2017
DOI: 10.1097/md.0000000000006542
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Case report

Abstract: Rationale:IgG4-related disease is a newly recognized fibroinflammatory disorder, characterized by tumefactive lesions, storiform fibrosis and IgG4-positive plasma cells infiltration. IgG4-related sclerosing cholangitis (IgG4-SC) is the most common extrapancreatic manifestation of IgG4-related disease, but it is frequently associated with autoimmune pancreatitis(AIP). Only few case was reported to be diagnosed with IgG4-SC in the absence of AIP, with a striking male preponderance. Here we report a female case o… Show more

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Cited by 9 publications
(6 citation statements)
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“…IRSC responses well to the steroids treatment and should not be treated by operation. Sometimes, differential diagnosis of IRSC and MOJ can be very difficult 8 , 9 . The differential diagnostic roles of the serum CA19-9 has not been reported between IRSC and MOJ.…”
Section: Introductionmentioning
confidence: 99%
“…IRSC responses well to the steroids treatment and should not be treated by operation. Sometimes, differential diagnosis of IRSC and MOJ can be very difficult 8 , 9 . The differential diagnostic roles of the serum CA19-9 has not been reported between IRSC and MOJ.…”
Section: Introductionmentioning
confidence: 99%
“…Various case reports with misdiagnosed IgG4-SC show different data regarding the effects of surgery and/or medical treatment [13], [16], [29], [32]. Some showed no recurrence after surgery without steroid therapy [29], some showed no improvement at all after resection plus steroids [13] as it was the situation in the herein reported patient. In another case, reported by Miki et al [16], hilar cholangiocarcinoma could not have been ruled out even though serum IgG4 level was elevated.…”
Section: Discussionmentioning
confidence: 77%
“…So most likely, the preoperative serum level was equally as high as the postoperative one. However, there are other cases were the IgG4 serum level was not measured [29] or the level was in the normal range [14]. Because of the clinical rarity and poor recognition of IgG4-SC, it is not surprising that measuring the serum IgG4 level could be missed, especially without manifestation of the extra-biliary organs.…”
Section: Discussionmentioning
confidence: 99%
“…Clinically, an early diagnosis of IgG4-RD remains a challenge, owing to its symptoms and pathology mimicking several other diseases such as infections and malignancies, even if the IgG4-RD diagnostic criteria are followed[ 10 ]. For example, clinically, IgG4-RD often presents with multifocal tumor-like masses that leads to confusions with malignancy.…”
Section: Discussionmentioning
confidence: 99%