IgG4-related disease
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- updated 10 May 2022:
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IgG4-related disease (IgG4-RD) is a systemic disease that is characterised by fibroinflammatory infiltration of various organs, including by plasma cells that express IgG4 (immunoglobulin G subclass 4).
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, IgG4-associated disease, and IgG4-related multiorgan lymphoproliferative syndrome. Since 2012, however, IgG4-related disease has become the preferred term 9,10.
Epidemiology
IgG4-related diseases are more common in middle-aged or elderly males.
Clinical presentation
Clinical manifestations vary by the organ involved. These organ systems may be either synchronously or sequentially involved.
Pathology
Location
The disease spectrum is broad across organ systems:
-
central nervous system
-
head and neck
-
thoracic
-
abdominopelvic
-
multisystem
inflammatory pseudotumours (e.g. presenting as paravertebral masses)
Microscopic appearance
There are three major histopathologic characteristics of IgG4-related disease 9:
dense lymphoplasmacytic infiltrate
at least focally storiform fibrosis
obliterative phlebitis
Two of these features are required for a confident pathologic diagnosis of IgG4-related disease, with a few organ-specific exceptions 9. Additional supporting features are non-obliterative phlebitis and eosinophilia 9.
Immunophenotype
Immunohistochemistry should demonstrate plasma cells that stain for IgG4. IgG4+ plasma cells must account for more than 40% of IgG+ plasma cells 9. The absolute density of IgG4+ plasma cells (number per high power field (hpf)) suggestive of the diagnosis varies by organ. For example, the cutoff is >10/hpf for pancreatic biopsy specimens and >100/hpf for lacrimal and salivary glands 9.
Markers
Serology is often, but not always, abnormal in IgG4-related diseases. A serum IgG4 level >135 mg/dL is supportive of the diagnosis 9.
Supportive are also circulating plasmablasts 12.
-<p><strong>IgG4-related disease (IgG4-RD) </strong>is a systemic disease that is characterised by fibroinflammatory infiltration of various organs, including by <a href="/articles/plasma-cells">plasma cells</a> that express IgG4 (immunoglobulin G subclass 4).</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, IgG4-associated disease, and IgG4-related multiorgan lymphoproliferative syndrome. Since 2012, however, IgG4-related disease has become the preferred term <sup>9,10</sup>.</p><h4>Epidemiology</h4><p>IgG4-related diseases are more common in middle-aged or elderly males.</p><h4>Clinical presentation</h4><p>Clinical manifestations vary by the organ involved. These organ systems may be either synchronously or sequentially involved.</p><h4>Pathology</h4><h5>Location</h5><p>The disease spectrum is broad across organ systems:</p><ul>-<li>-<p>central nervous system</p>-<ul>-<li><p><a href="/articles/igg4-related-hypertrophic-pachymeningitis-1">IgG4-related hypertrophic pachymeningitis</a></p></li>-<li><p><a href="/articles/igg4-related-hypophysitis">IgG4-related hypophysitis</a></p></li>-<li><p>chronic <a href="/articles/subdural-haemorrhage">subdural haemorrhage</a></p></li>-</ul>-</li>-<li>-<p>head and neck</p>-<ul>-<li><p><a href="/articles/sialadenitis">sialadenitis</a>/<a href="/articles/kuttner-tumour">chronic sclerosing sialadenitis</a> (Küttner tumour)</p></li>-<li><p><a href="/articles/riedel-thyroiditis">Riedel thyroiditis</a></p></li>-<li><p><a href="/articles/igg4-related-orbital-disease">IgG4-related orbital disease</a></p></li>-<li><p><a href="/articles/pansinusitis">pansinusitis</a></p></li>-</ul>-</li>-<li>-<p>thoracic</p>-<ul>-<li><p><a href="/articles/interstitial-pneumonia">interstitial pneumonia</a>/<a href="/articles/igg4-related-lung-disease-1">IgG4-related lung disease</a></p></li>-<li><p><a href="/articles/fibrosing-mediastinitis">fibrosing mediastinitis</a></p></li>-<li>-<p><a href="/articles/igg4-related-cardiovascular-disease">IgG4-related cardiovascular disease</a></p>-<ul><li><p><a href="/articles/igg4-related-coronary-disease-2">IgG4-related coronary disease</a></p></li></ul>-</li>-</ul>-</li>-<li>-<p>abdominopelvic</p>-<ul>-<li><p><a href="/articles/autoimmune-pancreatitis">autoimmune pancreatitis</a></p></li>-<li><p><a href="/articles/igg4-related-sclerosing-cholangitis">sclerosing cholangitis</a></p></li>-<li><p><a href="/articles/sclerosing-mesenteritis-1">sclerosing mesenteritis</a></p></li>-<li><p><a href="/articles/cholecystitis">cholecystitis</a> </p></li>-<li>-<p><a href="/articles/igg4-related-renal-diseases">IgG4-related renal diseases</a> <sup>11</sup></p>-<ul><li><p><a href="/articles/tubulo-interstitial-nephritis">tubulointerstitial nephritis</a></p></li></ul>-</li>-<li><p><a href="/articles/retroperitoneal-fibrosis-1">retroperitoneal fibrosis</a></p></li>-<li><p><a href="/articles/inflammatory-abdominal-aortic-aneurysm">inflammatory aortic aneurysm</a></p></li>-<li><p><a href="/articles/prostatitis">prostatitis</a></p></li>-<li>-<p>gastrointestinal <sup>8</sup></p>-<ul><li><p><a href="/articles/colorectal-igg4-related-disease">colorectal</a></p></li></ul>-</li>-</ul>-</li>-<li>-<p>multisystem</p>-<ul>-<li><p><a href="/articles/inflammatory-pseudotumours">inflammatory pseudotumours</a> (e.g. presenting as paravertebral masses)</p></li>-<li><p><a href="/articles/lymphadenopathy">lymphadenopathy</a></p></li>-</ul>-</li>-</ul><h5>Microscopic appearance</h5><p>There are three major histopathologic characteristics of IgG4-related disease <sup>9</sup>: </p><ul>-<li><p>dense lymphoplasmacytic infiltrate</p></li>-<li><p>at least focally storiform fibrosis</p></li>-<li><p>obliterative phlebitis</p></li>- +<p><strong>IgG4-related disease (IgG4-RD) </strong>is a systemic disease that is characterised by fibroinflammatory infiltration of various organs, including by <a href="/articles/plasma-cells">plasma cells</a> that express IgG4 (immunoglobulin G subclass 4).</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, IgG4-associated disease, and IgG4-related multiorgan lymphoproliferative syndrome. Since 2012, however, IgG4-related disease has become the preferred term <sup>9,10</sup>.</p><h4>Epidemiology</h4><p>IgG4-related diseases are more common in middle-aged or elderly males.</p><h4>Clinical presentation</h4><p>Clinical manifestations vary by the organ involved. These organ systems may be either synchronously or sequentially involved.</p><h4>Pathology</h4><h5>Location</h5><p>The disease spectrum is broad across organ systems:</p><ul>
- +<li>
- +<p>central nervous system</p>
- +<ul>
- +<li><p><a href="/articles/igg4-related-hypertrophic-pachymeningitis-1">IgG4-related hypertrophic pachymeningitis</a></p></li>
- +<li><p><a href="/articles/igg4-related-hypophysitis">IgG4-related hypophysitis</a></p></li>
- +<li><p>chronic <a href="/articles/subdural-haemorrhage">subdural haemorrhage</a></p></li>
- +</ul>
- +</li>
- +<li>
- +<p>head and neck</p>
- +<ul>
- +<li><p><a href="/articles/sialadenitis">sialadenitis</a>/<a href="/articles/kuttner-tumour">chronic sclerosing sialadenitis</a> (Küttner tumour)</p></li>
- +<li><p><a href="/articles/riedel-thyroiditis">Riedel thyroiditis</a></p></li>
- +<li><p><a href="/articles/igg4-related-orbital-disease">IgG4-related orbital disease</a></p></li>
- +<li><p><a href="/articles/pansinusitis">pansinusitis</a></p></li>
- +</ul>
- +</li>
- +<li>
- +<p>thoracic</p>
- +<ul>
- +<li><p><a href="/articles/interstitial-pneumonia">interstitial pneumonia</a>/<a href="/articles/igg4-related-lung-disease-1">IgG4-related lung disease</a></p></li>
- +<li><p><a href="/articles/fibrosing-mediastinitis">fibrosing mediastinitis</a></p></li>
- +<li>
- +<p><a href="/articles/igg4-related-cardiovascular-disease">IgG4-related cardiovascular disease</a></p>
- +<ul><li><p><a href="/articles/igg4-related-coronary-disease-2">IgG4-related coronary disease</a></p></li></ul>
- +</li>
- +</ul>
- +</li>
- +<li>
- +<p>abdominopelvic</p>
- +<ul>
- +<li><p><a href="/articles/autoimmune-pancreatitis">autoimmune pancreatitis</a></p></li>
- +<li><p><a href="/articles/igg4-related-sclerosing-cholangitis">sclerosing cholangitis</a></p></li>
- +<li><p><a href="/articles/sclerosing-mesenteritis-1">sclerosing mesenteritis</a></p></li>
- +<li><p><a href="/articles/cholecystitis">cholecystitis</a> </p></li>
- +<li>
- +<p><a href="/articles/igg4-related-renal-diseases">IgG4-related renal diseases</a> <sup>11</sup></p>
- +<ul><li><p><a href="/articles/tubulo-interstitial-nephritis">tubulointerstitial nephritis</a></p></li></ul>
- +</li>
- +<li><p><a href="/articles/retroperitoneal-fibrosis-1">retroperitoneal fibrosis</a></p></li>
- +<li><p><a href="/articles/inflammatory-abdominal-aortic-aneurysm">inflammatory aortic aneurysm</a></p></li>
- +<li><p><a href="/articles/prostatitis">prostatitis</a></p></li>
- +<li>
- +<p>gastrointestinal <sup>8</sup></p>
- +<ul><li><p><a href="/articles/colorectal-igg4-related-disease">colorectal</a></p></li></ul>
- +</li>
- +</ul>
- +</li>
- +<li>
- +<p>multisystem</p>
- +<ul>
- +<li><p><a href="/articles/inflammatory-pseudotumours">inflammatory pseudotumours</a> (e.g. presenting as paravertebral masses)</p></li>
- +<li><p><a href="/articles/lymphadenopathy">lymphadenopathy</a></p></li>
- +</ul>
- +</li>
- +</ul><h5>Microscopic appearance</h5><p>There are three major histopathologic characteristics of IgG4-related disease <sup>9</sup>: </p><ul>
- +<li><p>dense lymphoplasmacytic infiltrate</p></li>
- +<li><p>at least focally storiform fibrosis</p></li>
- +<li><p>obliterative phlebitis</p></li>
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IgG4-RD
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igg4-rd.
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