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IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material

Deshpande, Vikram
Sainani, Nisha I.
Chung, Raymond T.
Pratt, Daniel S.
Lauwers, Gregory Y.
Published in Modern Pathology. 2009, vol. 22, no. 10, p. 1287-1295
Abstract IgG4-associated cholangitis is a steroid-responsive hepatobiliary inflammatory condition associated with autoimmune pancreatitis that clinically and radiologically mimics primary sclerosing cholangitis. In this study, we conducted a morphological and immunohistochemical analysis of liver material obtained from individuals with IgG4-associated cholangitis, and compared these with well-characterized cases of primary sclerosing cholangitis. The study group consisted of 10 patients (9 biopsy and 1 hepatectomy case) with IgG4-associated cholangitis and 17 patients with primary sclerosing cholangitis (16 needle biopsy and 1 hepatectomy case). All patients with IgG4-associated cholangitis had pancreatic involvement as well, and six pancreatectomy samples revealed characteristic histopathological features of autoimmune pancreatitis. Primary sclerosing cholangitis cases were defined by the presence of a characteristic ERCP appearance. Clinical, pathological, radiological, and follow-up data were recorded for all cases. Portal and periportal inflammation was graded according to Ishak's guidelines. Immunohistochemical stains for IgG and IgG4 were performed. The cohort of patients with IgG4-associated cholangitis (mean age: 63 years) was older than individuals with primary sclerosing cholangitis (mean age: 44 years). Seven of these cases showed intrahepatic biliary strictures. IgG4-associated cholangitis liver samples showed higher portal (P=0.06) and lobular (P=0.009) inflammatory scores. Microscopic portal-based fibro-inflammatory nodules that were composed of fibroblasts, plasma cells, lymphocytes, and eosinophils were exclusively observed in five of the IgG4-associated cholangitis cases (50%). More than 10 IgG4-positive plasma cells per HPF (high power field) were observed in 6 of the IgG4-associated cholangitis cases (mean: 60, range: 0-140 per HPF), whereas all primary sclerosing cholangitis cases showed significantly lesser numbers (mean: 0.08, range: 0-1 per HPF). On a liver biopsy, the histological features of IgG4-associated cholangitis may be distinctive, and in conjunction with IgG4 immunohistochemical stain, may help distinguish this disease from primary sclerosing cholangitis.
Keywords AdultAgedAged, 80 and overAutoimmune Diseases/*complications/immunology/pathologyBiopsy, NeedleCholangiopancreatography, Magnetic ResonanceCholangitis/*immunology/pathology/therapyCholangitis, Sclerosing/*immunology/pathology/therapyDiagnosis, DifferentialFemaleHepatectomyHumansImmunoglobulin G/*bloodImmunohistochemistryImmunophenotypingLiver/*immunology/pathology/surgeryMaleMiddle AgedPancreatitis/*complications/immunology/pathologyPlasma Cells/immunologyPredictive Value of TestsSteroids/therapeutic use
PMID: 19633647
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Research groups Hépatologie chirurgicale (327)
Métastases du foie (657)
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DESHPANDE, Vikram et al. IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. In: Modern Pathology, 2009, vol. 22, n° 10, p. 1287-1295. doi: 10.1038/modpathol.2009.94 https://archive-ouverte.unige.ch/unige:19772

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Deposited on : 2012-04-23

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