AI Article Synopsis
- IgG4-related systemic disease (IRSD) is a newly recognized condition characterized by inflammation and fibrosis in multiple organs, as seen in a patient with 20 years of progressive symptoms.
- The patient exhibited elevated serum IgG4 levels and biopsy results showed strong positive immunostaining for IgG4, confirming the diagnosis.
- This case highlights the importance of early diagnosis and treatment, as IRSD is known to respond well to steroid therapy.
Article Abstract
IgG4-related systemic disease (IRSD) is a recently described entity with protean manifestations. We describe a patient who developed inflammation and fibrosis in multiple organs over 20 years, sequentially involving his pancreas, bile ducts, gallbladder, submandibular and lacrimal glands, and kidneys. He had an elevated serum IgG4 level. Retrospective analysis of biopsies showed strongly positive tissue immunostaining for IgG4, confirming the diagnosis of IRSD. This case illustrates the natural history of partially treated IRSD and its varied clinical presentations. Early diagnosis and treatment is important, as the condition is highly steroid-responsive.
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| http://dx.doi.org/10.5694/j.1326-5377.2010.tb03974.x | DOI Listing |
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