title: A case of mixed connective tissue disease (MCTD) complicated with MPO-ANCA-related necrotizing glomerulonephritis.
abstract: Renal diseases of mixed connective tissue disease (MCTD) are not unusual. Although most of them are SLE-like renal impairment with immune complex deposits, systemic sclerosis- (SSc) like renal impairments with intimal thickening of interlobular arteries or arterioles are also encountered. Several cases of SSc complicated with MPO-ANCA-related necrotizing glomerulonephritis (nGN) are reported. Here we report a case which developed MPO-ANCA-related nGN 16 years after the diagnosis of MCTD. She exhibited pauci-immune focal nGN and significantly high titer of MPO-ANCA. She was successfully treated with prednisolone and cyclophosphamide. We believe this is the first case in which MPO-ANCA-related nGN was demonstrated in a patient with MCTD.
authors.affiliation: Kidney Center, Toranomon Hospital, Tokyo, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Makita
authors.forename: H
authors.initials: H
authors.lastname: Katori
authors.forename: F
authors.initials: F
authors.lastname: Takemoto
authors.forename: M
authors.initials: M
authors.lastname: Yokota
authors.forename: Y
authors.initials: Y
authors.lastname: Kitamura
authors.forename: Y
authors.initials: Y
authors.lastname: Ubara
authors.forename: S
authors.initials: S
authors.lastname: Hara
authors.forename: A
authors.initials: A
authors.lastname: Yamada
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000276:immunology; D009195:Peroxidase / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase