Main Article Content
IgG4-related disease (IgG4-RD) is a complex fibroinflammatory disease first identified in 2003. It has been described in every organ. Type- 2 helper T cell immune-mediated responses are thought to be pathogenic. Serum IgG4 levels are not always elevated and immunohistochemical analysis is the gold standard for diagnosis. When available, histopathology typically reveals lymphoplasmacytic infiltration, fibrosis, and abundant IgG4-positive plasma cells.
Here, we review clinically salient features of IgG4-related disease including its epidemiology, pathophysiology, diagnosis, and emerging treatment strategies.
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
2. Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet 2015; 385: 1460–71.
3. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb;366(6):539-51.
4. Atallah PC, Kassier A, Powers S. IgG4-related disease with effusive–constrictive pericarditis, tamponade, and hepatopathy: A novel triad. Int J Cardiol. 2014 Sep 20;176(2):516-8.
5. Land D, Zwerina J, Pieringer H. IgG4-related disease: current challenges and future prospects. Therapeutics and Clinical Risk Management 2016:12 189–199.
6. Inoue D, Yoshida K, Yoneda N, et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore). 2015;94(15):e680.
7. Wallace ZS, Deshpande V, Mattoo H, et al. IgG4-related disease: clinical and laboratory features in one hundred twenty-five patients. Arthritis Rheumatol. 2015;67(9):2466–2475.
8. Stone JH. IgG4-related disease: pathophysiologic insights drive emerging treatment approaches. Clin Exp Rheumatol 2016; 34 (Suppl. 98): S00-S00.
9. Della Torre E, Mattoo H, Mahajan VS, Carruthers M, Pillai S, Stone JH. Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease. Allergy 2014;69:269–72.
10. Okazaki K, Uchida K, Koyabu M, Miyoshi H, Takaoka M. Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol 2011; 46: 277-88.
11. Sakamoto A, Ishizaka N, Imai Y, Nagai R. Serum levels of IgG4 and soluble interleukin-2 receptor in patients with abdominal and thoracic aortic aneurysm who undergo coronary angiography. Atherosclerosis 2012; 221: 602-3.
12. Tajima M, Nagai R, Hiroi Y. IgG4-Related Cardiovascular Disorders. Int Heart J. 2014;55(4):287-95. Epub 2014 Jun 5.
13. Ryu JH, Horie R, Sekiguchi H, Peikert T, Yi ES. Spectrum of disorders associated with elevated serum IgG4 levels encountered in clinical practice. Int J Rheumatol. 2012;2012:232960.
14. Stone JH, Brito-Zerón P, Bosch X, Ramos-Casals M. Diagnostic approach to the complexity of IgG4-related disease. Mayo Clin Proc. 2015; 90(7):927–939.
15. Khosroshahi A, Cheryk LA, Carruthers MN, Edwards JA, Bloch DB, Stone JH. Brief report: spuriously low serum IgG4 concentrations caused by the prozone phenomenon in patients with IgG4-related disease. Arthritis Rheumatol. 2014;66(1):213–217.
16. Wallace ZS, Mattoo H, Carruthers M et al.: Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations. Ann Rheum Dis 2015; 74: 190-5.
17. Dillon J, Dart A, Sutherland T. Imaging features of Immunoglobulin G4-related disease. J Med Imaging Radiat Oncol August 2016 1754-9485.
18. Mattoo H, Mahajan VS, Della-Torre E et al. De novo oligoclonal expansions of circulating plasmablasts in active and relapsing IgG-related disease. J Allergy Clin Immunol 2014; 134: 679-87.
19. Khosroshahi A, Wallace ZS, Crowe JL et al. Second International Symposium on IgG4-Related Disease: International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol 2015; 67: 1688-99.
20. Umehara H, Okazaki K, Masaki Y, et al; Research Program for Intractable Disease by Ministry of Health, Labor and Welfare (MHLW) Japan G4 team. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012;22(1):1–14.