IgG4-related disease
Updates to Article Attributes
IgG4-related disease (IgG4-RD) is a systemic disease that is characterised by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs.
Terminology
This condition has been known by many other names in the past, such as IgG4-related sclerosing disease, IgG4-related systemic disease, IgG4-related systemic sclerosing disease, IgG4-syndrome, and IgG4-associated disease, however a consensus in 2012 agreed upon the use of IgG4-related disease.
Epidemiology
IgG4 related-related diseases are more common in middle age or elderly males.
Clinical presentation
Clinical manifestations are apparent in the pancreas, biliary tree, gallbladder, salivary gland, retroperitoneum, kidney, lung, pituitary gland, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. These organ systems may be either synchronously or sequentially involved.
Pathology
The disease spectrum can include:
-
CNScentral nervous system - head and neck
- thoracic
- abdominopelvic
- multisystem
- inflammatory pseudotumours (e.g. presenting as paravertebral masses)
- lymphadenopathy
-<p><strong>IgG4-related disease (IgG4-RD) </strong>is a systemic disease that is characterised by extensive IgG4-positive <a href="/articles/plasma-cells">plasma cells</a> and <a href="/articles/t-lymphocyte">T-lymphocyte</a> infiltration of various organs.</p><h4>Terminology</h4><p>This condition has been known by many other names in the past, such as IgG4-related sclerosing disease, IgG4-related systemic disease, IgG4-related systemic sclerosing disease, IgG4-syndrome, and IgG4-associated disease, however a consensus in 2012 agreed upon the use of IgG4-related disease.</p><h4>Epidemiology</h4><p>IgG4 related diseases are more common in middle age or elderly males.</p><h4>Clinical presentation</h4><p>Clinical manifestations are apparent in the <a href="/articles/pancreas">pancreas</a>, biliary tree, gallbladder, salivary gland, retroperitoneum, kidney, lung, pituitary gland, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. These organ systems may be either synchronously or sequentially involved.</p><h4>Pathology</h4><p>The disease spectrum can include:</p><ul>-<li>CNS<ul>- +<p><strong>IgG4-related disease (IgG4-RD) </strong>is a systemic disease that is characterised by extensive IgG4-positive <a href="/articles/plasma-cells">plasma cells</a> and <a href="/articles/t-lymphocyte">T-lymphocyte</a> infiltration of various organs.</p><h4>Terminology</h4><p>This condition has been known by many other names in the past, such as IgG4-related sclerosing disease, IgG4-related systemic disease, IgG4-related systemic sclerosing disease, IgG4-syndrome, and IgG4-associated disease, however a consensus in 2012 agreed upon the use of IgG4-related disease.</p><h4>Epidemiology</h4><p>IgG4-related diseases are more common in middle age or elderly males.</p><h4>Clinical presentation</h4><p>Clinical manifestations are apparent in the <a href="/articles/pancreas">pancreas</a>, biliary tree, gallbladder, salivary gland, retroperitoneum, kidney, lung, pituitary gland, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. These organ systems may be either synchronously or sequentially involved.</p><h4>Pathology</h4><p>The disease spectrum can include:</p><ul>
- +<li>central nervous system<ul>
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