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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 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.
IgG4-RD: IgG4-related disease: IgG4-RKD: IgG4-related kidney disease IgG4-ROD: IgG4-related ophthalmic disease: IgG4-TIN: IgG4-related tubulointerstitial nephritis IgM: immunoglobulin M: IGT: impaired glucose tolerance: IHC: immunohistochemistry: IHD: ischaemic heart disease: IHPS: infantile hypertrophic pyloric stenosis IHSS
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 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.
IgG deficiency is a form of dysgammaglobulinemia where the proportional levels of the IgG isotype are reduced relative to other immunoglobulin isotypes.. IgG deficiency is often found in children as transient hypogammaglobulinemia of infancy, which may occur with or without additional decreases in IgA or IgM.
Chronic sclerosing sialadenitis is a chronic (long-lasting) inflammatory condition affecting the salivary gland.Relatively rare in occurrence, this condition is benign, but presents as hard, indurated and enlarged masses that are clinically indistinguishable from salivary gland neoplasms or tumors.
The water-accessible surface area of an IgG antibody. Immunoglobulin G (IgG) is a type of antibody.Representing approximately 75% of serum antibodies in humans, IgG is the most common type of antibody found in blood circulation. [1]
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