Abstract
BackgroundImmunoglobulin 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.MethodsAmong 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.ResultsPeriaortitis/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.ConclusionIgG4-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.
Highlights
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
IgG4-RD was diagnosed based on the Japanese Comprehensive Diagnostic Criteria for Immunoglobulin G4-related disease (IgG4RD) [24] in all cases, among which 125 cases of autoimmune pancreatitis (AIP) had been diagnosed based on the International Consensus Diagnostic Criteria 2011 [25] primarily due to characteristic imaging findings, high serum IgG4 concentration, the presence of extra-pancreatic lesions, and steroid responsiveness
Risk factors for periaortitis/periarteritis in IgG4-RD We analyzed for risk factors of periaortitis/periarteritis complications by comparing such clinical parameters as IgG4-RD onset age, gender, serum levels of various activity markers such as IgG4, IgG, complement proteins, soluble interleukin-2 receptor, rheumatoid factor (RF), and circulating immune complex (CIC), number of lesions, and history of corticosteroid therapy between patient groups with and without periaortitis/ periarteritis
Summary
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. Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition characterized by high serum immunoglobulin G4 (IgG4) concentration and IgG4-bearing plasma cell infiltration in affected organs [1,2,3] that is considered to be an immune-mediated disorder and possibly an autoimmune disease. The hallmark features of IgG4-RD can be summarized as systemic organ involvement, the ability to involve multiple organs either simultaneously or in a metachronous fashion, imaging findings of swelling, nodules, and/or increased organ wall thickness, elevated serum IgG4 concentration, lymphoplasmacytic and IgG4-bearing plasma cell infiltration within affected organs, and a generally favorable response to glucocorticoid therapy [1]. Intensive study of the aortic/arterial system has revealed another possible new disease concept, IgG4related periaortitis/periarteritis
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
