Abstract
Takayasu arteritis (TAK) is a systemic inflammatory condition characterized by vasculitis in mainly the aorta and their branches; however, few reports have demonstrated glomerulonephritis and subsequent nephrotic syndrome in patients with TAK. We encountered a 69-year-old woman with TAK who developed nephrotic syndrome owing to uncontrolled hypertension. Kidney biopsy demonstrated endotheliosis, aberrant proliferation of vascular smooth muscle cells, and concentric intimal hyperplasia without any clues of vasculitis. Treatment with sacubitril/valsartan reduced proteinuria and increased serum albumin without affecting renal function, which continued to suppress blood pressure and prevent recurrence of nephrotic syndrome over 2 years.