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

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 diagn...

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Bibliographic Details
Published in:Modern rheumatology 2013-01, Vol.23 (1), p.156-161
Main Authors: Murakami, Masanori, Shimane, Kenichi, Takahashi, Hiroshi, Tomiyama, Junji, Nagashima, Masakazu
Format: Article
Language:English
Subjects:
Adult
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - blood
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - complications
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - genetics
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - pathology
Antibodies, Antineutrophil Cytoplasmic - blood
Antibodies, Antineutrophil Cytoplasmic - immunology
Azathioprine - therapeutic use
Cytoplasm
Drug Therapy, Combination
Enzymes
Female
Genetic Predisposition to Disease
Genetics
Glucocorticoids - therapeutic use
HLA-DR Serological Subtypes - genetics
Humans
Immunosuppressive Agents - therapeutic use
Inflammatory diseases
Methylprednisolone - therapeutic use
Mixed Connective Tissue Disease - blood
Mixed Connective Tissue Disease - complications
Mixed Connective Tissue Disease - genetics
Mixed Connective Tissue Disease - pathology
Myeloblastin - blood
Myeloblastin - immunology
Peroxidase - blood
Peroxidase - immunology
Polyclonal antibodies
Prednisolone - therapeutic use
Pulse Therapy, Drug
Risk Factors
Treatment Outcome
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Summary: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 HLA-DR9, which confers risk of both diseases in Japanese populations.
ISSN:1439-7595
1439-7609
DOI:10.3109/s10165-012-0619-8