Clinical study| Volume 114, ISSUE 6, P454-462, April 15, 2003

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Evidence of autoimmunity in chronic periaortitis: a prospective study



      Chronic periaortitis includes idiopathic retroperitoneal fibrosis, inflammatory aneurysms of the abdominal aorta, and perianeurysmal retroperitoneal fibrosis. It is considered to be due to advanced atherosclerosis, but is often associated with systemic autoimmune disorders.


      We studied 16 consecutive patients who were diagnosed with chronic periaortitis by computed tomography. Each patient underwent a physical examination, routine laboratory tests, measurement of autoantibodies, thyroid echotomography, and chest radiography. Aortic wall or periaortic retroperitoneal samples from 9 patients who underwent surgery were available for histologic examination and immunohistochemical characterization of the inflammatory infiltrate.


      Twelve patients had constitutional symptoms, 14 had an elevated erythrocyte sedimentation rate, and 13 had an elevated C-reactive protein level. Antinuclear antibodies were positive in 10 patients. Three patients had autoimmune thyroiditis, and 1 had seropositive rheumatoid arthritis. Antineutrophil cytoplasmic antibodies were positive in 3 patients who presented with rapidly progressive renal failure. Pathologic examination of the aortic and periaortic specimens revealed moderate to severe inflammatory infiltration, mainly consisting of B cells and CD4+ T cells. Vasculitis with fibrinoid necrosis involving the aortic vasa vasorum and the small and medium retroperitoneal vessels was found in seven of the nine histologic samples.


      These clinical and pathologic features support the hypothesis that, at least in some patients, chronic periaortitis is a systemic autoimmune disease, perhaps involving a vasculitic process of small and medium vessels.
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        • Mitchinson M.J.
        Chronic periaortitis and periarteritis.
        Histopathology. 1984; 8: 589-600
        • Parums D.V.
        The spectrum of chronic periaortitis.
        Histopathology. 1990; 16: 423-431
        • Gilkeson G.S.
        • Allen N.B.
        Retroperitoneal fibrosis. A true connective tissue disease.
        Rheum Dis Clin North Am. 1996; 22: 23-38
        • Walker D.I.
        • Bloor K.
        • Williams G.
        • Gillie I.
        Inflammatory aneurysms of the abdominal aorta.
        Br J Surg. 1972; 59: 609-614
        • Crawford J.L.
        • Stowe C.L.
        • Safi H.J.
        • Crawford E.S.
        Inflammatory aneurysms of the aorta.
        J Vasc Surg. 1985; 1: 113-124
      1. Gaylis H, Isaacson C. Abdominal aortic inflammatory aneurysms. In: Bergan J, Yao JST, eds. Aortic Surgery. Philadelphia: W.B. Saunders; 1989:267-292

        • Pennell R.C.
        • Hollier L.H.
        • Lie J.T.
        • et al.
        Inflammatory abdominal aortic aneurysms: a thirty-year review.
        J Vasc Surg. 1985; 2: 859-869
        • Serra R.M.
        • Engle J.E.
        • Jones R.E.
        • Schoolwerth A.C.
        Perianeurysmal retroperitoneal fibrosis. An unusual cause of acute renal failure.
        Am J Med. 1980; 68: 149-153
        • Parums D.V.
        • Brown D.L.
        • Mitchinson M.J.
        Serum antibodies to oxidized low-density lipoprotein in chronic periaortitis.
        Arch Pathol Lab Med. 1990; 114: 383-387
        • Ramshaw A.L.
        • Roskell D.E.
        • Parums D.V.
        Cytokine gene expression in aortic adventitial inflammation associated with advanced atherosclerosis (chronic periaortitis).
        J Clin Pathol. 1994; 47: 721-727
        • Ramshaw A.L.
        • Parums D.V.
        The distribution of adhesion molecules in chronic periaortitis.
        Histopathology. 1994; 24: 23-32
        • Demko T.M.
        • Diamond J.R.
        • Groff J.
        Obstructive nephropathy as a result of retroperitoneal fibrosis. A review of its pathogenesis and associations.
        J Am Soc Nephrol. 1997; 8: 684-688
        • Cohle S.D.
        • Lie J.T.
        Inflammatory aneurysms of the aorta, aortitis and coronary arteritis.
        Arch Pathol Lab Med. 1988; 112: 1121-1125
        • De La Iglesia Martinez F.
        • Grana Gil J.
        • Gomez Veiga F.
        • et al.
        The association of retroperitoneal fibrosis and ankylosing spondylitis.
        J Rheumatol. 1992; 19: 1147-1149
        • Ter Maaten J.C.
        • Franssen C.F.M.
        • Daenekindt A.A.
        • Hoorntje S.J.
        Triple Wegener’s granulomatosis in the urogenital tract.
        Nephron. 1993; 63: 358-359
        • Kaipiainen-Seppanen O.
        • Jantunen E.
        • Kuusisto J.
        • Martin S.
        Retroperitoneal fibrosis with antineutrophil cytoplasmic antibodies.
        J Rheumatol. 1996; 23: 779-781
        • Parums D.V.
        • Chadwick D.R.
        • Mitchinson M.J.
        The localization of immunoglobulin in chronic periaortitis.
        Atherosclerosis. 1986; 61: 117-123
        • Poitras J.M.
        • Dennis G.J.
        Clinical images: diagnosis and treatment of retroperitoneal fibrosis (Ormond’s disease).
        Arthritis Rheum. 2000; 43: 943
        • Tsai T.C.
        • Chang P.Y.
        • Chen B.F.
        • et al.
        Retroperitoneal fibrosis and juvenile rheumatoid arthritis.
        Pediatr Nephrol. 1996; 10: 208-209
        • Mitchinson M.J.
        The pathology of idiopathic retroperitoneal fibrosis.
        J Clin Pathol. 1970; 23: 681-689
        • Dehner L.P.
        • Coffin C.M.
        Idiopathic fibrosclerotic disorders and other inflammatory pseudotumors.
        Semin Diagn Pathol. 1998; 15: 161-173
        • Vaglio A.
        • Manenti L.
        • Allegri L.
        • et al.
        ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?.
        J Intern Med. 2002; 251: 268-271
        • Savige J.
        • Davies D.
        • Falk R.J.
        • et al.
        Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features.
        Kidney Int. 2000; 57: 846-862
        • De Roux-Serratrice C.
        • Serratrice J.
        • Granel B.
        • et al.
        Periaortitis heralding Wegener’s granulomatosis.
        J Rheumatol. 2002; 29: 392-394
        • Hughes D.
        • Buckley P.J.
        Idiopathic retroperitoneal fibrosis is a macrophage-rich process. Implications for its pathogenesis and treatment.
        Am J Surg Pathol. 1993; 17: 482-490
        • Pasquinelli G.
        • Preda P.
        • Gargiulo M.
        • et al.
        An immunohistochemical study of inflammatory abdominal aortic aneurysms.
        J Submicrosc Cytol Pathol. 1993; 25: 103-112
        • Hofbauer L.C.
        • Magerstadt R.A.
        • Heufelder A.E.
        Hepatitis C related cryoglobulinemia associated with retroperitoneal fibrosis.
        J Rheumatol. 1996; 23: 554-557
        • Akman N.
        • Avanoglu Y.
        • Karabay K.
        • et al.
        Henoch-Schönlein purpura and retroperitoneal fibrosis.
        Acta Haematol. 1983; 70: 400-401
        • Hautekeete M.L.
        • Babany G.
        • Marcellin P.
        • et al.
        Retroperitoneal fibrosis after surgery for aortic aneurysm in a patient with periarteritis nodosa: successful treatment with corticosteroids.
        J Intern Med. 1990; 228: 533-536
        • Littlejohn G.O.
        • Keystone E.C.
        The association of retroperitoneal fibrosis with systemic vasculitis and HLA-B27: a case report and a review of the literature.
        J Rheumatol. 1981; 8: 665-669
        • Lindell O.I.
        • Sariola H.V.
        • Lehtonen T.A.
        The occurrence of vasculitis in perianeurysmal fibrosis.
        J Urol. 1987; 138: 727-729
        • Numano F.
        Vasa vasoritis, vasculitis and atherosclerosis.
        Int J Cardiol. 2000; 75: S1-S8
        • Kobayashi M.
        • Ito M.
        • Nakagawa A.
        • et al.
        Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behçet disease.
        Histopathology. 2000; 36: 362-371