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.
MeSH terms
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Adult
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Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / blood
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Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / complications*
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Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / genetics
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Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / pathology*
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Antibodies, Antineutrophil Cytoplasmic / blood
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Antibodies, Antineutrophil Cytoplasmic / immunology
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Azathioprine / therapeutic use
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Drug Therapy, Combination
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Female
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Genetic Predisposition to Disease
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Glucocorticoids / therapeutic use
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HLA-DR Serological Subtypes / genetics
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Humans
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Immunosuppressive Agents / therapeutic use
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Methylprednisolone / therapeutic use
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Mixed Connective Tissue Disease / blood
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Mixed Connective Tissue Disease / complications*
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Mixed Connective Tissue Disease / genetics
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Mixed Connective Tissue Disease / pathology*
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Myeloblastin / blood
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Myeloblastin / immunology
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Peroxidase / blood
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Peroxidase / immunology
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Prednisolone / therapeutic use
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Pulse Therapy, Drug
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Risk Factors
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Treatment Outcome
Substances
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Antibodies, Antineutrophil Cytoplasmic
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Glucocorticoids
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HLA-DR Serological Subtypes
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HLA-DR9 antigen
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Immunosuppressive Agents
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Prednisolone
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Peroxidase
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Myeloblastin
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Azathioprine
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Methylprednisolone