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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.
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 ...
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.
Multifocal fibrosclerosis's etiology is unknown, but one theory of the cause suggests that an autoimmune process is to blame. This is corroborated by the pathological features of cellular infiltration, which include plasma cells and lymphocytes, the commonly observed focal vasculitis on pathological examination, and the positive response of some multifocal fibrosclerosis patients to systemic ...
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.
IgG4-related disease has more recently been identified as a cause of aortitis, and also as a cause of periaortitis (inflammation surrounding the aorta). [3] There is a wide range of symptoms that are dependent on the location of the aortic inflammation or associated disorder.
Thyroiditis is generally caused by an immune system attack on the thyroid, resulting in inflammation and damage to the thyroid cells. This disease is often considered a malfunction of the immune system and can be associated with IgG4-related systemic disease, in which symptoms of autoimmune pancreatitis, retroperitoneal fibrosis and noninfectious aortitis also occur.
Idiopathic orbital inflammatory disease; Idiopathic sclerosing mesenteritis; IgG4-related ophthalmic disease; IgG4-related prostatitis; IgG4-related skin disease; Inflammatory myofibroblastic tumour; Interstitial nephritis