MYELOPEROXIDASE-SPECIFIC ANTINEUTROPHIL 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 o f blood pressure or plasma renin concentration. Renal biopsy revealed necrotizing crescentic glomerulonephritis (pauci-immune type) and the myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibodies (M PO-ANCA) titer was found to be elevated to 669 EU/ml. Methylprednisolo ne (MP) pulse therapy followed by prednisolone (PSL) and mizoribine (M ZR) did not suppress the progression of renal failure. Therefore, we s tarted double-filtration plasmapheresis (DFPP) which effectively remov ed MPO-ANCA and prevented renal failure despite the relatively low dos e of immunosuppressive agents.