Although no clear etiology is known, 70% of these patients have inflammatory bowel disease, usually ulcerative colitis. There is a male to female preponderance in a 2:1 ratio and at an average age of 40 years. Other rarely associated disorders are Reidel's thyroiditis, histiocytosis X, sarcoidosis, and retroperitoneal fibrosis.

The initial presentation of patients with sclerosing cholangitis is usually painless jaundice, pruritus, recurrent cholangitis, fatigue, and weight loss. Some may be asymptomatic with the disease only suspected by laboratory data.

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