Successful treatment of rapidly progressive lupus nephritis associated with anti-MPO antibodies by intravenous immunoglobulins

We report a case of systemic lupus erythematosus (SLE) associated with cres centic glomerulonephritis and myeloperoxidase-specific anti-neutrophil cyto plasmic antibodies (MPO-ANCA), A 34-year-old Japanese female patient diagno sed with SLE developed rapidly progressive renal failure and nephrotic synd rome, Haemodialysis was required to restore renal function. Methylprednisol one pulse therapy followed by plasmapheresis did not suppress the progressi on of renal failure, so she was treated with high-dose intravenous immunogl obulin (IV-IG) therapy, which was well tolerated and effectively prevented renal failure. A renal biopsy showed diffuse proliferative lupus nephritis (WHO classification IVc) with predominant crescent formation and scant sube ndothelial immune deposits. These findings indicate that, in addition to lu pus nephritis, which usually results from the deposition of circulating or locally formed immune complexes, MPO-ANCA may be involved in the pathogenes is of crescentic glomerulonephritis, Furthermore, we propose that IV-IG is an effective therapy for MPO-ANCA-related renal crisis in lupus nephritis.