis, is a rare liver condition occurring mainly in females. It is characterized by yellow discoloration of the skin (jaundice) associated with obstruction and inflammation of the large bile ducts which normally transport bile. Additionally, abnormal cells accumulate in the liver associated with degenerative liver disease (cirrhosis). Blockage may not occur in small bile ducts. Some long-term severe cases of Primary Sclerosing Cholangitis may develop into Primary Biliary Cirrhosis. (For more information on this disorder, choose "Primary Biliary Cirrhosis" as your search term in the Rare Disease Database. Therapies: Standard Treatment of Primary Sclerosing Cholangitis may benefit from imaging methods such as X-ray or CAT Scan to identify the exact location and nature of blockages. Surgery to remove blockages and enlarge narrowed bile ducts may be of benefit to help prevent liver deterioration. Lost vitamins should be replaced when required to prevent complications related to these deficiencies. Antibiotics may be useful in controlling inflammation or infection. The drug cholestyramine may be effective in controlling itching. Other treatment is symptomatic and supportive. Therapies: Investigational Liver transplants for experimental treatment of the most severe cases of Primary Sclerosing Cholangitis are being considered. Other treatments being tested include drainage of blocked bile through tubes inserted in ducts and enlarging abnormally narrowed bile ducts (at least temporarily) by inserting a tiny balloon inside the duct and inflating it (endoscopic balloon dilation). Effectiveness and side effects of these procedures and devices have not been fully documented and more extensive research is being pursued before their therapeutic value for Primary Sclerosing Cholangitis can be evaluated. This disease entry is based upon medical information available through December 1988. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Primary Sclerosing Cholangitis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Digestive Diseases Information Clearinghouse Box NDIC Bethesda, MD 20892 (301) 468-2162 American Liver Foundation 998 Pomptom Avenue Cedar Grove, NJ 07009 (201) 857-2626 (800) 223-0179 The United Liver Foundation 11646 West Pico Blvd. Los Angeles, CA 90064 (213) 445-4204 or 445-4200 Children's Liver Foundation 14245 Ventura Blvd. Sherman Oaks, CA 91423 (818) 906-3021 References PRIMARY SCLEROSING CHOLANGITIS: J.J. Alberti-Flor, et al.; South Med J (February 1985, issue 78(2)). Pp. 173-177. DIAGNOSIS AND TREATMENT OF PRIMARY SCLEROSING CHOLANGITIS: R.H. Wiesner, et al.; Semin Liver Dis (August 1985, issue 5(3)). Pp. 241-253. THERAPEUTIC INVESTIGATIONS IN PRIMARY SCLEROSING CHOLANGITIS: R. Grijm, et al.; Dig Dis Sci (August 1986, issue 31(8)). Pp. 792-798.