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IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity.

Published on Sep 1, 2010in Advances in Anatomic Pathology3.53
· DOI :10.1097/PAP.0b013e3181ee63ce
Wah Cheuk30
Estimated H-index: 30
,
John K. C. Chan81
Estimated H-index: 81
Abstract
An elevated serum titer of immunoglobulin G4 (IgG4), the least common (3% to 6%) of the 4 subclasses of IgG, is a surrogate marker for the recently characterized IgG4-related sclerosing disease. The syndrome affects predominantly middle-aged and elderly patients, with male predominance. The patients
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Background & Aims Autoimmune pancreatitis (AIP) has been divided into subtypes 1 (lymphoplasmacytic sclerosing pancreatitis) and 2 (idiopathic duct centric pancreatitis). We compared clinical profiles and long-term outcomes of types 1 and 2 AIP. Methods We compared clinical presentation, relapse, and vital status of 78 patients with type 1 AIP who met the original HISORt criteria and 19 patients with histologically confirmed type 2 AIP. Results At presentation, patients with type 1 AIP were olde...
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#1John K. ChanH-Index: 31
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A 70-year old man with a 14 year history of Sjogren syndrome, interstitial pneumonia, and autoimmune hepatitis (AIH) was admitted to our hospital due to hyponatremia with a one month history of fatigue, thirst, and nausea. Laboratory tests on admission revealed that this patient had a central adrenal insufficiency. Pituitary magnetic resonance imaging (MRI) further showed swelling of the stalk and posterior lobe of his pituitary, suggesting infundibulo-hypophysitis. Based on his past history of ...
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IgG4-related diseases comprise a recently recognized systemic syndrome characterized by mass-forming lesions in mainly exocrine tissue that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4-positive plasma cells in the affected tissues, and the serum IgG4 level is increased in these patients. The present study describes the history, autoimmune pancreatitis (AIP), IgG4-related lymphadenopathy and lymphomagenesis based upon ocular adnexal IgG4-related disease. Lymphop...
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We present a case of a 23-year-old gentleman who presented with dysphagia, weight loss, and recurrent esophageal strictures requiring multiple dilatations. An endoscopic ultrasound with esophagogastroduodenoscopy revealed a mass present in the distal esophagus. Fine needle aspiration suggested that the mass in the lower esophagus resembled a gastrointestinal stromal tumor. After surgical resection, final pathologic analysis revealed that the tumor was comprised of benign-appearing fibroinflammat...
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