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p-ANCA-associated periaortitis with histological proof of Wegener’s granulomatosis: case report

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Abstract

We describe a 63-year-old man who presented with an inflammatory aortic aneurysm. The patient had p-ANCA antibodies directed against myeloperoxidase. A diagnosis of idiopathic periaortitis was made. Seven years later, he was rehospitalized because of fever, weight loss, and polyneuropathy. After revision of the aortic biopsy, which showed necrotizing vasculitis with palisading granuloma, a diagnosis of Wegener’s granulomatosis (WG) was made. This case report illustrates an unusual disease course in WG, resembling large vessel vasculitis, and we discuss the possible mechanisms of large vessel involvement in this form of vasculitis.

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References

  1. Leavitt RY, Fauci AS, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107

    Article  CAS  PubMed  Google Scholar 

  2. Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192

    Article  CAS  PubMed  Google Scholar 

  3. Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98:76–85

    Article  CAS  PubMed  Google Scholar 

  4. Falk RJ, Jennette JC (1988) Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescenteric glomerulonephritis. N Engl J Med 25:1651–1657

    Article  Google Scholar 

  5. Blockmans D (2003) The use of (18F)fluoro-deoxyglucose positron emission tomography in the assessment of large vessel vasculitis. Clin Exp Rheumatol 21 [Suppl 32]:S15–S22

    CAS  PubMed  Google Scholar 

  6. Nakabayashi K, Kamiya Y, Nagasawa T (2000) Aortitis syndrome associated with positive perinuclear antineutrophil cytoplasm antibody: report of three cases. Int J Cardiol 75 [Suppl 1]:S89–S94

    Article  PubMed  Google Scholar 

  7. Sakemi T, Tomiyoshi Y, Yano H, Ikeda Y, Matsuo Y, Kudo S (1998) Retroperitoneal fibrosis with perinuclear antineutrophil cytoplasmic antibodies and a longitudinally extended periaortic soft-tissue structure on CT. Nephron 78:218–220

    Article  CAS  PubMed  Google Scholar 

  8. Vaglio A, Corradi D, Manenti L, Ferretti S, Garini G, Buzio C (2003) Evidence of autoimmunity in chronic periaortitis: a prospectieve study. Am J Med 114:454–462

    Article  PubMed  Google Scholar 

  9. Fink AM, Miles KA, Wraight EP (1994) Indium-111 labelled leucocyte uptake in aortitis. Clin Radiol 49:863–866

    Article  CAS  PubMed  Google Scholar 

  10. Sieber SC, Cuello B, Gelfman NA, Garfinkel HB (1990) Pulmonary capillaritis and glomerulonephritis in an antineutrophil cytoplasmic antibody-positive patient with prior granulomatous aortitis. Arch Pathol Lab Med 114:1223–1226

    CAS  PubMed  Google Scholar 

  11. Blockmans D, Baeyens H, Van Loon R, Lauwers G, Bobbaers H (2000) Periaortitis and aortic dissection due to Wegener’s granulomatosis. Clin Rheumatol 19:161–164

    Article  CAS  PubMed  Google Scholar 

  12. de Roux-Serratrice C, Serratrice J, Granel B, Disdier P, Bartoli J-M, Pache X, Astoul P, Carbe L, Branchereau A, Weiller P-J (2002) Periaortitis heralding Wegener’s granulomatosis. J Rheumatol 29:392–394

    Google Scholar 

  13. Pasquinelli G, Preda P, Gargiulo M et al (1993) An immunohistochemical study of inflammatory abdominal aortic aneurysms. J Submicrosc Cytol Pathol 25:103–112

    CAS  PubMed  Google Scholar 

  14. Toshiyuki I, Katsumi E, Masahiro S, Shigenobu N, Mitsuaki I, Kan T (1992) Polyarteritis nodosa with aortic dissection: necrotizing vasculitis of the vasa vasorum. J Rheumatol 19:1632–1636

    Google Scholar 

  15. Nishino H, DeRemee RA, Rubino FA, Parisi JE (1993) Wegener’s granulomatosis associated with vasculitis of the temporal artery: report of 5 cases. Mayo Clin Proc 68:194–196

    Article  Google Scholar 

  16. Genereau T, Lortholary O, Pottier MA et al (1999) Temporal artery biopsy: a diagnostic tool for systemic necrotizing vasculitis. French Vaculitis Study Group. Arthritis Rheum 42:2674–2681

    Article  CAS  PubMed  Google Scholar 

  17. Esteban MJ, Font C, Hernandez-Rodriguez J et al (2001) Small-vessel vasculitis surrounding a spared temporal artery: clinical and pathological findings in a series of twenty-eight patients. Arthritis Rheum 44:1387–1395

    Article  CAS  PubMed  Google Scholar 

  18. Hamidou MA, Moreau A, Toquet C, El Kouri D, De Faucal P, Grolleau JY (2003) Temporal arteritis associated with systemic necrotizing vasculitis. J Rheumatol 30:2165–2169

    PubMed  Google Scholar 

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Carels, T., Verbeken, E. & Blockmans, D. p-ANCA-associated periaortitis with histological proof of Wegener’s granulomatosis: case report. Clin Rheumatol 24, 83–86 (2005). https://doi.org/10.1007/s10067-004-0998-0

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  • DOI: https://doi.org/10.1007/s10067-004-0998-0

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