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Dramatic regression of mesenteric abnormalities demonstrated on angiography following prednisolone and cyclophosphamide combination therapy in a patient with polyarteritis nodosa associated with Sjögren’s syndrome

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Modern Rheumatology

Abstract

A 63-year-old woman, who had been followed for Sjögren’s syndrome, was admitted due to cryoglobulinemia, leukocytoclastic vasculitis, and mononeuritis multiplexa. In spite of the administration of 60 mg prednisolone, fecal occult blood was strongly positive. The colonoscopy showed multiple colonic ulcers, and a diagnosis of polyarteritis nodosa (PAN) was made because abdominal angiography revealed markedly serpentine and narrowed superior and inferior mesenteric arteries. After steroid pulse therapy and daily oral administration of cyclophosphamide were initiated, her symptoms improved and abdominal angiographic findings were finally normalized. Although there are only three case reports on improvements in abdominal angiographic findings of PAN in the literature, our case and previously reported cases suggest that improvements in angiographic findings may reflect a good prognosis of PAN.

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References

  1. García-Carrasco M, Ramos-Casals M, Rosas J, Pallarés L, Calvo-Alen J, Cervera R, et al. Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). 2002;81:270–80.

    Article  Google Scholar 

  2. Soliotis FC, Moutsopoulos HM. Sjogren’s syndrome. Autoimmunity. 2004;37:305–7.

    Article  PubMed  CAS  Google Scholar 

  3. Lotti TM, Comacchi C, Ghersetich I. Cutaneous necrotizing vasculitis. Relation to systemic disease. Adv Exp Med Biol. 1999;455:115–25.

    PubMed  CAS  Google Scholar 

  4. Ramos-Casals M, Anaya JM, García-Carrasco M, Rosas J, Bové A, Claver G, et al. Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients. Medicine (Baltimore). 2004;83:96–106.

    Article  Google Scholar 

  5. Ferri C, Mascia MT. Cryoglobulinemic vasculitis. Curr Opin Rheumatol. 2006;18:54–63.

    PubMed  Google Scholar 

  6. Inoh M, Tokuda M, Maruoka T, Kinashi M, Kurata N, Okawa M, et al. Multiple colonic ulcers in a patient with Sjögren’s syndrome (in Japanese). Ryumachi. 1995;35:585–8.

    PubMed  CAS  Google Scholar 

  7. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33:1088–93.

    Google Scholar 

  8. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–92.

    Article  PubMed  CAS  Google Scholar 

  9. Fauci AS, Doppman JL, Wolff SM. Cyclophosphamide-induced remissions in advanced polyarteritis nodosa. Am J Med. 1978;64:890–4.

    Article  PubMed  CAS  Google Scholar 

  10. Fauci AS, Katz P, Haynes BF, Wolff SM. Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med. 1979;301:235–8.

    PubMed  CAS  Google Scholar 

  11. Leib ES, Restivo C, Paulus HE. Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med. 1979;67:941–7.

    Article  PubMed  CAS  Google Scholar 

  12. Oriente P, Riccio A, Farinaro C, Farinaro E, Scarpa R, Vignone L, et al. Cyclophosphamide treatment in polyarteritis nodosa. Clin Rheumatol. 1986;5:193–200.

    Article  PubMed  CAS  Google Scholar 

  13. Guillevin L, Cohen P, Mahr A, Arène JP, Mouthon L, Puéchal X, et al. Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients. Arthritis Rheum. 2003;49:93–100.

    Article  PubMed  CAS  Google Scholar 

  14. Hekali P, Kajander H, Pajari R, Stenman S, Somer T. Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa. Acta Radiol. 1991;32:143–8.

    Article  PubMed  CAS  Google Scholar 

  15. Vázquez JJ, San Martín P, Barbado FJ, Gil A, Guerra J, Arnalich F, et al. Angiographic findings in systemic necrotizing vasculitis. Angiology. 1981;32:773–9.

    Article  PubMed  Google Scholar 

  16. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75:17–28.

    Article  CAS  Google Scholar 

  17. Terrier B, Lacroix C, Guillevin L, Hatron PY, Dhote R, Maillot F, et al. Club Rhumatismes et Inflammation. Diagnostic and prognostic relevance of neuromuscular biopsy in primary Sjögren’s syndrome-related neuropathy. Arthritis Rheum. 2007;57:1520–9.

    Article  PubMed  Google Scholar 

  18. García-Bragado F, Vilardell M, Fonollosa V, Gallart MT, Rodrigo MJ, Tornos J. Cryoglobulins in systemic and rheumatological diseases. Report of 70 cases (author’s transl) (in Spanish). Med Clin (Barc). 1980;74:209–13.

    Google Scholar 

  19. Lamprecht P, Gause A, Gross WL. Cryoglobulinemic vasculitis. Arthritis Rheum. 1999;42:2507–16.

    Article  PubMed  CAS  Google Scholar 

  20. Travers RL, Allison DJ, Brettle RP, Hughes GR. Polyarteritis nodosa: a clinical and angiographic analysis of 17 cases. Semin Arthritis Rheum. 1979;8:184–99.

    Article  PubMed  CAS  Google Scholar 

  21. Sellar RJ, Mackay IG, Buist TA. The incidence of microaneurysms in polyarteritis nodosa. Cardiovasc Intervent Radiol. 1986;9:123–6.

    Article  PubMed  CAS  Google Scholar 

  22. Ewald EA, Griffin D, McCune WJ. Correlation of angiographic abnormalities with disease manifestations and disease severity in polyarteritis nodosa. J Rheumatol. 1987;14:952–6.

    PubMed  CAS  Google Scholar 

  23. Robins JM, Bookstein JJ. Regressing aneurysms in periarteritis nodosa. A report of 3 cases. Radiolog. 1972;104:39–42.

    CAS  Google Scholar 

  24. Kato H, Koike S, Yamamoto M, Ito Y, Yano E. Coronary aneurysms in infants and young children with acute febrile mucocutaneous lymph node syndrome. J Pediatr. 1975;86:892–8.

    Article  PubMed  CAS  Google Scholar 

  25. McClure PH, Westcott JL. Periarteritis nodosa with perirenal hemorrhage: a case report with angiographic findings. J Urol. 1969;102:126–9.

    PubMed  CAS  Google Scholar 

  26. Hagspiel KD, Angle JF, Spinosa DJ, Matsumoto AH. Diagnosis please. Case 13: polyarteritis nodosa–systemic necrotizing vasculitis with involvement of hepatic and superior mesenteric arteries. Radiology. 1999;212:359–64.

    PubMed  CAS  Google Scholar 

  27. Yamada H, Oka M, Mori S, Takahashi T, Kuwata S, Aizawa C, et al. A young female case of polyarteritis nodosa strongly suspected by typical angiographic findings which improved rapidly after prednisolone and cyclophosphamide therapy. Intern Med. 1992;31:431–3.

    Article  PubMed  CAS  Google Scholar 

  28. Harada M, Yoshida H, Ikeda H, Yoshida T, Takedatsu H, Nagamatsu H, et al. Polyarthritis nodosa with mesenteric aneurysms demonstrated by angiography: report of a case and successful treatment of the patient with prednisolone and cyclophosphamide. J Gastroenterol. 1999;34:702–5.

    Article  PubMed  CAS  Google Scholar 

  29. Raza K, Carruthers DM, Exley AR, Thambyrajah J, Bacon PA, Rowe IF. Dramatic aneurysm regression in polyarteritis nodosa following high dose pulse cyclophosphamide. J Rheumatol. 2000;27:1320–1.

    PubMed  CAS  Google Scholar 

  30. Hughes LB, Bridges SL Jr. Polyarteritis nodosa and microscopic polyangiitis: etiologic and diagnostic considerations. Curr Rheumatol Rep. 2002;4(1):75–82.

    Article  PubMed  Google Scholar 

  31. Rose GA. The natural history of polyarteritis. Br Med J. 1957;2:1148–52.

    Article  PubMed  CAS  Google Scholar 

  32. Frohnert PP, Sheps SG. Long-term follow-up study of periarteritis nodosa. Am J Med. 1967;43:8–14.

    Article  PubMed  CAS  Google Scholar 

  33. Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001;44:666–75.

    Article  PubMed  CAS  Google Scholar 

  34. Bourgarit A, Le Toumelin P, Pagnoux C, Cohen P, Mahr A, Le Guern V, et al. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine (Baltimore). 2005;84:323–30.

    Article  Google Scholar 

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Correspondence to Sonosuke Yukawa.

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Yukawa, S., Tahara, K., Yukawa, N. et al. Dramatic regression of mesenteric abnormalities demonstrated on angiography following prednisolone and cyclophosphamide combination therapy in a patient with polyarteritis nodosa associated with Sjögren’s syndrome. Mod Rheumatol 18, 416–421 (2008). https://doi.org/10.1007/s10165-008-0072-x

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  • DOI: https://doi.org/10.1007/s10165-008-0072-x

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