ANTINEUTROPHIL CYTOPLASMIC ANTIBODY - A PROGNOSTIC INDICATOR IN PRIMARY SCLEROSING CHOLANGITIS

Considerable variability has been reported in the frequency and specif icity of anti-neutrophil cytoplasmic antibody with a perinuclear stain ing pattern (pANCA) in patients with chronic liver disease, especially in primary sclerosing cholangitis (PSC), and in inflammatory bowel di sease. This study examines the presence of pANCA in patients with thes e disorders, in particular those with PSC complicated by other biliary disease, and also patients who had undergone orthotopic liver transpl antation. An indirect immunofluorescent technique was used to measure pANCA with serum diluted 1 : 20. Ten of 39 (26%) patients with PSC had detectable pANCA, as did two of nine (22%) with autoimmune chronic ac tive hepatitis (AICAH) but none of the 51 patients with other forms of chronic liver disease. The presence of pANCA was significantly more f requent in patients who had PSC with biliary tract complications, in p articular calculi (seven of 16 with vs three of 23 without; P = 0.03). Eight of the 12 pANCA-positive patients with PSC or AICAH had undergo ne hepatic transplantation. This was more likely than in patients with PSC or AICAH who were pANCA negative (10 of 36; P = 0.02). To date, p ANCA has been detected after transplantation in four patients with PSC and one with AICAH, In patients with PSC or AICAH, pANCA should be so ught as a marker of prognosis.