Younger onset myeloperoxidase-specific antineutrophil cytoplasmic antibody- (MPO-ANCA) related glomerulonephritis accompanied with nephrotic syndrome.
It is known that nephrotic syndrome rarely accompanies myeloperoxidase-specific antineutrophil cytoplasmic antibody- (MPO-ANCA) related glomerulonephritis. We present a case of younger onset MPO-ANCA-related glomerulonephritis accompanied with nephrotic syndrome in a female patient. It was diagnosed through the renal biopsy and the detection of a high titer of MPO-ANCA and steroid therapy (intravenous steroid pulse therapy and oral administration), anticoagulant therapy and antiplatelet therapy were initiated. Since her nephrotic syndrome persisted in spite of the decrease of MPO-ANCA, we conducted a second renal biopsy. We found active necrotizing crescentic glomerulonephritis with a small deposition of immunoglobulin and fibrinogen on the glomeruli. To suppress her disease activity, we administered second steroid-pulse therapy and MPO-ANCA titer disappeared. However, as her nephrotic syndrome, which was accompanied by severe hyperlipidemia, persisted, we tried to treat her using low-density lipoprotein (LDL) apheresis. It was effective temporarily, but she finally fell into end-stage renal failure. We discuss here the possibility of double nephropathy by considering her clinical and renal pathologic features.
Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan. kaneko@sch.md.shinshu-u.ac.jp
Y
Y
Kaneko
Y
Y
Kamijo
N
N
Kobayashi
M
M
Higuchi
T
T
Ehara
K
K
Hora
H
H
Shigematsu
K
K
Kiyosawa
D000328:Adult; D017668:Age of Onset; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D005260:Female; D005921:Glomerulonephritis / Q000097:blood* / Q000150:complications*; D006801:Humans; D009404:Nephrotic Syndrome / Q000150:complications*; D009195:Peroxidase / Q000097:blood*
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase