Difference between revisions of "IgG4-related systemic diseases"

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'''IgG4-related systemic disease''', also '''IgG4-related disease''', is a disease characterized IgG4 positive [[plasma cells]] and fibrosis.<ref name=pmid21124086>{{Cite journal  | last1 = Khosroshahi | first1 = A. | last2 = Stone | first2 = JH. | title = A clinical overview of IgG4-related systemic disease. | journal = Curr Opin Rheumatol | volume = 23 | issue = 1 | pages = 57-66 | month = Jan | year = 2011 | doi = 10.1097/BOR.0b013e3283418057 | PMID = 21124086 }}</ref>
The '''IgG4-related systemic diseases''', also '''IgG4-related diseases''', are diseases characterized IgG4 positive [[plasma cells]] and fibrosis.<ref name=pmid21124086>{{Cite journal  | last1 = Khosroshahi | first1 = A. | last2 = Stone | first2 = JH. | title = A clinical overview of IgG4-related systemic disease. | journal = Curr Opin Rheumatol | volume = 23 | issue = 1 | pages = 57-66 | month = Jan | year = 2011 | doi = 10.1097/BOR.0b013e3283418057 | PMID = 21124086 }}</ref>


There are several IgG4-related diseases:
==List of IgG4-related diseases==
*Lymphoplasmacytic sclerosing pancreatitis - a type of autoimmune pancreatitis.
*Lymphoplasmacytic sclerosing pancreatitis - a type of autoimmune pancreatitis.
*[[Retroperitoneal fibrosis]].<ref name=pmid18609677>{{Cite journal  | last1 = Kamisawa | first1 = T. | last2 = Okamoto | first2 = A. | title = IgG4-related sclerosing disease. | journal = World J Gastroenterol | volume = 14 | issue = 25 | pages = 3948-55 | month = Jul | year = 2008 | doi =  | PMID = 18609677 }}</ref>
*[[Retroperitoneal fibrosis]].<ref name=pmid18609677>{{Cite journal  | last1 = Kamisawa | first1 = T. | last2 = Okamoto | first2 = A. | title = IgG4-related sclerosing disease. | journal = World J Gastroenterol | volume = 14 | issue = 25 | pages = 3948-55 | month = Jul | year = 2008 | doi =  | PMID = 18609677 }}</ref>
*[[Riedel's thyroiditis]].
*[[Riedel's thyroiditis]].
*[[Inflammatory pseudotumour]].<ref name=pmid18609677/>
*[[Inflammatory pseudotumour]],<ref name=pmid18609677/> also known as ''inflammatory myofibroblastic tumour'' (''IMT''); probably only a subset of IMT.<ref name=pmid21297584>{{Cite journal  | last1 = Saab | first1 = ST. | last2 = Hornick | first2 = JL. | last3 = Fletcher | first3 = CD. | last4 = Olson | first4 = SJ. | last5 = Coffin | first5 = CM. | title = IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link? | journal = Mod Pathol | volume = 24 | issue = 4 | pages = 606-12 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.226 | PMID = 21297584 }}</ref>
 
*Sclerosing cholangitis<ref name=pmid18609677/> (IgG4-associated cholangitis<ref>{{Cite journal  | last1 = Barnabas | first1 = A. | last2 = Chapman | first2 = RW. | title = Primary sclerosing cholangitis: is any treatment worthwhile? | journal = Curr Gastroenterol Rep | volume = 14 | issue = 1 | pages = 17-24 | month = Feb | year = 2012 | doi = 10.1007/s11894-011-0230-8 | PMID = 22124849 }}</ref>).
*Sclerosing cholangitis<ref name=pmid18609677/> (IgG4-associated cholangitis<ref>{{Cite journal  | last1 = Barnabas | first1 = A. | last2 = Chapman | first2 = RW. | title = Primary sclerosing cholangitis: is any treatment worthwhile? | journal = Curr Gastroenterol Rep | volume = 14 | issue = 1 | pages = 17-24 | month = Feb | year = 2012 | doi = 10.1007/s11894-011-0230-8 | PMID = 22124849 }}</ref>).
*[[Autoimmune pancreatitis]] (lymphoplasmacytic sclerosing pancreatitis).
*[[Autoimmune pancreatitis]] (lymphoplasmacytic sclerosing pancreatitis).
*Others.
*Others.
==Microscopic==
Features:<ref name=pmid28701054>{{cite journal |authors=Abraham M, Khosroshahi A |title=Diagnostic and treatment workup for IgG4-related disease |journal=Expert Rev Clin Immunol |volume=13 |issue=9 |pages=867–875 |date=September 2017 |pmid=28701054 |pmc=5896560 |doi=10.1080/1744666X.2017.1354698 |url=}}</ref>
*At least 10 IgG4 +ve plasma cells.†
*>40% of [[plasma cell]]s IgG4 +ve.
Note:
*† Minimum number dependent on anatomical site.<ref name=pmid28701054/>
** Lymph node: 100 (or 50) plasma cells/HPF, where 1 HPF ~ 0.196 mm*mm and 3 HPFs (0.588 mm*mm) are assessed.<ref>{{cite journal |authors=Cheuk W, Chan JK |title=Lymphadenopathy of IgG4-related disease: an underdiagnosed and overdiagnosed entity |journal=Semin Diagn Pathol |volume=29 |issue=4 |pages=226–34 |date=November 2012 |pmid=23068302 |doi=10.1053/j.semdp.2012.07.001 |url=}}</ref>
==Sign-out==
===Not present===
<pre>
The provided clinical history is noted. Plasma cells are not readily apparent (<10 plasma cells/0.196 mm*mm).
</pre>
==See also==
*[[Plasma cells]].
*[[HPFitis]].


==References==
==References==

Latest revision as of 14:45, 3 March 2022

The IgG4-related systemic diseases, also IgG4-related diseases, are diseases characterized IgG4 positive plasma cells and fibrosis.[1]

List of IgG4-related diseases

Microscopic

Features:[5]

  • At least 10 IgG4 +ve plasma cells.†
  • >40% of plasma cells IgG4 +ve.

Note:

  • † Minimum number dependent on anatomical site.[5]
    • Lymph node: 100 (or 50) plasma cells/HPF, where 1 HPF ~ 0.196 mm*mm and 3 HPFs (0.588 mm*mm) are assessed.[6]

Sign-out

Not present

The provided clinical history is noted. Plasma cells are not readily apparent (<10 plasma cells/0.196 mm*mm).

See also

References

  1. ↑ Khosroshahi, A.; Stone, JH. (Jan 2011). "A clinical overview of IgG4-related systemic disease.". Curr Opin Rheumatol 23 (1): 57-66. doi:10.1097/BOR.0b013e3283418057. PMID 21124086.
  2. ↑ 2.0 2.1 2.2 Kamisawa, T.; Okamoto, A. (Jul 2008). "IgG4-related sclerosing disease.". World J Gastroenterol 14 (25): 3948-55. PMID 18609677.
  3. ↑ Saab, ST.; Hornick, JL.; Fletcher, CD.; Olson, SJ.; Coffin, CM. (Apr 2011). "IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link?". Mod Pathol 24 (4): 606-12. doi:10.1038/modpathol.2010.226. PMID 21297584.
  4. ↑ Barnabas, A.; Chapman, RW. (Feb 2012). "Primary sclerosing cholangitis: is any treatment worthwhile?". Curr Gastroenterol Rep 14 (1): 17-24. doi:10.1007/s11894-011-0230-8. PMID 22124849.
  5. ↑ 5.0 5.1 Abraham M, Khosroshahi A (September 2017). "Diagnostic and treatment workup for IgG4-related disease". Expert Rev Clin Immunol 13 (9): 867–875. doi:10.1080/1744666X.2017.1354698. PMC 5896560. PMID 28701054. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5896560/.
  6. ↑ Cheuk W, Chan JK (November 2012). "Lymphadenopathy of IgG4-related disease: an underdiagnosed and overdiagnosed entity". Semin Diagn Pathol 29 (4): 226–34. doi:10.1053/j.semdp.2012.07.001. PMID 23068302.
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