Introduction
Monoclonal gammopathy of renal significance (MGRS) is a recently described disorder caused by pathologic monoclonal protein (M-protein) leading to renal disease but little hematologic disease burden1. Failure to treat patients with MGRS can lead to poor outcomes due to progression of MGRS to more advanced monoclonal gammopathies, or end stage renal disease2. We report a case of MGRS leading to nephrotic syndrome and immunotactoid glomerulopathy (ITG). We hypothesized that aggressive treatment of the patient’s MGRS through B-cell depletion and proteasome inhibition would improve her glomerulopathy and clinical outcomes. She is currently responding well to treatment with bortezomib, dexamethasone, and rituximab.