Elsevier

Autoimmunity Reviews

Volume 5, Issue 7, August 2006, Pages 458-464
Autoimmunity Reviews

Autoimmune aspects of chronic periaortitis

https://doi.org/10.1016/j.autrev.2006.03.011Get rights and content

Abstract

Chronic periaortitis (CP) includes idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis. These entities are characterised by a fibro-inflammatory tissue which develops around the abdominal aorta and the iliac arteries, and spreads into the surrounding retroperitoneum to entrap adjacent structures such as the ureters. CP often affects patients with advanced atherosclerosis, and several lines of evidence support the view that it could result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta such as oxidised-low density lipoproteins and ceroid. However, because most CP patients also suffer from constitutional symptoms and show elevated acute-phase reactant levels, positive autoantibodies and, in some cases, autoimmune diseases affecting other organs, CP may also be considered a manifestation of a systemic autoimmune disease.

CP is usually diagnosed using computed tomography or magnetic resonance imaging, but retroperitoneal biopsy may also be necessary; positron emission tomography is useful in assessing the full extent of the disease and the metabolic activity of the retroperitoneal tissue. Ureterolysis and aneurysm repair are frequently performed, but the inflammatory and chronic-relapsing nature of the disease often compels the use of medical therapy, which is based on steroids and immunosuppressants.

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:

  • CP is characterised by a periaortic fibro-inflammatory tissue and may be associated with aneurysmal dilatation of the aorta.

  • CP is often associated with advanced atherosclerosis and is proposed to arise from an exaggerated inflammatory response to atherosclerotic plaque antigens.

  • CP is linked

References (40)

  • R. Marcolongo et al.

    Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases

    Am J Med

    (2004)
  • L. Hechelhammer et al.

    Endovascular repair of inflammatory abdominal aneurysm: a retrospective analysis of CT follow-up

    J Vasc Interv Radiol

    (2005)
  • R.N. Jois et al.

    Chronic periaortitis

    Rheumatology (Oxford)

    (2004)
  • A. Vaglio et al.

    Chronic periaortitis: a spectrum of diseases

    Curr Opin Rheumatol

    (2005)
  • D.V. Parums

    The spectrum of chronic periaortitis

    Histopathology

    (1990)
  • A. Vaglio et al.

    Retroperitoneal fibrosis

    Lancet

    (2006)
  • D.A. Breems et al.

    The role of advanced atherosclerosis in idiopathic retroperitoneal fibrosis. Analysis of nine cases

    Neth J Med

    (2000)
  • T.E. Rasmussen et al.

    Inflammatory aortic aneurysms: a clinical review with new perspectives in pathogenesis

    Ann Surg

    (1997)
  • D. Martorana et al.

    Chronic periaortitis and HLA-DRB103: another clue to an autoimmune origin

    Arthritis Rheum

    (2006)
  • A. Davidson et al.

    Autoimmune diseases

    N Engl J Med

    (2001)
  • Cited by (73)

    • Unique Presentation of an Inflammatory Abdominal Aortic Aneurysm With Rhabdomyolysis

      2019, EJVES Short Reports
      Citation Excerpt :

      The etiology of IAAA remains unknown. Hypotheses range from unknown antigens within the atherosclerotic plaque to a generalised autoimmune process from low level autoantibodies.2,3 It is unknown whether the present patient had an unknown antigen that contributed to his IAAA, but he had no history of autoimmune diseases.

    • Imaging the pathological abdominal aorta

      2016, Feuillets de Radiologie
    • Retroperitoneal Fibrosis

      2013, Rheumatic Disease Clinics of North America
      Citation Excerpt :

      Twenty-five percent to 60% of patients with RPF will be positive for antinuclear antibodies.11,36 Vaglio and colleagues37 also reported the presence of other autoantibodies in patients with RPF. In a 2006 study they reported that 31% of patients with RPF have the presence of antithyroid antibodies, 14% have documented anti–smooth-muscle antibodies, 14% have rheumatoid factor, and 10% are positive for perinuclear or cytoplasmic antineutrophil cytoplasmic antibodies (ANCA).

    View all citing articles on Scopus
    View full text