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• W2149493996 abstract "We read with great interest the article by Björnsson et al on the natural history of small duct primary sclerosing cholangitis (PSC).1Björnsson E. Olsson R. Bergquist A. et al.The natural history of small-duct primary sclerosing cholangitis.Gastroenterology. 2008; 134: 975-980Abstract Full Text Full Text PDF PubMed Scopus (198) Google Scholar It is shown that small duct PSC has a better long-term prognosis as compared with large duct PSC. Cholangiocarcinoma does not seem to occur in patients with small duct PSC, in contrast with patients with large duct PSC. Therefore, it is important to differentiate these entities. We wondered whether the autoantibody profile in patients with small and large duct PSC differs. In large duct PSC, 60%–90% of patients test positive for an atypical perinuclear antineutrophil cytoplasmic antibody (P-ANCA).2Duerr R.H. Targan S.R. Landers C.J. et al.Neutrophil cytoplasmic antibodies: a link between primary sclerosing cholangitis and ulcerative colitis.Gastroenterology. 1991; 100: 1385-1391PubMed Google Scholar, 3Tervaert J.W. Mulder A.H. Horst G. et al.Antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis, ulcerative colitis, and autoimmune diseases.Gastroenterology. 1992; 102: 1090-1091PubMed Google Scholar, 4Roozendaal C. Van Milligen de Wit A.W.M. Haagsma E.B. et al.Antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis: defined specificities may be associated with distinct clinical features.Am J Med. 1998; 105: 393-399Abstract Full Text Full Text PDF PubMed Scopus (54) Google Scholar, 5Bogdanos D.P. Invernizzi P. Mackay I.R. et al.Autoimmune liver serology: current diagnostic and clinical challenges.World J Gastroenterol. 2008; 14: 3374-3387Crossref PubMed Scopus (157) Google Scholar Whether P-ANCA is also present in small duct PSC is not yet studied. We report herein a patient with small duct PSC who proved to be P-ANCA positive. A 16-year-old girl presented to the outpatient hepatology clinic with fatigue and pruritus and elevated liver enzymes: alanine aminotransferase, 307 U/L (normal, 35); aspartate aminotransferase, 184 U/L (normal, 30); γ-glutamyl transpeptidase, 316 U/L (normal, 35); and alkaline phosphatase, 427 U/L (normal, 140). Bilirubin levels were normal. P-ANCA (1/256) and antinuclear antibodies were strongly positive. Anti-liver kidney microsomes 1 antibodies, anti-mitochondria antibodies, and smooth muscle antibodies were negative. Viral, metabolic, and toxic liver diseases were excluded. Magnetic resonance cholangiopancreatography was normal, but a liver biopsy showed signs of chronic biliary pathology with little inflammation suggestive of small duct PSC. The diagnosis of small duct PSC was made and ursodeoxycholic acid was started. After 1 year, the patient deteriorated and an PSC/autoimmune hepatitis overlap syndrome6Gregorio G.V. Portmann B. Karani J. et al.Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study.Hepatology. 2001; 33: 544-553Crossref PubMed Scopus (526) Google Scholar, 7Invernizzi C. Beuers U. Overlap syndromes among autoimmune liver diseases.World J Gastroenterol. 2008; 14: 3368-3373Crossref PubMed Scopus (9) Google Scholar was suspected. Therefore, treatment with prednisolone and azathioprine was initiated, but was stopped after 2 years because therapy was not effective with significant side effects. Ursodeoxycholic acid was continued. At present, minor liver test abnormalities persist (<2 times the upper limit of normal). Antinuclear antibodies became negative, whereas atypical P-ANCA is persistently positive. Our patient had a small duct PSC and tested positive for P-ANCA. Whether P-ANCA positivity is found in small duct PSC patients has not been reported.1Björnsson E. Olsson R. Bergquist A. et al.The natural history of small-duct primary sclerosing cholangitis.Gastroenterology. 2008; 134: 975-980Abstract Full Text Full Text PDF PubMed Scopus (198) Google Scholar, 8Angulo P. Maor-Kendler Y. Lindor K.D. Small-duct primary sclerosing cholangitis: a long-term follow-up study.Hepatology. 2002; 35: 1494-1500Crossref PubMed Scopus (179) Google Scholar, 9Broomé U. Glaumann H. Lindström E. et al.Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC).J Hepatol. 2002; 36: 586-589Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar, 10Nikolaidis N.L. Giouleme O.I. Tziomalos K.A. et al.Small-duct primary sclerosing cholangitis A single-center seven-year experience.Dig Dis Sci. 2005; 50: 324-326Crossref PubMed Scopus (29) Google Scholar Because autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseases,5Bogdanos D.P. Invernizzi P. Mackay I.R. et al.Autoimmune liver serology: current diagnostic and clinical challenges.World J Gastroenterol. 2008; 14: 3374-3387Crossref PubMed Scopus (157) Google Scholar we think that more studies are needed to investigate whether P-ANCA could be used as a marker antibody for small duct PSC. ReplyGastroenterologyVol. 136Issue 1PreviewWe thank Tervaert et al for their interest in our recent paper published in Gastroenterology.1 Unfortunately, no information on atypical perinuclear antineutrophilic cytoplasmic antibodies (P-ANCA) was available in our series.1 The same is true for other previously published reports on patients with small duct primary sclerosing cholangitis (PSC).2–6 Presence of multiple autoantibodies is frequent in patients with classic, large duct PSC.7 Although 60%–90% of patients with classic PSC test positive for P-ANCA, neither the pathogenetic or the diagnostic role of P-ANCA in PSC has been clarified. Full-Text PDF" @default.
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• W2149493996 title "Antineutrophil Cytoplasmic Antibodies in Small-Duct Primary Sclerosing Cholangitis" @default.
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