A case of fulminant ANCA-negative Wegener’s granulomatosis with primary ENT involvement


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The paper describes a rare case of ANCA-negative Wegener's granulomatosis with a severe, fulminant course, which is manifested by primary ENT involvement.

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作者简介

D. Fernando

Aleksandro-Mariinsk Regional Clinical Hospital; Astrakhan State Medical University; Astrakhan Branch, Otorhinolaryngology Research and Clinical Center, Federal Biomedical Agency of Russia

Email: dinafernando.lor@gmail.com
Candidate of Medical Sciences

V. Shpotin

Aleksandro-Mariinsk Regional Clinical Hospital; Astrakhan State Medical University; Astrakhan Branch, Otorhinolaryngology Research and Clinical Center, Federal Biomedical Agency of Russia

A. Gostyunin

Aleksandro-Mariinsk Regional Clinical Hospital

S. Kudaev

Postmortem Examination Bureau

参考

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  2. Mohammad A., Jacobsson L., Westman K. et al. Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritisnodosa // Rheumatology (Oxford). - 2009; 12: 1560-5.
  3. Leavitt R., Fauci A., Bloch D. et. al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis // Arthritis Rheum. - 1990; 33: 1101-7.
  4. Зубкова Л.Л., Балданова И.Р., Борхонова И.В. и др. Гранулематоз Вегенера (случай из практики) // Вестн. Бурятского гос. университета. - 2009; 12: 99-101.
  5. Kashiwagi T., Hayama N., Fujita E. et al. A case of (double) ANCA-negative granulomatosis with polyangiitis (Wegener’s) // CEN Case Reports - 2012; 1: 104-11.
  6. Savige J., Pollock W., Trevisin M. What do antineutrophil cytoplasmic antibodies (ANCA) tell us? // Best Pract. Res. Clin. Rheumatol. - 2005; 19 (2): 263-76.
  7. Hagen E., Daha M., Hermans J. et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization // Kidney Int. - 1998; 53 (3): 743-53.
  8. Hoffman G., Specks U. Antineutrophil cytoplasmic antibodies // Arthritis Rheum. - 1998; 41 (9): 1521-37.
  9. Pagnoux C., Wolter N. Vasculitis of the upper airways // Swiss. Med. Wkly. -2012; 142: 13541.

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