ANCA-Associated Necrotizing Glomerulonephritis Overlapping with Mesangial Proliferative Lupus Nephritis Refractory to Plasmapheresis, Steroid Pulse Therapy, and a Combination of Mycophenolate Mofetil and Rituximab

Necrotizing glomerulonephritis (GN) associated with antineutrophil cytoplasmic antibody (ANCA) has been increasingly recognized in the context of class III or IV lupus nephritis (LN), hereafter designated as ANCA-associated necrotizing LN. While this subset of GN appears to portend an unfavorable renal outcome, it is not clear whether it represents a distinct entity and benefits from a more aggressive therapy. We report a 78-year-old woman who presented with rapidly progressive GN and was found to have a double-stranded DNA (dsDNA) antibody, hypocomplementemia, antiphospholipid antibody, and strongly positive myeloperoxidase antibody. Renal pathology showed necrotizing and crescentic GN on a background of mesangial proliferative GN. Her kidney disease did not improve despite the treatment with plasmapheresis, three doses of methylprednisolone pulse therapy followed by prednisone at 1 mg/kg/day, rituximab, and mycophenolate mofetil. This case not only reinforces the notion that ANCA-associated necrotizing LN is associated with high dsDNA antibody, hypocomplementemia, and worse renal outcome but also adds new insight into the full spectrum of this emerging disease entity by demonstrating that the concurrence of ANCA-associated vasculitis is not specific for class III or IV LN but can also be seen on a background of class II LN.