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A case of MPO-ANCA-positive polyarteritis nodosa complicated by exudative otitis media, mononeuritis multiplex, and acute renal failure

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Abstract

In December 2008, a 69-year-old Japanese woman was admitted to the Department of Otorhinolaryngology because of hearing impairment due to bilateral exudative otitis media, and was discharged without complete recovery despite conventional treatment. Two weeks later, she was readmitted for worsened deafness, numbness, gait disturbance, and general fatigue. She was referred to our department for general investigation. On admission, laboratory examination revealed severe inflammatory signs and active nephritic urinary sediments. Cranial computed tomography (CT) revealed progressive exudative otitis media and sinusitis. Initially, Wegener’s granulomatosis was suspected. Nasal cavity biopsy, however, showed no granuloma formation or vasculitis. Serology revealed high titer of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA), suggestive of microscopic polyangitis (MPA). However, contrast CT identified stenosis of a celiac artery, and renal biopsy showed tubulointerstitial changes with minor glomerular abnormalities. Therefore, polyarteritis nodosa (PAN) was suspected and treatment with intravenous methylprednisolone was initiated. However, a lacunar infarct developed followed by cerebral hemorrhage, and the patient died 19 days after readmission. Autopsy revealed fibrinoid necrosis, neutrophilic infiltration, and giant cell reaction in small to medium-sized arteries in multiple organs. These findings led to diagnosis of systemic vasculitis anatomically compatible with PAN. This was a rare case of a patient with MPO-ANCA-positive PAN who may have developed bilateral exudative otitis media and hearing loss as the initial manifestation of PAN.

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References

  1. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337:1512–23.

    Article  CAS  PubMed  Google Scholar 

  2. Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. 2003;349:160–9.

    Article  CAS  PubMed  Google Scholar 

  3. Savage CO, Harper L, Cockwell P, Adu D, Howie A. ABC of arterial and vascular disease: vasculitis. BMJ. 2000;320:1325–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Balow JE. Renal vasculitis. Curr Opin Nephrol Hypertens. 1993;2:231–7.

    Article  CAS  PubMed  Google Scholar 

  5. Gutierrez E. Microscopic polyangiitis. In: Latinis KM, Dao K, Gutierrez E, Shapherd R, Velázquez CR, editors. Rheumatology Subspecialty Consult. Philadelphia: Lippincott Williams & Wilkins; 2004. p. 150–2.

    Google Scholar 

  6. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg Strass syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094–100.

    Article  CAS  PubMed  Google Scholar 

  7. Tanaka M, Matsuo K, Nakamura H, Ishikawa S, Matsuyama K. Two cases of classical polyarteritis nodosa associated with MPO-ANCA. Jpn J Nephrol. 2006;48:371–6.

    Google Scholar 

  8. Sugimoto T, Kanasaki K, Koyama T, Yokomaku Y, Yasuda H, Kashiwagi A, et al. A case of myeloperoxidase-antineutrophil cytoplasmic antibody positive-polyarteritis nodosa complicated by interstitial pneumonia and rapidly progressive renal failure. Clin Rheumatol. 2007;26:429–32.

    Article  PubMed  Google Scholar 

  9. Sugisaki K, Takeda I, Kanno T, Oguchi Y, Kasukawa R. A case of polyarteritis nodosa limited to lower legs with a high titer of MPO-ANCA under precedence of idiopathic pulmonary fibrosis. Fukushima J Med Sci. 2003;49:141–8.

    Article  PubMed  Google Scholar 

  10. Sano K, Sakaguchi N, Ito M, Koyama M, Kobayashi M, Hotchi M. Histological diversity of vasculitic lesions in MPO-ANCA-positive autopsy cases. Pathol Int. 2001;51:460–6.

    Article  CAS  PubMed  Google Scholar 

  11. Rott T, Vizjak A, Koselj M. ANCA-associated vasculitis–an autopsy study. Wien Klin Wochenschr. 2000;112(15–16):671–5.

    CAS  PubMed  Google Scholar 

  12. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008;26((5 Suppl51)):S94–104.

    CAS  PubMed  Google Scholar 

  13. Kirkland GS, Savige J, Wilson D, Heale W, Sinclair RA, Hope RN. Classical polyarteritis nodosa and microscopic polyarteritis with medium vessel involvement—a comparison of the clinical and laboratory features. Clin Nephrol. 1997;47:176–80.

    CAS  PubMed  Google Scholar 

  14. Appel GB, Radhakrishanan J, D’Agati V. Microscopic polyangiitis and polyarteritis nodosa. In: Brenner BM, editor. Brenner and Rector’s The Kidney. 8th ed. Philadelphia: WB Saunders; 2008. p. 1087–91.

    Google Scholar 

  15. Ford RG, Siekert RG. Central nervous system manifestations of periarteritis nodosa. Neurology. 1965;15:114–22.

    Article  CAS  PubMed  Google Scholar 

  16. Hashimoto H. Systemic autoimmune disease: periarteritis nodosa and necrotizing vasculitis. Nihon Rinsyo. 1988;46:152–7.

    Google Scholar 

  17. Malamud N, Foster OB. Periarteritis nodosa; a clinicopathologic report, with special reference to the central nervous system. Arch Neurol Psychiatry. 1941;47:828–38.

    Article  Google Scholar 

  18. Wolf M, Kronenberg J, Engelberg S, Leventon G. Rapidly progressive hearing loss as a symptom of polyarteritis nodosa. Am J Otolaryngol. 1987;8:105–8.

    Article  CAS  PubMed  Google Scholar 

  19. Tsunoda K, Akaogi J, Ohya N, Murofushi T. Sensorineural hearing loss as the initial manifestation of polyarteritis nodosa. J Laryngol Otol. 2001;115:311–2.

    CAS  PubMed  Google Scholar 

  20. Takagi D, Nakamaru Y, Maguchi S, Furuta Y, Fukuda S. Clinical features of bilateral progressive hearing loss associated with myeloperoxidase-antineutrophil cytoplasmic antibody. Ann Otol Rhinol Laryngol. 2004;13:388–93.

    Article  Google Scholar 

  21. Bomholt A, Knudsen JB, Permin H, Tommerup B, Gormsen J. Profound sensorineural hearing loss in polyarteritis nodosa. An atypical case of Cogan’s syndrome. Arch Otorhinolaryngol. 1982;236:53–8.

    Article  CAS  PubMed  Google Scholar 

  22. Gussen R. Polyarteritis nodosa and deafness. A human temporal bone study. Arch Otolaryngol. 1977;217:263–71.

    CAS  Google Scholar 

  23. Jenkins HA, Pollak AM, Fisch U. Polyarteritis nodosa as a cause of sudden deafness. Am J Otol. 1981;2:99–107.

    Article  CAS  Google Scholar 

  24. Adkins WH, Ward PH. Temporal bone showing polyarteritis nodosa, otosclerosis and occult neuroma. Laryngoscope. 1986;96:645–52.

    Article  CAS  PubMed  Google Scholar 

  25. Yoon TH, Paparella MM, Schachern PA. Systemic vasculitis: a temporal bone histopathologic study. Laryngoscope. 1989;99:600–9.

    Article  CAS  PubMed  Google Scholar 

  26. Oomura M, Yamawaki T, Naritomi H, Terai T, Shigeno K. Polyarteritis nodosa in association with subarachnoid hemorrhage. Intern Med. 2006;45:655–8.

    Article  PubMed  Google Scholar 

  27. Provenzale JM, Allen NB. Neuroradiologic findings in polyarteritis nodosa. Am J Neuroradiol. 1996;17:1119–26.

    CAS  PubMed  Google Scholar 

  28. Arimura Y, Nagasawa T. Anti-neutrophil cytoplasmic autoantibody (ANCA) in systemic vasculitic syndrome. Jpn J Clin Pathol. 1993;41:866–75.

    CAS  Google Scholar 

  29. Geffriaud-Ricouard C, Noël LH, Chauveau D, Houhou S, Grunfeld JP, Lesavre P. Clinical spectrum associated with ANCA of defined antigen specificities in 98 selected patients. Clin Nephrol. 1993;39:125–36.

    CAS  PubMed  Google Scholar 

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Correspondence to Atsushi Yamauchi.

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Yamamoto, T., Matsuda, J., Kadoya, H. et al. A case of MPO-ANCA-positive polyarteritis nodosa complicated by exudative otitis media, mononeuritis multiplex, and acute renal failure. Clin Exp Nephrol 15, 754–760 (2011). https://doi.org/10.1007/s10157-011-0457-6

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  • DOI: https://doi.org/10.1007/s10157-011-0457-6

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