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IgG4-related systemic disease of the pancreas with involvement of the lung: a case report and literature review

By Hurley JR and Leslie KO


IgG-related systemic disease (ISD) remains exceedingly rare and unfamiliar, particularly extrapancreatic disease. We report a patient with separate presentations of IgG4 pulmonary disease and recurring IgG4 related biliary sclerosis and pancreatitis. Because of the intricate and perplexing pathogenesis, overlapping organ systems and wide variation in disease presentation, ISD in its entirety remains undefined. Accurate identification of ISD is critical to avoid permanent organ damage especially since treatment is nearly always successful with corticosteroids. As recognition and awareness of this disease grows, development of standard diagnostic criteria and treatment plans are needed

Topics: IgG4-related systemic disease, pancreas, biliary sclerosis, pancreatitis, hypergammaglobulinemia, corticosteroid, interstitial lung disease, IgG4, Medical emergencies. Critical care. Intensive care. First aid, RC86-88.9, Internal medicine, RC31-1245, Medicine, R, DOAJ:Internal medicine, DOAJ:Medicine (General), DOAJ:Health Sciences, General works, R5-130.5
Publisher: Arizona Thoracic Society
Year: 2013
OAI identifier: oai:doaj.org/article:158e62abf1c645509d689305e1cd6126
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  • http://dx.doi.org/10.13175/swj... (external link)
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