title: ANCA-Associated Vasculitis in a Patient with Systematic Sclerosis and Sjögren's Syndrome: A Case Report.
abstract: A 65-year-old woman with a limited form of systematic sclerosis (SSc) and Sjögren's syndrome (SS) was admitted to our hospital for the evaluation of renal dysfunction. Her serum creatinine was 1.6 mg/dl, proteinuria was 1.6 g/day, and the urine sediment contained 20-29 erythrocytes/high-power field. Myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-SS-A/SS-B antibodies and anti-centromere antibodies were positive. A renal biopsy showed focal necrotizing glomerulonephritis with focal interstitial lymphoplasmacytic infiltration. A diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) was made. A steroid therapy was initiated and AAV subsided. This is a rare case of AAV in a patient with anti-centromere-positive limited SSc and SS.
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Kubota
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Ueno
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Koki
authors.initials: K
authors.lastname: Mise
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Hazue
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Kikuchi
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan ; Okinaka Memorial Institute for Medical Research, Tokyo, Japan.
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
authors.affiliation: Department of Pathology, Toranomon Hospital, Tokyo, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Fujii
authors.affiliation: Department of Pathology, Toranomon Hospital, Tokyo, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
authors.affiliation: Department of Rheumatology, Tokyo Kyosai Hospital, Tokyo, Japan.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Nonomura
authors.affiliation: Nephrology Center, Toranomon Hospital, Tokyo, Japan ; Okinaka Memorial Institute for Medical Research, Tokyo, Japan.
authors.forename: Yosifumi
authors.initials: Y
authors.lastname: Ubara
keywords: ANCA-associated vasculitis; Anti-neutrophil cytoplasmic antibody; Sjögren's syndrome; Systemic sclerosis