Passerini P, Ponticelli C
Division of Nephrology and Dialysis, IRCCS, Maggiore Hospital, Milan, Italy.
Curr Opin Nephrol Hypertens. 2001 Mar;10(2):189-93. doi: 10.1097/00041552-200103000-00006.
The prognosis of untreated patients with focal segmental glomerulosclerosis is poor, as the disease progress to end-stage renal disease in approximately 50--70% of nephrotic patients. Although focal segmental glomerulosclerosis was initially considered to be a steroid-resistant disease, several studies have shown a better responsiveness to more prolonged courses of steroids. For patients with steroid-resistant or -dependent focal segmental glomerulosclerosis, cyclosporine A and cytotoxic agents have shown efficacy in clinical trials. Plasmapheresis or LDL-apheresis may represent a rescue treatment in patients who do not respond to other therapies. The role of other agents used in focal segmental glomerulosclerosis, including azathioprine, mycophenolate mofetil, tacrolimus, pefloxacin or vitamin E is still poorly defined.
未经治疗的局灶节段性肾小球硬化患者预后较差，因为在大约50%-70%的肾病患者中，该疾病会进展为终末期肾病。尽管局灶节段性肾小球硬化最初被认为是一种激素抵抗性疾病，但多项研究表明，延长激素疗程会有更好的反应。对于激素抵抗或依赖的局灶节段性肾小球硬化患者，环孢素A和细胞毒性药物在临床试验中已显示出疗效。血浆置换或低密度脂蛋白吸附术可能是对其他治疗无反应患者的一种挽救性治疗方法。用于局灶节段性肾小球硬化的其他药物，包括硫唑嘌呤、霉酚酸酯、他克莫司、培氟沙星或维生素E的作用仍不明确。
