Vol.23 No.1

Case Report

ANCA-associated vasculitis with dual ANCA positivity in coexistence with mixed connective tissue disease

Authors

Masanori Murakami1 , Kenichi Shimane1 , Hiroshi Takahashi1 , Junji Tomiyama2 , Masakazu Nagashima1

  • Department of Rheumatology, Tokyo Metropolitan Bokutoh Hospital, 4-23-15 Kotobashi, Sumida-Ku, Tokyo, 130-8575, Japan
  • Department of Internal Medicine, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan
Received:

21 December 2011

Accepted:

13 February 2012

Published online:

3 March 2012

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Abstract

We here report a rare case of dual antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a 38-year-old Japanese woman previously diagnosed with mixed connective tissue disease. The patient was found to be positive for myeloperoxidase- and proteinase 3-ANCA, and was diagnosed with AAV following admission to hospital with fervescence, polyarthralgia, purpura, and asymmetric numbness of the extremities. Examination of her genetic background revealed that she carried HLADR9, which confers risk of both diseases in Japanese populations.

Key words

Anti-neutrophil cytoplasmic antibody - HLA-DRB1*09:01 - Microscopic polyangiitis - Mixed connective tissue disease