ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES IN PRIMARY SCLEROSING CHOLANGITIS - DEFINED SPECIFICITIES MAY BE ASSOCIATED WITH DISTINCT CLINICAL-FEATURES

PURPOSE: The clinical significance of antineutrophil cytoplasmic autoa ntibodies (ANCA) in primary sclerosing cholangitis has not been establ ished. We investigated whether analysis of the antigenic specificities of ANCA is useful for delineating clinical subsets of the disease. ME THODS: Sixty-nine patients with primary sclerosing cholangitis were st udied. The presence of ANCA was analyzed by indirect immunofluorescenc e. Antibodies directed against specific antigens-proteinase 3, myelope roxidase, elastase, bactericidal/permeability-increasing protein, cath epsin G, and lactoferrin-were identified by enzyme-linked immunosorben t assay. RESULTS: ANCA were detected by indirect immunofluorescence in 46 (67%) patients. In antigen-specific enzyme-linked immunosorbent as says, 37 (55%) of the 69 patients had antibodies to one or more antige ns: 32 (46%) had antibodies to bactericidal/permeability-increasing pr otein, 16 (23%) had antibodies to cathepsin G, and 15 (22%) had antibo dies to lactoferrin. Only 3 patients had antibodies to proteinase 3. A ntibodies to myeloperoxidase or elastase were not detected. Twenty (29 %) patients had antibodies to different antigens simultaneously. ANCA as detected by indirect immunofluorescence were not significantly asso ciated with the presence of cirrhosis nor with the coexistence of infl ammatory bowel disease. However, antibodies to bactericidal/permeabili ty-increasing protein and cathepsin G were both associated with the pr esence of cirrhosis, and antibodies to lactoferrin were more frequentl y detected in patients with primary sclerosing cholangitis in conjunct ion with ulcerative colitis than in those without inflammatory bowel d isease. CONCLUSION: Defined specificities of ANCA in primary sclerosin g cholangitis may be related to particular clinical features of the di sease. (C) 1998 by Excerpta Medica, Inc.