primary sclerosing cholangitis / primary biliary cirrhosis

Introduction

primary sclerosing cholangitis (PSC) is a rare condition which is mainly associated with patients with inflammatory bowel disease (IBD) and usually is diagnosed when persistently high AP levels are found as it is usually asymptomatic in the early phases
previously known as primary biliary sclerosis

secondary causes of sclerosing cholangitis excluded, and at least two of:
- serum alkaline phosphatase (ALP) > 1.5x the upper limit of normal for longer than 6 months
- cholangiography demonstrating biliary strictures or irregularity consistent with PSC
- +/- liver biopsy consistent with PSC

can affect men and women at any age but 2-3x more common in males, although it is commonly diagnosed in the fourth decade of life (30-50 yr olds)
1-16 cases per 100,000 population in the United States or Europe
75% have inflammatory bowel disease (IBD) - usually ulcerative colitis
3-7% of patients with ulcerative colitis develop primary sclerosing cholangitis
increased risk if family member has it
other disease associations:
- sarcoidosis, thyroid disease, Peyronie’s disease, retroperitoneal fibrosis, psoriasis, rheumatoid arthritis, Celiac disease, Sjogren’s syndrome, chronic pancreatitis, lupus, diabetes mellitus, Wegener’s granulomatosis, pyoderma gangrenosum, Grave’s disease, Langerhans cell histiocytosis and certain immunodeficiency disorders ¹⁾

ascending cholangitis risk and patients often have multiple life threatening episodes
cirrhosis
- liver failure may occur 10-15 yrs after diagnosis
- pruritus
- malabsorption
- portal hypertension
hepatocellular carcinoma
gallbladder and bile duct tumours (occurs in 8-15% of pts)
colorectal cancer (bowel cancer)

no Rx is available to manage the primary sclerosing cholangitis itself
supportive care - antipruritics, dietary supps
liver transplant may be an option and is often needed within 10 years of diagnosis

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