Aortic diseases comprise aneurysms, dissections, and several other pathologies. In general, aging is associated with a slow but progressive dilation of the aorta, along with increased stiffness and pulse pressure. The progression of aortic disease is characterized by subclinical development or acute presentation. Recent evidence suggests that inflammation participates causally in different clinical manifestations of aortic diseases. As of yet, diagnostic imaging and surveillance is mainly based on ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI). Little medical therapy is available so far to prevent or treat the majority of aortic diseases. Endovascular therapy by the introduction of covered stentgrafts provides the main treatment option, although open surgery and implantation of synthetic grafts remain necessary in many situations. Because of the risks associated with surgery, there is a need for identification of pharmaceutical targets interfering with the pathophysiology of aortic remodeling. The participation of innate immunity and inflammasome activation in different cell types is common in aortic diseases. This review will thus focus on inflammasome activities in vascular cells of different chronic and acute aortic diseases and discuss their role in development and progression. We will also identify research gaps and suggest promising therapeutic targets, which may be used for future medical interventions.

Background: Although patients with multiple arterial dissections in distinct arterial regions rarely present with known connective tissue syndromes, we hypothesized that mild connective tissue abnormalities are common findings in these patients. Methods: From a consecutive register of 322 patients with cervical artery dissection (CeAD), we identified and analyzed 4 patients with a history of additional dissections in other vascular beds. In three patients, dermal connective tissue was examined by electron microscopy. DNA from all four patients was studied by whole-exome sequencing and copy number variation (CNV) analysis. Results: The collagen fibers of dermal biopsies were pathologic in all three analyzed patients. One patient carried a CNV disrupting the COL3A1 and COL5A2 genes (vascular or hypermobility type of Ehlers–Danlos syndrome), and another patient a CNV in MYH11 (familial thoracic aortic aneurysms and dissections). The third patient carried a missense substitution in COL5A2. Conclusion: Three patients showed morphologic alterations of the dermal connective tissue, and two patients carried pathogenic variants in genes associated with arterial connective tissue dysfunction. The findings suggest that genetic testing should be recommended after recurrent arterial dissections, independently of apparent phenotypical signs of connective tissue disorders.

The aim of this study was to investigate sex-dependent aneurysm distributions. A total of 3107 patients with arterial aneurysms were diagnosed from 2006 to 2016. Patients with anything other than true aneurysms, hereditary connective tissue disorders or vasculitides (n = 918) were excluded. Affected arterial sites and age at first aneurysm diagnosis were compared between women and men by an unpaired two-tailed t-test and Fisher’s exact test. The study sample consisted of 2189 patients, of whom 1873 were men (85.6%) and 316 women (14.4%) (ratio m:w = 5.9:1). Men had considerably more aneurysms in the abdominal aorta (83.4% vs. 71.1%; p < 0.001), common iliac artery (28.7% vs. 8.9%; p < 0.001), internal iliac artery (6.6% vs. 1.3%; p < 0.001) and popliteal artery (11.1% vs. 2.5%; p < 0.001). In contrast, women had a higher proportion of aneurysms in the ascending aorta (4.4% vs. 10.8%; p < 0.001), descending aorta (11.1% vs. 36.4%; p < 0.001), splenic artery (0.9% vs. 5.1%; p < 0.001) and renal artery (0.8% vs. 6.0%; p < 0.001). Age at disease onset and further aneurysm distribution showed no considerable difference. The infrarenal segment might be considered a natural border for aneurysm formation in men and women suspected to have distinct genetic, pathophysiologic and ontogenetic factors. Screening modalities for women at risk might need further adjustment, particularly thoracic cross-sectional imaging complementation.

The aim of this study was to investigate histopathological differences in abdominal aortic aneurysms (AAAs) between patients with multiple and single arterial aneurysms, as we suspect that there are different underlying mechanisms in aneurysm formation. Analysis was based on a previous retrospective study on patients with multiple arterial aneurysms (mult-AA; defined as at least four, n = 143) and a single AAA (sing-AAA, n = 972) who were admitted to our hospital for treatment between 2006 and 2016. Available paraffin-embedded AAA wall specimens were derived from the Vascular Biomaterial Bank Heidelberg (mult-AA, n = 12 vs. sing-AAA, n = 19). Sections were analyzed regarding structural damage of the fibrous connective tissue and inflammatory cell infiltration. Alterations to the collagen and elastin constitution were assessed by Masson–Goldner trichrome and Elastica van Gieson staining. Inflammatory cell infiltration, response and transformation were assessed by CD45 and IL-1β immunohistochemistry and von Kossa staining. The extent of aneurysmal wall alterations was assessed by semiquantitative gradings and was compared between the groups using Fisher’s exact test. IL-1β was significantly more present in the tunica media in mult-AA compared to sing-AAA (p = 0.022). The increased expression of IL-1β in mult-AA compared to sing-AAA indicates inflammatory processes play a role in aneurysm formation in patients with multiple arterial aneurysms.

Summary: Background: The aim of this retrospective cross-sectional observational study was to determine differences of patients with multiple arterial aneurysms to patients with single arterial aneurysms. Patients and methods: Patients with the diagnosis of an arterial aneurysm from January 2006 to January 2016 in the department of vascular surgery Heidelberg were investigated. Excluded were patients with hereditary disorders of connective tissue or systemic inflammatory disease, as well as other arterial pathologies than true aneurysms. Patients with multiple aneurysms (defined by at least four aneurysms) were compared to patients with single aneurysms concerning age at initial diagnosis, sex and affected arterial site. To verify the findings, a replication of the study was performed at a comparable institution. Results: Of 3107 patients with arterial aneurysms, 918 were excluded. Of the resulting 2189 patients, 1238 (56.6%) patients had a single, 808 (36.9%) two or three, and 143 (6.5%) at least four aneurysms (group mult-AA). Nine hundred seventy-two patients (44.4%) had a single abdominal aortic aneurysm (group sing-AAA). Age at initial diagnosis differed between mult-AA (66.7±9.5 y) and sing-AAA (69.1±8.6 y) (p=0.0338). Within mult-AA, 138 patients (96.5%) were male, compared with 865 patients (89.0%) in sing-AAA (p=0.0041). The most frequent aneurysm localization shifted from the abdominal aorta and its branches in patients with a single aneurysm (n=1029; 83.1%) to pelvic and leg arteries in patients with at least four aneurysms (n=318; 63.2%). The replication of the study at the department of vascular surgery Frankfurt confirmed the younger age at initial diagnosis in mult-AA (67.3±12.5 y) compared to sing-AAA (70.9±9.6 y) (p=0.0259) and the distribution shift toward the arteries below the aortic bifurcation in mult-AA. Conclusions: Patients with multiple aneurysms are younger at initial diagnosis and differ concerning aneurysm localization compared to patients with a single aneurysm.

Summary: Background: The aim of this retrospective single-centre cross-sectional observational study was to investigate co-prevalence of arterial aneurysm location systematically. Patients and methods: Patients with the diagnosis of any arterial aneurysm from January 2006 to January 2016 were investigated in a single centre. Patients with hereditary disorders of connective tissue, systemic inflammatory disease, or arterial pathologies other than true aneurysms were excluded. Aneurysm locations were assessed for every patient included. For patients with at least two co-existing aneurysms, co-prevalence of aneurysm location was investigated by calculating correlation coefficients and applying Fisher’s exact test. This study report is prepared according to the STROBE statement. Results: Of 3107 identified patients with arterial aneurysms, 918 were excluded. Of the remaining 2189 patients, 951 patients with at least two aneurysms were included in the study. Bilateral aneurysm combinations of paired iliac, femoral and popliteal arteries showed the highest correlation (ϕ=0.35 to 0.67), followed by bilateral combinations of subclavian (ϕ=0.36) and internal carotid (ϕ=0.38) arteries. Abdominal aortic aneurysms in combination with visceral artery aneurysms (ϕ=−0.24 to −0.12), popliteal arteries (ϕ=−0.22) and the ascending aorta (ϕ=−0.19) showed the lowest correlation, followed by the descending aorta in combination with the common iliac arteries (ϕ=−0.12 to −0.13). Conclusions: In our study sample, aneurysm co-prevalence was highly non-random. This should be considered in the context of aneurysm screening programs.