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I g G 4‐related disease
Author(s) -
Kubo Kanae,
Yamamoto Kazuhiko
Publication year - 2016
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.12586
Subject(s) - igg4 related disease , medicine , autoimmune pancreatitis , retroperitoneal fibrosis , pathological , pathology , fibrosis , disease , pathogenesis , thyroiditis , etiology , immunology
Abstract Immunoglobulin G 4‐related disease ( I g G 4‐ RD ) is a regional or systemic fibro‐inflammatory disease of unknown etiology. It presents a distinctive histopathological appearance of dense lymphoplasmacytic infiltrates with abundant I g G 4‐positive plasma cells, storiform fibrosis and obliterative phlebitis with the appearance of inflammatory swelling or tumefactive lesions. This new disease entity includes a wide variety of diseases such as M ikulicz disease, autoimmune pancreatitis, R iedel thyroiditis, interstitial nephritis and retroperitoneal fibrosis. G lucocorticoid therapy can resolve the clinical and pathological abnormalities and impaired organ function. I g G 4‐ RD was recognized internationally in 2011, and new evidence has accumulated regarding its pathogenesis, clinical features and treatment. In this review, we outline our present understanding of I g G 4‐ RD .