Idiopathic multicentric Castleman disease (iMCD) is a systemic lymphoproliferative disease involving multiple organs, including the kidneys. Membranous nephropathy (MN) has been rarely reported as a complication of iMCD. We herein report the case of a 48-year-old man with a 9-year history of iMCD that was complicated by treatment-resistant nephrotic syndrome due to MN. The first renal biopsy performed at the age of 45 years showed diffuse and global MN with a mild glomerular endothelial injury. He was treated with combined therapy of corticosteroids, immunosuppressants, and tocilizumab, an anti-interleukin-6 (IL-6) receptor monoclonal antibody, which was administered every 2-3 weeks. However, nephrotic syndrome persisted, and renal impairment slowly worsened. Serial biopsy performed at 3 years after the first biopsy confirmed advanced lesions of both MN-related and glomerular endothelial injuries. Modification of the therapeutic strategy to weekly administration of tocilizumab gradually led to the remission of proteinuria, allowing the termination of corticosteroids. Thus, the present case suggests a close link between excessive IL-6 actions and the development of glomerular lesions in iMCD. Successful treatment by strict inhibition of IL-6 actions, in this case, may provide a clue for deciding the therapeutic strategy for severe renal complications associated with iMCD.
Keywords: Idiopathic multicentric Castleman disease; Membranous nephropathy; Renal involvement; Thrombotic microangiopathy-like lesion; Tocilizumab.