Effective improvement of minimal change nephrotic syndrome with uncontrollable high low-density lipoprotein cholesterol
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CASE REPORT
Effective improvement of minimal change nephrotic syndrome with uncontrollable high low‑density lipoprotein cholesterol level using evolocumab accompanied by the development of acute pancreatitis Risa Asai1 · Tomohiro Kaneko1 · Miyako Seki1 · Shuichi Tsuruoka2 Received: 11 December 2019 / Accepted: 17 April 2020 © Japanese Society of Nephrology 2020
Abstract Nephrotic syndrome is sometimes refractory; however, it is rarely accompanied by acute pancreatitis. A 47-year-old Japanese woman complaining of limb edema was diagnosed with nephrotic syndrome. Blood and urine examinations suggested minimal change nephrotic syndrome (MCNS), and pulse intravenous methylprednisolone was administered, followed by oral prednisolone. Although proteinuria improved, the patient’s condition remained unchanged, and diuresis was insufficient. As in patients with other nephrotic syndromes, this patient showed significant dyslipidemia. Atorvastatin was started for remarkable dyslipidemia since her admission, but her low-density lipoprotein cholesterol (LDL-C) level did not improve significantly. During the clinical course, she developed acute pancreatitis, and large-volume fluid replacement was performed. Although diuretic levels were increased in response to the increased fluid volume, diuresis was not enough, and lung edema developed. Extracorporeal ultrafiltration was started to ameliorate the lung edema. With the onset of pancreatitis, oral intake, including atorvastatin, was discontinued, and prednisolone was administered intravenously. To treat the high-LDL cholesterolemia, 140 mg of evolocumab was injected subcutaneously. Nausea slightly decreased on the following day, and the administration of 150 mg cyclosporine was initiated. LDL-C levels, proteinuria, and renal function promptly ameliorated. The results of a renal biopsy suggested MCNS. On the 44th day of hospitalization, she had complete remission. Evolocumab is potentially effective for severe nephrotic syndrome with uncontrollable dyslipidemia. Keywords Minimal change nephrotic syndrome · Acute pancreatitis · Dyslipidemia · Evolocumab · PCSK9 inhibitor
Introduction Minimal change nephrotic syndrome (MCNS) is usually sensitive to steroids, but it is sometimes resistant to steroids and can be refractory. Refractory nephrotic syndrome often presents with extremely high levels of low-density lipoprotein cholesterol (LDL-C) resistant to β-hydroxy β-methylglutaryl-CoA (HMG-CoA) reductase inhibitors. Early remission can be expected if LDL apheresis (LDLA) is performed in patients with steroid-resistant primary * Risa Asai [email protected] 1
Department of Nephrology, Nippon Medical School TamaNagayama Hospital, Tama, Tokyo, Japan
Department of Nephrology, Nippon Medical School, Bunkyo‑ku, Tokyo, Japan
2
nephrotic syndrome with uncontrollable high-LDL cholesterolemia [1]; however, in Japan, LDL-A is only covered by insurance if the cause is focal glomerulosclerosis; hence, MCNS is not covered by insurance. We experienced a case of MCNS that did not remit easi
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