Severe IgG4-Related Disease in a Young Child: A Diagnosis Ch
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Introduction
Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome of unknown aetiology comprised of a collection of disorders that share specific pathologic, serologic, and clinical features. It is characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and elevated serum IgG4 concentrations. The disease was initially recognized in the pancreas but has now been described in virtually every organ system: the biliary tree, salivary glands, periorbital tissues, kidney, lungs, lymph nodes, meninges, aorta, breast, prostate, thyroid, pericardium, and skin. The histopathological features bear striking similarities across the involved organs. However, some organs, like the lymph nodes, show a distinct involvement and histopathological features....

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4325228/
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