Uzma A Syeda1, Nora G Singer, Marina Magrey. 1. Department of Medicine, Division of Rheumatology, Case Western Reserve University/MetroHealth Medical Center, Cleveland, OH 44109, USA.
Abstract
OBJECTIVES: To report the successful use of rituximab in a patient with anti- glomerular basement membrane (GBM) antibody disease and to review the literature regarding rituximab use in anti-GBM mediated disease. METHODS: We report a case of anti-GBM antibody disease with both anti-GBM antibodies and anti-myeloperoxidase (MPO) specific p-ANCA, who developed thrombotic thrombocytopenic purpura (TTP) on high dose prednisone, plasmapheresis, and cyclophosphamide therapy. The patient was then treated with rituximab. We analyzed the clinical features of five additional patients of anti-GBM disease treated with rituximab identified through a systematic literature review. RESULTS: Our patient was 68-year-old female who presented with acute renal failure. Renal biopsy showed crescentic glomerulonephritis with linear deposits of IgG antibody along the glomerular basement membrane. Treatment was initiated with high dose prednisone, plasmapheresis and oral cyclophosphamide, with subsequent development of leukopenia and TTP and discontinuance of cyclophosphamide. Treatment with rituximab was initiated with clinical improvement of her hematological parameters but not her renal function. Among the five previously reported cases of anti-GBM disease treated with rituximab, three received brief course of IV cyclophosphamide prior to use of rituximab. Except one patient, all recovered renal function and remained dialysis independent. The anti-GBM antibody level remained undetected in all patients. CONCLUSIONS: Combination of prednisone, plasmapheresis, and rituximab can be an effective therapy in patients with an anti-GBM antibody disease complicated with TTP.
OBJECTIVES: To report the successful use of rituximab in a patient with anti- glomerular basement membrane (GBM) antibody disease and to review the literature regarding rituximab use in anti-GBM mediated disease. METHODS: We report a case of anti-GBM antibody disease with both anti-GBM antibodies and anti-myeloperoxidase (MPO) specific p-ANCA, who developed thrombotic thrombocytopenic purpura (TTP) on high dose prednisone, plasmapheresis, and cyclophosphamide therapy. The patient was then treated with rituximab. We analyzed the clinical features of five additional patients of anti-GBM disease treated with rituximab identified through a systematic literature review. RESULTS: Our patient was 68-year-old female who presented with acute renal failure. Renal biopsy showed crescentic glomerulonephritis with linear deposits of IgG antibody along the glomerular basement membrane. Treatment was initiated with high dose prednisone, plasmapheresis and oral cyclophosphamide, with subsequent development of leukopenia and TTP and discontinuance of cyclophosphamide. Treatment with rituximab was initiated with clinical improvement of her hematological parameters but not her renal function. Among the five previously reported cases of anti-GBM disease treated with rituximab, three received brief course of IV cyclophosphamide prior to use of rituximab. Except one patient, all recovered renal function and remained dialysis independent. The anti-GBM antibody level remained undetected in all patients. CONCLUSIONS: Combination of prednisone, plasmapheresis, and rituximab can be an effective therapy in patients with an anti-GBM antibody disease complicated with TTP.