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.
Kaneko
Y
Y
Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan. kaneko@sch.md.shinshu-u.ac.jp
Kamijo
Y
Y
Kobayashi
N
N
Higuchi
M
M
Ehara
T
T
Hora
K
K
Shigematsu
H
H
Kiyosawa
K
K
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