OBJECTIVE: To evaluate the prognostic value of peri-nuclear Anti- Neutrophil Cytoplasmic Antibodies (p-ANCA) in subjects with primary sclerosing cholangitis (PSC) and in particular whether it is associated with inflammatory bowel disease (IBD).
PATIENTS AND METHODS: In the last 9 years,64 patientswere admitted to our Hospital with PSC: 34 males, mean age 53 years, range 17-90. All the patients had a cholestatic pattern of elevated serum enzymes. The diagnosis was made in most cases with magnetic resonance and in small percentages with endoscopic retrograde cholangiopancreatography (ERCP) or liver biopsy. In 33 patients with PSC, colonoscopy and p-ANCA assays were possible.
RESULTS: Thirteen patients showed p-ANCA positivity: 8 had ulcerative colitis (UC), with associated colon cancer (CC) in 3 cases, 3 had Crohn's disease (CD), 2 had no inflammatory bowel disease (IBD). Twenty patients were p-ANCA negative: only 1 with indeterminate IBD, while 19 had no IBD.
CONCLUSIONS: IBD, or especially UC, is present in85% of patients with CSP and p-ANCA positivity, whereas 95% of patients with PSC who test negative for p- ANCA do not have IBD.