Abdominal aortic aneurysm as an IgG4-related disease

Abstract

Summary 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.

The Incidence of IgG4-Related and Inflammatory Abdominal Aortic Aneurysm Is Rare in a 101 Patient Cohort

“…Despite this high IgG4 burden in studies from Japan, Koo et al reported 3 out of 29 positive aortic cases (10.9%) from Korea [ 29 ]. The largest study to date stems from the Czech Republic and reported 7 out of 114 AAA patient samples (6.1%) that were positive for IgG4-related disease, comparable to the results shown here ( Table 1 , Figure 1 , Supplementary Figure S1 ) [ 13 ]. Hence, one could hypothesize a different disease distribution over different ethnicities; this may, however, harbor selection and reporting bias, also including aneurysm sizes and emergency procedures, when, i.e., no sample can be acquired during surgery.…”

“…Here, the involvement of the recognized systemic inflammatory disorder known as immunoglobulin G4 (IgG4)-related disease has been linked to AAA by many groups, suggesting approx. 5–10% of all AAAs; however, up to 57% of inflAAA cohorts appear to be IgG4 positive to varying degrees [ 12 , 13 , 14 , 15 , 16 ]. Hence, diagnostic criteria involving both histology and blood sampling have been established to define inflammatory and IgG4-positive AAA.…”

AAA Revisited: A Comprehensive Review of Risk Factors, Management, and Hallmarks of Pathogenesis

“…IFN-γ and IL-17 secreted by CD8+ T-cells and Th17 CD4+ T-cells have also been implicated in AAA pathogenesis [ 394 , 395 , 396 ]. B-cell-derived immunoglobulins such as IgG4 and IgE drive macrophage and mast cell activation and degranulation [ 397 , 398 ]. Regulatory CD4+ T-cells have a protective function by releasing anti-inflammatory cytokines such as IL-10 and TGF-β [ 399 , 400 , 401 ], but both regulatory CD4+ cells and their mediators are reduced in AAA patients [ 402 ].…”

Masses orbitaires bilatérales révélant une maladie associée aux Ig G4 au Burkina Faso.

“…Les nodules cardiaques sont très rares dans la maladie associée aux Ig G4 [21]. Des atteintes anévrysmales des artères coronaires, de l'aorte et des cas d' aortite ont été décrites [22][23][24][25].En fonction de la localisation des nodules, on peut noter un retentissement sur la fonction cardiaque [21].…”