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IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and a usually prompt response to oral steroids.
IgG4-related autoimmune diseases are characterized by excessive fibrosis. In case of Riedel's thyroiditis, fibrosis extends beyond the capsule and involves contiguous neck structures, clinically simulating thyroid carcinoma. There is a rapid thyroid enlargement. Compression of trachea, dysphagia are probable outcomes.
Although a clear understanding of the various skin lesions in IgG4-related disease is a work in progress, skin lesions have been classified into subtypes based on documented cases: [2] Angiolymphoid hyperplasia with eosinophilia (or lesions that mimic it) [3] and cutaneous pseudolymphoma; Cutaneous plasmacytosis [Note 1]
IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease, [2] which is characterised by infiltration of lymphocytes and plasma cells and subsequent fibrosis in involved structures. It can involve one or more of the orbital structures.
The diagnosis of IgG4-related prostatitis could be made from histological examination if prostate biopsy or surgery has been performed. [6] The hallmark histopathological features of established IgG4-related disease are storiform fibrosis, a dense lymphoplasmacytic (lymphocytes and plasma cells) infiltrate rich in IgG4-positive plasma cells, and obliterative phlebitis.
Pages in category "IgG4-related disease" The following 25 pages are in this category, out of 25 total. This list may not reflect recent changes. ...
Health authorities around the world are still grappling with lessons learned at the start of the Covid-19 pandemic and trying to determine the best way to prevent a new one.. Many researchers have ...
Type 1 AIP is now regarded as a manifestation of IgG4-related disease, [2] and those affected have tended to be older and to have a high relapse rate. Type 1 pancreatitis, is as such as manifestation of IgG4 disease, which may also affect bile ducts in the liver, salivary glands, kidneys and lymph nodes.