Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on sleep in brain health

LIVE: Thursday 2nd May 2024, 18:00-19:30 (CEST)

Quality sleep is essential for health. But what happens to our brains when sleep patterns are disturbed? Join our experts to explore the interplay between sleep disruption and neurological diseases, and the questions that you need to be asking your patients to help you prevent the harmful effects of sleep deprivation.
ADVANCED SEARCH
Log in
Top
Arthritis Research & Therapy

Open Access 01-12-2017 | Research article

Clinical features of IgG4-related periaortitis/periarteritis based on the analysis of 179 patients with IgG4-related disease: a case–control study

Authors: Makiko Ozawa, Yasunari Fujinaga, Junpei Asano, Akira Nakamura, Takayuki Watanabe, Tetsuya Ito, Takashi Muraki, Hideaki Hamano, Shigeyuki Kawa

Published in: Arthritis Research & Therapy | Issue 1/2017

Login to get access

Abstract

Background

Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic condition characterized by high serum immunoglobulin G4 (IgG4) concentration and IgG4-bearing plasma cell infiltration in affected organs. Although it has become evident that IgG4-RD also involves the systemic aortic/arterial system, the precise details of this condition remain unclear. The present study sought to clarify the clinical features of IgG4-related periaortitis/periarteritis.

Methods

Among 223 patients with IgG4-RD, 179 (131 male, median onset age 67 years) were recruited for this study. Periaortitis/periarteritis was defined as vessel wall thickness with circumferential enhancement on contrast-enhanced computed tomography.

Results

Periaortitis/periarteritis was identified in 65 (36.3%; 53 male) of 179 IgG-RD patients. The distribution of IgG4-related periaortitis/periarteritis could be broadly classified into five types, with the most prevalent Type 2 (44.6%) being localized at the infra-renal artery portion of the abdominal aorta and continuing to the iliac arteries. The infra-renal artery region of the abdominal aorta was most frequently involved (>80%) among IgG4-related periaortitis/periarteritis cases. Comparisons of clinical parameters between IgG4-RD patients with and without periaortitis/periarteritis revealed significantly higher propensities for older IgG4-RD onset age and highly active disease state featuring elevated serum IgG, IgG4, circulating immune complex, and soluble interleukin-2 receptor. All patients showed improvement of wall thickening after steroid therapy, although nine patients (20.9%) exhibited worsening of luminal dilatation. The main risk factor for this manifestation was prior luminal dilatation according to multivariate analysis.

Conclusion

IgG4-related periaortitis/periarteritis predominantly occurred at the infra-renal artery portion of the abdominal aorta, affected older IgG4-RD onset patients, and was prevalent in highly active disease states. As reported previously, the main risk factor for worsening luminal dilation after corticosteroid therapy was the existence of luminal dilation beforehand.
Literature
1.
Kawa S, Hamano H, Kiyosawa K. Autoimmune pancreatitis and IgG4-related disease. In: Rose N, MacKay I, editors. The autoimmune diseases. 5th ed. St Louis, MO: Academic Press; 2013. p. 935–49.
2.
Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344(10):732–8.CrossRefPubMed
3.
Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, Nakazawa K, Shimojo H, Kiyosawa K. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002;359(9315):1403–4.CrossRefPubMed
4.
Yamamoto M, Takahashi H, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Shinomura Y, Imai K. A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease. Mod Rheumatol. 2006;16(6):335–40.CrossRefPubMedPubMedCentral
5.
Kobayashi H, Shimokawaji T, Kanoh S, Motoyoshi K, Aida S. IgG4-positive pulmonary disease. J Thorac Imaging. 2007;22(4):360–2.CrossRefPubMed
6.
Nakazawa T, Naitoh I, Hayashi K, Okumura F, Miyabe K, Yoshida M, Yamashita H, Ohara H, Joh T. Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol. 2012;47(1):79–87.CrossRefPubMed
7.
Kawano M, Saeki T, Nakashima H, Nishi S, Yamaguchi Y, Hisano S, Yamanaka N, Inoue D, Yamamoto M, Takahashi H, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011;15(5):615–26.CrossRefPubMed
8.
Yoshimura Y, Takeda S, Ieki Y, Takazakura E, Koizumi H, Takagawa K. IgG4-associated prostatitis complicating autoimmune pancreatitis. Intern Med. 2006;45(15):897–901.CrossRefPubMed
9.
Mavrogeni S, Markousis-Mavrogenis G, Kolovou G. IgG4-related cardiovascular disease. The emerging role of cardiovascular imaging. Eur J Radiol. 2017;86:169–75.CrossRefPubMed
10.
Kasashima S, Zen Y, Kawashima A, Konishi K, Sasaki H, Endo M, Matsumoto Y, Kawakami K, Kasashima F, Moriya M, et al. Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis. Am J Surg Pathol. 2008;32(2):197–204.CrossRefPubMed
11.
Sakata N, Tashiro T, Uesugi N, Kawara T, Furuya K, Hirata Y, Iwasaki H, Kojima M. IgG4-positive plasma cells in inflammatory abdominal aortic aneurysm: the possibility of an aortic manifestation of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32(4):553–9.CrossRefPubMed
12.
Matsumoto Y, Kasashima S, Kawashima A, Sasaki H, Endo M, Kawakami K, Zen Y, Nakanuma Y. A case of multiple immunoglobulin G4-related periarteritis: a tumorous lesion of the coronary artery and abdominal aortic aneurysm. Hum Pathol. 2008;39(6):975–80.CrossRefPubMed
13.
Ito H, Kaizaki Y, Noda Y, Fujii S, Yamamoto S. IgG4-related inflammatory abdominal aortic aneurysm associated with autoimmune pancreatitis. Pathol Int. 2008;58(7):421–6.CrossRefPubMed
14.
Tseng CW, Tsai JJ, Chen CC. Abdominal aortitis associated with autoimmune pancreatitis. Clin Gastroenterol Hepatol. 2009;7(1):e3–4.CrossRefPubMed
15.
Stone JH, Khosroshahi A, Hilgenberg A, Spooner A, Isselbacher EM, Stone JR. IgG4-related systemic disease and lymphoplasmacytic aortitis. Arthritis Rheum. 2009;60(10):3139–45.CrossRefPubMed
16.
Qian Q, Kashani KB, Miller DV. Ruptured abdominal aortic aneurysm related to IgG4 periaortitis. N Engl J Med. 2009;361(11):1121–3.CrossRefPubMed
17.
Kasashima S, Zen Y, Kawashima A, Endo M, Matsumoto Y, Kasashima F. A new clinicopathological entity of IgG4-related inflammatory abdominal aortic aneurysm. J Vasc Surg. 2009;49(5):1264–71. discussion 1271.CrossRefPubMed
18.
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 (Hoboken). 2010;62(3):316–22.CrossRef
19.
Kasashima S, Zen Y, Kawashima A, Endo M, Matsumoto Y, Kasashima F, Ohtake H, Nakanuma Y. A clinicopathologic study of immunoglobulin G4-related sclerosing disease of the thoracic aorta. J Vasc Surg. 2010;52(6):1587–95.CrossRefPubMed
20.
Ishida M, Hotta M, Kushima R, Asai T, Okabe H. IgG4-related inflammatory aneurysm of the aortic arch. Pathol Int. 2009;59(4):269–73.CrossRefPubMed
21.
Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Yoshikawa J, Miyayama S, Nakanuma Y, Matsui O. Immunoglobulin G4-related periaortitis and periarteritis: CT findings in 17 patients. Radiology. 2011;261(2):625–33.
22.
Kasashima S, Kawashima A, Endo M, Matsumoto Y, Kasashima F, Zen Y, Nakanuma Y. A clinicopathologic study of immunoglobulin G4-related disease of the femoral and popliteal arteries in the spectrum of immunoglobulin G4-related periarteritis. J Vasc Surg. 2013;57(3):816–22.CrossRefPubMed
23.
Mizushima I, Inoue D, Yamamoto M, Yamada K, Saeki T, Ubara Y, Matsui S, Masaki Y, Wada T, Kasashima S, et al. Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study. Arthritis Res Ther. 2014;16(4):R156.CrossRefPubMedPubMedCentral
24.
Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Yoshino T, Nakamura S, Kawa S, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21–30.CrossRefPubMed
25.
Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, Kim MH, Kloppel G, Lerch MM, Lohr M, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40(3):352–8.CrossRefPubMed
26.
JCS Joint Working Group. Guidelines for diagnosis and treatment of aortic aneurysm and aortic dissection (JCS 2011): digest version. Circ J. 2013;77(3):789–828.
27.
Kamisawa T, Okazaki K, Kawa S, Ito T, Inui K, Irie H, Nishino T, Notohara K, Nishimori I, Tanaka S, et al. Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 III. Treatment and prognosis of autoimmune pancreatitis. J Gastroenterol. 2014;49(6):961–70.CrossRefPubMed
28.
Sughimoto K, Shimamura Y, Tezuka C, Tsubota K, Liu H, Okumura K, Masuda Y, Haneishi H. Effects of arterial blood flow on walls of the abdominal aorta: distributions of wall shear stress and oscillatory shear index determined by phase-contrast magnetic resonance imaging. Heart Vessels. 2016;31(7):1168–75.CrossRefPubMed
29.
Castelein T, Coudyzer W, Blockmans D. IgG4-related periaortitis vs idiopathic periaortitis: is there a role for atherosclerotic plaque in the pathogenesis of IgG4-related periaortitis? Rheumatology (Oxford). 2015;54(7):1250–6.CrossRef
30.
Tajima M, Hiroi Y, Takazawa Y, Muraoka H, Iwata H, Yamashita H, Hirata Y, Nagai R. Immunoglobulin G4-related multiple systemic aneurysms and splenic aneurysm rupture during steroid therapy. Hum Pathol. 2014;45(1):175–9.CrossRefPubMed
31.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.CrossRefPubMed
32.
Garg SK, Mohan S, Kumar S. Diagnostic value of 3D contrast-enhanced magnetic resonance angiography in Takayasu's arteritis—a comparative study with digital subtraction angiography. Eur Radiol. 2011;21(8):1658–66.CrossRefPubMed
Metadata
Title
Clinical features of IgG4-related periaortitis/periarteritis based on the analysis of 179 patients with IgG4-related disease: a case–control study
Authors
Makiko Ozawa
Yasunari Fujinaga
Junpei Asano
Akira Nakamura
Takayuki Watanabe
Tetsuya Ito
Takashi Muraki
Hideaki Hamano
Shigeyuki Kawa
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Arthritis Research & Therapy / Issue 1/2017
Electronic ISSN: 1478-6362
DOI
https://doi.org/10.1186/s13075-017-1432-8

ACC 2024 Congress Coverage

Heart Failure Video

Year in Review: Heart failure and cardiomyopathies

Watch now

Watch this official video from ACC.24 to hear Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

16-04-2024 Type 2 Diabetes News

Incentivised to exercise

11-04-2024 Lifestyle Modifications News

New intervention for STEMI-related cardiogenic shock

10-04-2024 Myocardial Infarction News

» View more highlights on the ACC 2024 congress hub page

Image Credits