title: 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 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.
authors.affiliation: Department of Rheumatology, Tokyo Metropolitan Bokutoh Hospital, 4-23-15 Kotobashi, Sumida-Ku, Tokyo, 130-8575, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Murakami
authors.forename: Kenichi
authors.initials: K
authors.lastname: Shimane
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Junji
authors.initials: J
authors.lastname: Tomiyama
authors.forename: Masakazu
authors.initials: M
authors.lastname: Nagashima
mesh_terms: D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000097:blood / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood / Q000276:immunology; D001379:Azathioprine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D020022:Genetic Predisposition to Disease; D005938:Glucocorticoids / Q000627:therapeutic use; D059810:HLA-DR Serological Subtypes / Q000235:genetics; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D008947:Mixed Connective Tissue Disease / Q000097:blood / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D053146:Myeloblastin / Q000097:blood / Q000276:immunology; D009195:Peroxidase / Q000097:blood / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use; D020551:Pulse Therapy, Drug; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D059810:HLA-DR Serological Subtypes; C038988:HLA-DR9 antigen; D007166:Immunosuppressive Agents; D011239:Prednisolone; D009195:Peroxidase; D053146:Myeloblastin; D001379:Azathioprine; D008775:Methylprednisolone