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In approximately 51–70% of people with this disease, serum IgG4 concentrations are elevated during an acute phase. [ 1 ] [ 2 ] [ 3 ] It is a relapsing-remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved.
A ratio above 40 percent indicates abnormal elevated levels of IgG4 positive plasma cells. [3] Even though IgG levels can be measured in serum, histopathological analysis has been concluded to be the most accurate characterization method of PCG lesions. [3]
Increased serum levels of gamma globulins, immunoglobulin G (IgG) or IgG4. The presence of serum autoantibodies such as anti-nuclear antibody (ANA), anti-lactoferrin antibody, anti-carbonic anhydrase II antibody, and rheumatoid factor (RF). Contrast-enhanced CT demonstrates a diffusely enlarged (sausage-shaped) pancreas.
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.
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 ] IgG molecules are created and released by plasma B cells .
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.
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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.