Rethinking the Triggering Inflammatory Processes of Chronic Periaortitis

Abstract

Chronic periaortitis (CP) is an uncommon inflammatory disease which primarily involves the infrarenal portion of the abdominal aorta. However, CP should be regarded as a generalized disease with three different pathophysiological entities, namely idiopathic retroperitoneal fibrosis (RPF), inflammatory abdominal aortic aneurysm and perianeurysmal RPF. These entities share similar histopathological characteristics and finally will lead to fibrosis of the retroperitoneal space. Beside fibrosis, an infiltrate with variable chronic inflammatory cell is present. The majority of these cells are lymphocytes and macrophages as well as vascular endothelial cells, most of which are HLA-DR-positive. B and T cells are present with a majority of T cells of the T-helper phenotype. Cytokine gene expression analysis shows the presence of interleukin (IL)-1α, IL-2, IL-4, interferon-γ and IL-2 receptors. Adhesion molecules such as E-selectin, intercellular adhesion molecule-1 and the vascular cell adhesion molecule-1 were also found in aortic tissue, and may play a significant role in CP pathophysiology. Although CP pathogenesis remains unknown, an exaggeratedinflammatory response to advanced atherosclerosis (ATS) has been postulatedto be the main process. Autoimmunity has also beenproposed as a contributing factor based on immunohistochemical studies. The suspected allergen may be a component of ceroid, which is elaborated within the atheroma. We review the pathogenesis and the pathophysiology of CP, and its potential links with ATS. Clinically relevant issues are summarized in each section with regard to the current working hypothesis of this complex inflammatory disease.

Pathology of human plaque vulnerability: Mechanisms and consequences of intraplaque haemorrhages

“…Intracellular ceroids are also highly cytotoxic participating in the formation of the necrotic core [51]. Immunoglobulins are colocalized with ceroids in plaques [52] and ceroid antibodies are present in the serum of patients [53], suggesting that outwardly convected extracellular ceroids could serve as neo-antigens for promoting the adaptive immune response in the adventitia [54,55].…”

Autoantibodies against oxidized low-density lipoprotein and lipid profile in patients with chronic periaortitis: case–control study

“…Although the presence of these antibodies may be an epiphenomenon of little pathogenetic significance, the postulated pathogenesis of chronic periaortitis as being the result of an autoallergic reaction to OxLDL has since been adopted by many investigators (6)(7)(8). The described association with autoimmune disorders and the often observed beneficial response to immunosupressive treatment would further fit the proposed hypothesis (9,10).…”

“…Deranged immunological self-tolerance, possibly by genetic predisposition, may play a role (8,10,16). An association between chronic periaortitis and HLA-DRB1*03, an allele linked to several autoimmune conditions, was recently noted (42).…”

Chronic Periaortitis as a Systemic Autoimmune Disease

“…The majority of lymphocytes, macrophages and most vascular endothelial cells are HLA-DR-positive. The Ki67 and BerH2 staining is found in B cells and T-helper cells, indicating that these cells were proliferating and activated (Meier et al, 2007). Two main inflammatory patterns are usually seen, perivascular and diffuse.…”