Autoimmune aspects of chronic periaortitis
Section snippets
Epidemiologic and genetic aspects
CP is a rare disorder; although there are no conclusive data concerning its epidemiologic characteristics, a recent study on IRF has demonstrated that its incidence and prevalence are 1/1,000,000 person-year and 1.38 cases/100,000 inhabitants [7]. On the other hand, studies on the aneurysmal forms of CP have shown that they represent about 3% to 10% of the total abdominal aortic aneurysms [8], [9].
The male/female ratio is usually 2:1–3:1, with the higher incidence in the male patients being
Histopathological aspects
CP is a fibro-inflammatory disease which affects the aortic wall and the surrounding retroperitoneum. It grossly appears as a white and firm periaortic mass extending between the origin of the renal arteries and the bifurcation of the common iliac vessels [13].
The pathological examination of the retroperitoneum reveals a mixture of chronic inflammation and fibrous tissue (Fig. 1 A); the proportions of these two components may vary with the disease stage: in the active phases, the
Clinical manifestations and laboratory findings
CP usually presents with abdominal, back or lumbar pain, which is often dull but, in patients with ureteral involvement, also colic-like. Pain is usually associated with constitutional symptoms, such as malaise, anorexia and fever. Other presenting signs or symptoms include leg edema, varicocele, testicular pain, claudication, dysuria, frequency and, in cases with advanced bilateral ureteral stenosis, oligo-anuria [1], [4], [6], [8], [18]. Thrombophlebitis or deep vein thrombosis may also
Association with autoimmune diseases
The view that CP has an autoimmune origin is supported by its frequent association with autoimmune diseases involving other organs or structures. In a study on a series of 16 consecutive CP patients, we reported that three of them had ANCA-positive rapidly progressive renal failure, three Hashimoto's thyroiditis and one rheumatoid arthritis [6]. In a case-control study comparing classic atherosclerotic abdominal aortic aneurysms and IAAAs, a higher incidence of systemic autoimmune or
Pathogenesis
A series of studies conducted by Parums and Mitchinson led to the hypothesis that CP arises as a result of a local reaction to antigens such as ceroid and oxidised low-density lipoproteins (LDL), which can be found in the atherosclerotic plaques of the abdominal aorta [3], [16], [22], [23]. According to this hypothesis, these lipids are elaborated within plaque macrophages and then presented to T- and B-cells, thus eliciting a local inflammatory reaction which eventually leads to adventitial
Diagnosis
Computed tomography (CT) and magnetic resonance imaging (MRI) are judged the modalities of choice for the diagnosis of CP. It is still controversial whether patients diagnosed as having CP on the basis of CT/MRI require retroperitoneal biopsy; however, biopsy should be considered mandatory when the retroperitoneal mass has atypical localisations, such as pelvic, peri-pancreatic or peri-ureteral [4].
On CT, CP appears isodense with muscle; it develops around the anterior and lateral sides of the
Treatment
The treatment of CP is largely based on the use of corticosteroids. In most patients, they induce remission of the clinical symptoms, normalisation of the acute-phase reactant levels, reduction in size of the retroperitoneal mass and also resolution of the obstructive complications [1], [4], [18], [22], [35]. Given the lack of prospective randomised trials, the best dose, administration route, and duration of steroid therapy have not yet been established. Corticosteroids have significant side
Conclusions
CP represents a complex spectrum of rare diseases, and it is likely that both atherosclerosis and a generalised perturbation of self-tolerance contribute to its pathogenesis. Further studies are warranted in order to elucidate the potential triggers of the disease, the pathways leading to the aortic-periaortic inflammation and to the disproportionate fibrogenic reaction. Additionally, prospective clinical studies are needed to establish the most effective and safest therapeutic approaches as
Acknowledgments
The authors would like to thank Dr. Pietro Schianchi for his assistance in the preparation of the images and Kisha Piggott for her revision of the English language used in the manuscript.
Take-home messages:
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CP is characterised by a periaortic fibro-inflammatory tissue and may be associated with aneurysmal dilatation of the aorta.
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CP is often associated with advanced atherosclerosis and is proposed to arise from an exaggerated inflammatory response to atherosclerotic plaque antigens.
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CP is linked
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