Clinical importance of the IgG4 related disease
Authors:
Zbyněk Hrnčíř 1; Jan Laco 2
Authors‘ workplace:
Univerzita Karlova v Praze, Lékařská fakulta v Hradci Králové, II. interní klinika
1; Univerzita Karlova v Praze, Lékařská fakulta v Hradci Králové, Fingerlandův ústav patologie
2
Published in:
Čas. Lék. čes. 2011; 150: 438-441
Category:
Review Articles
Overview
IgG4 related disease is a chronic immunopathological disorder, proposed as a new diagnostic entity, and characterized as follows: 1. background is in multiorgan lymphoplasmocellular infiltration associated with presence of large number of polyclonal IgG4+ plasma cells, and fibrosclerosis; 2. structural damage of the affected organs is frequently associated with pseudotumorous enlargement and/or other morphological changes; for example aortitis with aneurysmatic dilatation, sclerosing pancreatitis, retroperitoneal fibrosis ect.; 3. serum concentration of IgG4 is usually significantly elevated; 4. responsivity to glucocorticoids is a hallmark of the disease: in rare cases of glucocorticoid-resistance a response to rituximab was observed. At presence the disease related to IgG4 is considered as a rare disorder. Presented knowledge may be useful for better elucidation of its importance in clinical specialities.
Key words:
IgG4-related disease, IgG4+ plasma cells, IgG4 serum concentration, pseudotumorous enlargement, glucocorticoid-responsivity, rituximab.
Sources
1. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001; 344: 732–738.
2. Zen Y, Nakamura Y. IgG4-related disease. A cross-sectional study of 114 cases. Am J Surg 2010; 34: 1812–1819.
3. Suga K, Kawakami Y, Hiyama A, Takeuchi M. F-18 FDG PET--CT findings in Mikulicz disease and systemic involvement of IgG4-related lesions. Clin Nucl Med 2009; 34: 164–167.
4. Hrnčíř Z, Laco J, Slezák R, Rencová E, Drahošová M, Brtková J. Mikuliczova choroba s jednostranným exoftalmem – nemoc spojená s IgG4. Čes Revmatol 2011; v tisku.
5. Laco J. Systémová sklerózující choroba spojená s imunoglobuliny IgG4 – současné poznatky. Cesk Patol 2010; 46: 82–85.
6. Fragoulis GE, Moutsopoulos HM. IgG4 syndrome: Old disease, new perspective. J Rheumatol 2010; 37: 1369–1370.
7. Guarneri F, Guarneri C, Bevenga S. Helicobacter pylori and autoimmune pancreatitis: Role of carbonic anhydrase via molecular mimicry? J Cell Mol Med 2005; 9: 741–744.
8. Ihrler S, Harrison JD. Mikulicz’s disease and Mikulicz’s syndrome: Analysis of the original case report of 1892 in the light of current knowledge identifies a MALT lymphoma. Oral Surg Oral Med Oral Pathol Radiol Endod 2005; 100: 334–339.
9. Masaki Y, Dong L, Kurose N, Kitigawa K, Morikawa Y, et al. Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 2009; 68: 1310–1315.
10. Laco J, Ryska A, Celakovský P, Dolezalova H, Mottl R, Tucek L. Chronic sclerosing sialadenitis as one of the immunoglobulin G4-related disease: a clinicopathologic study of six cases from Central Europe. Histopathology 2011; 58: 1157–1163.
11. Dahlgren M, Khosroshahi A, Nielsen GP, Desphande V, Stone JH. Riedelęs thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res 2010; 62: 1312–1318.
12. Hori M, Makita N, Andoh T, Takiyama H, Yajima Y, et al. Long-term clinical course of IgG4-related systemic disease accompanied by hypophysitis. Endocrine J 2010; 57: 485–492.
13. Cheuk A, Yuen HKL, Chan ACL, Shih LY, Kuo TT, et al. Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: A previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 2008; 32: 1159–1167.
14. Stone JH, Khosroshahi A, Deshpande V, Stone JR. IgG4-related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res 2010; 62: 316–322.
15. Laco J, Steiner I, Holubec T, Dominik J, Holubcova Z, Vojacek J. Isolated thoracic aortitis: clinicopathological and immunohistochemical study of 11 cases. Cardiovasc Pathol 2010; Oct 29. doi:10.1016/j.carpath.2010.09.003.
16. Masaki Y, Sugai S, Umehara H. IgG4-related diseases including Mikulicz’s disease and sclerosing pancreatitis: diagnostic insights. J Rheumatol 2010; 37: 1380–1385.
17. Yamamoto M, Ohara M, Suzuki Ch, Oka T, Naishiro Y, et al. A case of Mikulicz’s disease complicated by autoimmune pancreatitis, in which impaired glucose tolerance was improved by glucocorticoid treatment. Jpn J Clin Immunol 2005; 28: 349–356.
18. Aoki A, Sato K, Itabashi M, Takei T, Yoshida T, et al. A case of Mikulicz’ disease complicated with severe interstitial nephritis associated with IgG4. Clin Exp Nephrol 2009; 13: 367–372.
19. Shimoyama K, Ogawa N, Sawaki T, Karasawa H, Masaki Y, et al. A case of Mikulicz’s disease complicated with interstitial nephritis successfully treated by high-dose corticosteroid. Mod Rheumatol 2006; 16: 176–182.
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Addictology Allergology and clinical immunology Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric gastroenterology Paediatric surgery Paediatric cardiology Paediatric neurology Paediatric ENT Paediatric psychiatry Paediatric rheumatology Diabetology Pharmacy Vascular surgery Pain managementArticle was published in
Journal of Czech Physicians
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