Immunoglobulin G4–Associated Cholangitis: Clinical Profile and Response to Therapy

Ghazale, Amaar; Chari, Suresh T.; Zhang, Lizhi; Smyrk, Thomas C.; Takahashi, Naoki; Levy, Michael J.; Topazian, Mark D.; Clain, Jonathan E.; Pearson, Randall K.; Petersen, Bret T.; Vege, Santhi Swaroop; Lindor, Keith D.; Farnell, Michael B.

doi:10.1053/j.gastro.2007.12.009

Cited by 826 publications

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“…11 All lesions incorporated into the spectrum of the disease, including the pancreatic manifestations are characterized by a plasma cell-rich, often mass-forming inflammatory process with numerous IgG4-positive plasma cells. 11 In a recent publication, Ghazale et al 6 proposed the term 'IgG4-associated cholangitis' to describe the biliary manifestation of autoimmune pancreatitis. Although we acknowledge that this new terminology is not universally accepted, our findings and those of previous investigators support this nomenclature.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with IgG4-associated cholangitis are generally older men (85%), presenting with obstructive jaundice (77%) and autoimmune pancreatitis (92%). 6 On imaging, the biliary strictures are confined to the intrapancreatic bile duct in 51% of the cases, but the proximal extrahepatic bile and intrahepatic branches are involved in 49% of the cases. 6 Importantly, in one series, the majority of strictures responded to steroids, although relapses were common after withdrawal.…”

Section: Discussionmentioning

confidence: 99%

“…6 On imaging, the biliary strictures are confined to the intrapancreatic bile duct in 51% of the cases, but the proximal extrahepatic bile and intrahepatic branches are involved in 49% of the cases. 6 Importantly, in one series, the majority of strictures responded to steroids, although relapses were common after withdrawal. 6 However, although it has been reported by some that imaging studies may help segregate IgG4-associated cholangitis from primary sclerosing cholangitis, in our experience we were unable to make this distinction.…”

Section: Discussionmentioning

confidence: 99%

“…[3][4][5] Among these, autoimmune pancreatitis-associated sclerosing cholangitis has recently received significant attention, in part, because of the steroid-responsive nature of this disease. 4,6,7 Similar to manifestations of IgG4-associated disease, autoimmune pancreatitis-associated sclerosing cholangitis shows increased numbers of IgG4-positive plasma cells, justifying the recently suggested nomenclature of IgG4-associated cholangitis. 6 As IgG4-associated cholangitis is a steroid-responsive process, whereas primary sclerosing cholangitis is typically steroid resistant, it is thus vital for management to distinguish IgG4-associated cholangitis from primary sclerosing cholangitis.…”

mentioning

confidence: 91%

“…4,6,7 Similar to manifestations of IgG4-associated disease, autoimmune pancreatitis-associated sclerosing cholangitis shows increased numbers of IgG4-positive plasma cells, justifying the recently suggested nomenclature of IgG4-associated cholangitis. 6 As IgG4-associated cholangitis is a steroid-responsive process, whereas primary sclerosing cholangitis is typically steroid resistant, it is thus vital for management to distinguish IgG4-associated cholangitis from primary sclerosing cholangitis. Consequently, our goal was to determine whether this distinction could be made on a liver biopsy.…”

mentioning

confidence: 91%

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

et al. 2009

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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 ¼ 0.06) and lobular (P ¼ 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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%