Abstract
IgG4-related disease (IgG4-RD) is a newly discovered clinical disease entity that may be present in patients with a wide variety of conditions, previously diagnosed as Mikulicz disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, tubulointerstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, inflammatory pseudotumor, and others. IgG4-RD is commonly characterized by tumefaction of affected organs, elevated serum IgG4 concentration, and tissue infiltration by IgG4-positive plasma cells. IgG4-RD has been observed most frequently in middle-aged to elderly men, and its clinical symptoms are usually relatively mild. Histopathologic findings include marked IgG4-positive cell infiltration with an IgG4/IgG cell ratio >40%. In some organs, storiform or swirling fibrosis and obliterative phlebitis are characteristic and important findings in diagnosing IgG4-RD. Many patients with IgG4-RD can be treated effectively by systemic glucocorticoid administration, but 25% to 50% of them relapse on low maintenance dose or after discontinuation of glucocorticoids.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
