Myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibodies -- related scleroderma renal crisis treated with double-filtration plasmapheresis.
A 46-year-old female patient who was diagnosed with systemic sclerosis (SSc) developed rapidly progressive renal failure without elevation of blood pressure or plasma renin concentration. Renal biopsy revealed necrotizing crescentic glomerulonephritis (pauci-immune type) and the myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibodies (MPO-ANCA) titer was found to be elevated to 669 EU/ml. Methylprednisolone (MP) pulse therapy followed by prednisolone (PSL) and mizoribine (MZR) did not suppress the progression of renal failure. Therefore, we started double-filtration plasmapheresis (DFPP) which effectively removed MPO-ANCA and prevented renal failure despite the relatively low dose of immunosuppressive agents.
Omote
A
A
Department of Internal Medicine, Kamo Hospital, Motoshiro-cho, Toyota.
Muramatsu
M
M
Sugimoto
Y
Y
Hosono
S
S
Murakami
R
R
Tanaka
H
H
Watanabe
Y
Y
Sano
H
H
Kato
K
K
D058186:Acute Kidney Injury / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D000925:Anticoagulants / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000378:metabolism / Q000628:therapy; D001707:Biopsy, Needle; D003131:Combined Modality Therapy; D004176:Dipyridamole / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009504:Neutrophils / Q000276:immunology*; D009195:Peroxidase / Q000276:immunology*; D010956:Plasmapheresis / Q000379:methods; D011239:Prednisolone / Q000627:therapeutic use; D012263:Ribonucleosides / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000378:metabolism
D019268:Antibodies, Antineutrophil Cytoplasmic; D000925:Anticoagulants; D007166:Immunosuppressive Agents; D012263:Ribonucleosides; C010052:mizoribine; D004176:Dipyridamole; D011239:Prednisolone; D009195:Peroxidase; D008775:Methylprednisolone