Clinical and Experimental Nephrology

, Volume 13, Issue 5, pp 512–517 | Cite as

Fatal hemoperitoneum due to rupture of the left gastric artery in a patient with microscopic polyangiitis

  • Kensei Yahata
  • Chinatsu Okamoto
  • Hirotaka Imamaki
  • Koichi Seta
  • Tsuyoshi Terashima
  • Sachiko Minamiguchi
  • Akira Sugawara
Case Report


Microscopic polyangiitis is a vasculitis which primarily affects capillaries, venules or arterioles. Involvement of small and medium-sized arteries may also occur. A 70-year-old Japanese female with a fever and cough was diagnosed with pneumonia and antibiotics were administered. Her symptoms initially improved, but her fever recurred and she experienced malaise and loss of appetite. Her renal function gradually worsened and she was positive for myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA). She was referred to our hospital on the suspicion of ANCA-associated glomerulonephritis. However, her depressive mental symptoms did not allow her to undergo a renal biopsy. She was clinically diagnosed with ANCA-associated glomerulonephritis, and oral corticosteroids and intravenous methylprednisolone were administered. Her symptoms and renal function were improved, but she died suddenly 15 days after admission. An autopsy disclosed approximately 700 mL bloody ascites. Coagulation adhered to the lesser curvature of the stomach, but the source of hemorrhage could not be detected macroscopically because the gastric mucosa did not show abnormal findings. The histological findings revealed that the left gastric artery showed necrotizing angiitis and rupture. In the kidneys, cellular crescents were found in approximately 10%, fibrous crescents were found in approximately 10%, sclerosis and collapse were found approximately 30% of the glomeruli, and necrotizing angiitis was observed in interlobular arteries and arterioles. From these findings, she was finally diagnosed with microscopic polyangiitis. Microscopic polyangiitis is an extremely rare cause of spontaneous intraperitoneal bleeding, but it must be carefully considered in the differential diagnosis for the appropriate management of such patients.


Microscopic polyangiitis Antineutrophil cytoplasmic antibodies (ANCA) Rapidly progressive glomerulonephritis (RPGN) Hemoperitoneum 


  1. 1.
    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.PubMedCrossRefGoogle Scholar
  2. 2.
    Gross WL, Schmitt WH, Csernok E. ANCA and associated diseases: immunodiagnostic and pathogenetic aspects. Clin Exp Immunol. 1993;91:1–12.PubMedGoogle Scholar
  3. 3.
    Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW. Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential. Kidney Int. 1994;46:1–15.PubMedCrossRefGoogle Scholar
  4. 4.
    Pettersson EE, Sundelin B, Heigl Z. Incidence and outcome of pauci-immune necrotizing and crescentic glomerulonephritis in adults. Clin Nephrol. 1995;43:141–9.PubMedGoogle Scholar
  5. 5.
    Guillevin L, Lhote F, Amouroux J, Gherardi R, Callard P, Casassus P. Antineutrophil cytoplasmic antibodies, abnormal angiograms and pathological findings in polyarteritis nodosa and Churg-Strauss syndrome: indications for the classification of vasculitides of the polyarteritis Nodosa Group. Br J Rheumatol. 1996;35:958–64.PubMedCrossRefGoogle Scholar
  6. 6.
    Davson J, Ball J, Platt R. The kidney in periarteritis nodosa. Q J Med. 1948;17:175–202.PubMedGoogle Scholar
  7. 7.
    Sneller MC, Langford CA, Fauci AS. The vasculitis syndromes. In: Kasper DL, Fauci AS, Longo DL, Braunwald E, Hauser SL, Jameson JL, editors. Harrison’s principles of internal medicine, 16th ed. New York: McGraw-Hill; 2004. p. 2002–14.Google Scholar
  8. 8.
    Franssen CF, Ter Maaten JC, Hoorntje SJ. Spontaneous splenic rupture in Wegener’s vasculitis. Ann Rheum Dis. 1993;52:314.PubMedCrossRefGoogle Scholar
  9. 9.
    Takamatsu K, Ikeda Y, Nakauchi Y, Kawada M, Hashimoto K, Furihata M. Henoch-Schonlein purpura with rapidly progressive glomerulonephritis and fatal intraperitoneal hemorrhage in an adult. Nippon Jinzo Gakkai Shi. 1994;36:63–8.PubMedGoogle Scholar
  10. 10.
    Aoki N, Soma K, Owada T, Ishii H. Wegener’s granulomatosis complicated by arterial aneurysm. Intern Med. 1995;34:790–3.PubMedCrossRefGoogle Scholar
  11. 11.
    den Bakker MA, Tangkau PL, Steffens TW, Tjiam SL, van der Loo EM. Rupture of a hepatic artery aneurysm caused by Wegener’s granulomatosis. Pathol Res Pract. 1997;193:61–6.Google Scholar
  12. 12.
    Ravanan R, Weale AR, Lear PA, Thornton MJ, Kamel D, Tomson CR. Hemoperitoneum in a patient with ANCA-associated polyangiitis syndrome. Am J Kidney Dis. 2004;44:908–11.PubMedCrossRefGoogle Scholar
  13. 13.
    Kurata A, Nishimura Y, Yamato T, Arimura Y, Yamada A, Sakamoto A, et al. Systemic granulomatous necrotizing vasculitis in a MPO-ANCA-positive patient. Pathol Int. 2004;54:636–40.PubMedCrossRefGoogle Scholar
  14. 14.
    Dutton-Gaddis JG, Oyekan TB, Haraway GD, Crapse FJ. Middle colic artery rupture in a patient with ANCA associated vasculitis: a case report. J Okla State Med Assoc. 2004;97:364–6.PubMedGoogle Scholar
  15. 15.
    Ishizawa T, Yamamoto T, Kawanishi T, Haida K, Sekikawa T. A case of Churg-Strauss syndrome presenting with intra-abdominal bleeding. Nippon Fukubu Kyukyu Igakukai Shi. 2005;25:91–4.Google Scholar
  16. 16.
    Yamazaki M, Takei T, Otsubo S, Iwasa Y, Yabuki Y, Suzuki K, et al. Wegener’s granulomatosis complicated by intestinal ulcer due to cytomegalovirus infection and by thrombotic thrombocytopenic purpura. Intern Med. 2007;46:1435–40.PubMedCrossRefGoogle Scholar
  17. 17.
    Guillevin L, Le Thi Huong D, Godeau P, Jais P, Wechsler B. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol. 1988;27:258–64.PubMedCrossRefGoogle Scholar
  18. 18.
    Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore). 2005;84:115–28.CrossRefGoogle Scholar
  19. 19.
    Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337:1512–23.PubMedCrossRefGoogle Scholar
  20. 20.
    Morgan MD, Harper L, Williams J, Savage C. Anti-neutrophil cytoplasm-associated glomerulonephritis. J Am Soc Nephrol. 2006;17:1224–34.PubMedCrossRefGoogle Scholar
  21. 21.
    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.PubMedCrossRefGoogle Scholar
  22. 22.
    Sakai H, Kurokawa K, Koyama A, Arimura Y, Kida H, Shigematsu H, et al. Guidelines for the management of rapidly progressive glomerulonephritis. Nippon Jinzo Gakkai Shi. 2002;44:55–82.PubMedGoogle Scholar
  23. 23.
    Langford CA. Treatment of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: where do we stand? Arthritis Rheum. 2001;44:508–12.PubMedCrossRefGoogle Scholar
  24. 24.
    ReMine SG, McIlrath DC. Bowel perforation in steroid-treated patients. Ann Surg. 1980;192:581–6.PubMedCrossRefGoogle Scholar
  25. 25.
    Fadul CE, Lemann W, Thaler HT, Posner JB. Perforation of the gastrointestinal tract in patients receiving steroids for neurologic disease. Neurology. 1988;38:348–52.PubMedGoogle Scholar
  26. 26.
    Heimdal K, Hirschberg H, Slettebo H, Watne K, Nome O. High incidence of serious side effects of high-dose dexamethasone treatment in patients with epidural spinal cord compression. J Neurooncol. 1992;12:141–4.PubMedCrossRefGoogle Scholar
  27. 27.
    Goodgame RW. Gastrointestinal cytomegalovirus disease. Ann Intern Med. 1993;119:924–35.PubMedGoogle Scholar
  28. 28.
    Sackier JM, Kelly SB, Clarke D, Rees AJ, Wood CB. Small bowel haemorrhage due to cytomegalovirus vasculitis. Gut. 1991;32:1419–20.PubMedCrossRefGoogle Scholar
  29. 29.
    Nguyen-Ho P, Jewell LD, Thomson AB. Hemorrhagic intestinal Henoch-Schonlein purpura complicated by cytomegalovirus infection. Can J Gastroenterol. 1998;12:71–4.PubMedGoogle Scholar

Copyright information

© Japanese Society of Nephrology 2009

Authors and Affiliations

  • Kensei Yahata
    • 1
  • Chinatsu Okamoto
    • 1
  • Hirotaka Imamaki
    • 1
  • Koichi Seta
    • 1
  • Tsuyoshi Terashima
    • 2
  • Sachiko Minamiguchi
    • 2
  • Akira Sugawara
    • 1
  1. 1.Department of NephrologyNational Hospital Organization Kyoto Medical CenterKyotoJapan
  2. 2.Department of PathologyNational Hospital Organization Kyoto Medical CenterKyotoJapan

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