Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
2009-9-30
pubmed:abstractText
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
1530-0285
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
22
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1287-95
pubmed:meshHeading
pubmed-meshheading:19633647-Adult, pubmed-meshheading:19633647-Aged, pubmed-meshheading:19633647-Aged, 80 and over, pubmed-meshheading:19633647-Autoimmune Diseases, pubmed-meshheading:19633647-Biopsy, Needle, pubmed-meshheading:19633647-Cholangiopancreatography, Magnetic Resonance, pubmed-meshheading:19633647-Cholangitis, pubmed-meshheading:19633647-Cholangitis, Sclerosing, pubmed-meshheading:19633647-Diagnosis, Differential, pubmed-meshheading:19633647-Female, pubmed-meshheading:19633647-Hepatectomy, pubmed-meshheading:19633647-Humans, pubmed-meshheading:19633647-Immunoglobulin G, pubmed-meshheading:19633647-Immunohistochemistry, pubmed-meshheading:19633647-Immunophenotyping, pubmed-meshheading:19633647-Liver, pubmed-meshheading:19633647-Male, pubmed-meshheading:19633647-Middle Aged, pubmed-meshheading:19633647-Pancreatitis, pubmed-meshheading:19633647-Plasma Cells, pubmed-meshheading:19633647-Predictive Value of Tests, pubmed-meshheading:19633647-Steroids
pubmed:year
2009
pubmed:articleTitle
IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material.
pubmed:affiliation
The James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street,Boston, MA 02114, USA. VDESHPANDE@PARTNERS.ORG
pubmed:publicationType
Journal Article, Comparative Study