The objectives of this study were to evaluate patients with aortic abdominal aneurysm (AAA) with regard to immunoglobulin (Ig)G4-related disease (IgG4-RD). IgG4-RD represents a recently defined condition comprised of a collection of disorders characterized by IgG4 hypergammaglobulinemia, the presence of IgG4-positive plasma cells in organs affected with fibrotic or sclerotizing changes and typical histopathological features. It was identified as a possible cause of vasculitis in large vessels. Studies have been published on a possible association between inflammatory aortic or cardiovascular disease and IgG4-RD. We examined 114 patients with AAA requiring surgery in order to identify findings which are characteristic of IgG4-RD. Aneurysm samples from seven patients showed histopathological features consistent with IgG4-RD and the presence of IgG4+ plasma cells. Only two of these seven patients showed elevated IgG4 serum levels higher 1·35 g/l. In five of the patients, the concentration of serum IgG4 was lower than 1·20 g/l, with the number of IgG4+ plasma cells being higher than 50/high-power field. These findings were consistent with AAA being a heterogeneous group of inflammatory diseases with different pathogenesis.

Department of Clinical and Experimental Pathology IKEM Prague Czech Republic

Department of Clinical Biochemistry Haematology and Immunology Na Homolce Hospital Prague Czech Republic

Department of Pathology Na Homolce Hospital Prague Czech Republic

Department of Vascular Surgery Na Homolce Hospital Prague Czech Republic

Zobrazit více v PubMed

Go AS, Mozaffarian D, Roger VL, Heart American et al Association Statistics Committee and Stroke Statistics Subcommittee. Executive summary: heart disease and stroke statistics 2014 update: a report from the American Heart Association. Circulation 2014; 129: 399–410. PubMed

Golledge J, Norman P. Atherosclerosis and abdominal aortic aneurysm: cause, response or common risk factors? Arterioscler Thromb Vasc Biol 2010; 30:1075–7. PubMed PMC

Maegdefessel L, Dalman RL, Tsao PS. Pathogenesis of abdominal aortic aneurysms: microRNAs, proteases, genetic associations. Annu Rev Med 2014; 65:49–62. PubMed

Davis FM, Rateri DL, Daugherty L. Mechanisms of aortic aneurysm formation: translating preclinical studies into clinical therapies. Heart 2014; 100:1498–505. PubMed

Shimizu K, Mitchell RN, Libby P. Inflammation and cellular immune responses in abdominal aortic aneurysms. Arterioscler Thromb Vasc Biol 2006; 26:987–94. PubMed

Rizas KD, Ippagunta N, Tilson MD. Immune cells and molecular mediators in the pathogenesis of the abdominal aortic aneurysm. Cardiol Rev 2009; 17:201–10. PubMed

Lindholt JS, Shi GP. Chronic inflammation, immune response, and infection in abdominal aortic aneurysms. Eur J Vasc Endovasc Surg 2006; 31:453–63. PubMed

Lu S, White JV, Lin WL et al Aneurysmal lesions of patients with abdominal aortic aneurysm contain clonally expanded T cells. J Immunol 2014; 192:4897–912. PubMed PMC

Peshkova IO, Schaefer G, Koltsova EK. Atherosclerosis and aortic aneurysm – is inflammation a common denominator? FEBS J 2016; 283:1636–52. PubMed

Pradhan‐Nabzdyk L, Huang CH, Logerfo FW et al Current siRNA targets in atherosclerosis and aortic aneurysm. Discov Med 2014; 17:233–46. PubMed PMC

Kamisawa T, Zen Y, Pillai S et al IgG4‐related disease. Lancet 2014; 385:1460–71. PubMed

Mahajan VS, Mattoo H, Deshpande V et al IgG4 related diseases. Annu Rev Pathol 2014; 9:315–47. PubMed

Jennette JC, Falk RJ, Bacon PA et al 2012 Revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2013; 65:1–11. PubMed

Perugino CA, Wallace ZS, Meyersohn N et al Large vessel involvement by IgG4‐related disease. Medicine (Balt) 2016; 95:e3344. PubMed PMC

Laco J, Podhola M, Kamaradova K et al Idiopathic vs. secondary retroperitoneal fibrosis: a clinicopathological study of 12 cases with emphasis to possible relationship to IgG4‐related disease. Virchows Arch 2013; 463:721–30. PubMed

Hourai R, Kasashima S, Sohmiya K et al IgG4‐positive cell infiltration in various cardiovascular disorders – results from histopathological analysis of surgical samples. BMC Cardiovasc Disord 2017; 17:52. PubMed PMC

Deshpande V, Zen Y, Chan JK et al Consensus statement on the pathology of IgG4‐related disease. Mod Pathol 2012; 25:1189–92. PubMed

Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4‐related systemic disease. Curr Opin Rheumatol 2011; 23:88–94. PubMed

Agaimy A, Weyand M, Strecker T. Inflammatory thoracic aortic aneurysm (lymphoplasmacytic thoracic aortitis): a 13‐year‐experience at a German Heart Center with emphasis on possible role of IgG4. Int J Clin Exp Pathol 2013; 6:1713–22. PubMed PMC

Koo BS, Koh YW, Hong S et al Frequency of immunoglobulin G4‐related aortitis in cases with aortic resection and their clinical characteristics compared to other aortitis. Int J Rheum Dis 2014; 13:420–4. PubMed

Ebbo M, Daniel L, Pavic M et al IgG4‐related systemic disease: features and treatment response in a French cohort: results of a multicenter registry. Medicine (Balt) 2012; 91:49–56. PubMed

Fox RI, Fox CM. IgG4 levels and plasmablasts as a marker for IgG4‐related disease (IgG4‐RD). Ann Rheum Dis 2015; 74:1–3. PubMed

Raparia K, Molina CP, Quiroga‐Garza G et al Inflammatory aortic aneurysm: possible manifestation of IgG4‐related sclerosing disease. Int J Clin Exp Pathol 2013; 6:469–75. PubMed PMC

Benassi F, Molardi A, Nicolini F et al IgG4‐related aortitis of the ascending aorta in a patient undergoing emergent coronary artery bypass graft: a challenging disease. J Genet Synd Gene Ther 2013; 4:188–91.

Kasashima S, Zen Y, Kawashima A et al A new clinicopathological entity of IgG4‐related inflammatory abdominal aortic aneurysm. J Vasc Surg 2009; 49:1264–71. PubMed

Ozawa M, Fujinaga Y, Asano J et al Clinical features of IgG4‐related periaortitis/periarteritis based on the analysis of 179 patients with IgG4‐related disease: a case–control study. Arthritis Res Ther 2017; 19:223. PubMed PMC

Inoue D, Zen Y, Abo H et al Immunoglobulin G4‐related periaortitis and periarteritis: CT findings in 17 patients. Radiology 2011; 261:625–33. PubMed

Yabusaki S, Oyama‐Manabe N, Manabe O et al Characteristics of immunoglobulin G4‐related aortitis/periaortitis and periarteritis on fluorodeoxyglucose positron emission tomography/computed tomography co‐registered with contrast‐enhanced computed tomography. Eur J Nuclear Med Mol Imaging Res 2017; 7:20–7. PubMed PMC