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


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.


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.

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 management fenix.admin.empty
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account