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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.
However, surgery is not a good treatment for this condition as AIP responds well to immunosuppressive treatment. There are two categories of AIP: Type 1 and Type 2, each with distinct clinical profiles. 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 ...
It is now believed that Riedel's thyroiditis is one manifestation of a systemic disease that can affect many organ systems called IgG4-related disease. It is often a multi-organ disease affecting pancreas, liver, kidney, salivary and orbital tissues and retroperitoneal space. The hallmarks of the disease are fibrosis and infiltration by IgG4 ...
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.
Existing treatment [ edit ] Standard, and most effective, therapy to date is glandular sialoadenectomy, which is associated with fairly low operative morbidity; however, in recent times, the administration of steroid (which can shrink the inflammatory lesion and is known to reduce serum IgG4 values) has been considered favorably, [ 16 ] [ 17 ...
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. ...
The typical treatment for plasma cell granuloma is a complete surgical excision of the lesional mass. [ 3 ] [ 4 ] Generally when the mass is removed, patients will display an absolute reversal of symptoms. [ 3 ]
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.