A case of mixed connective tissue disease (MCTD) complicated with MPO-ANCA-related necrotizing glomerulonephritis.
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.
Makita
N
N
Kidney Center, Toranomon Hospital, Tokyo, Japan.
Katori
H
H
Takemoto
F
F
Yokota
M
M
Kitamura
Y
Y
Ubara
Y
Y
Hara
S
S
Yamada
A
A
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*
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase