| 초록 |
Outcomes of EPS: What Can Be Done? Encapsulating peritoneal sclerosis (EPS) is a rare but potentially life-threatening complication of peritoneal dialysis (PD) that is characterized by intraperitoneal inflammation and fibrosis, and the development of a fibrocollagenous membrane that encases bowel loops, leading to ultrafiltration failure, bowel obstruction and increased mortality risk. EPS may manifest even after patients have switched to haemodialysis or undergone kidney transplantation. There are several risk factors for developing EPS but the predominant ones are long term PD duration with chronic exposure to bioincompatible PD fluids, and peritonitis episodes. Additional risk factors include exposure to certain disinfectants or medications, and polymorphisms in genes involved in inflammation, angiogenesis and fibrosis. Diagnosis can be challenging, as early symptoms are non-specific and can mimic other conditions. However, early diagnosis is crucial for successful treatment outcomes. Current therapies include drugs (tamoxifen and glucocorticoids), nutritional support and surgery for advanced cases with severe bowel obstruction. Tamoxifen has shown promise in reducing mortality rates though there have been varied outcomes, likely related to differences in disease severity. Immunosuppressants such as azathioprine, mycophenolate mofetil or mTOR inhibitors may help mitigate inflammation, fibrin deposition, collagen synthesis and maturation. Emerging treatments, such as N-acetylcysteine, colchicine, rosiglitazone, thalidomide and renin-angiotensin system (RAS) inhibitors have shown potential in animal studies but require further investigation for clinical efficacy. Despite advances in PD care, mortality rates associated with EPS remain significant. Therefore it is essential to reduce the incidence of EPS and to improve patient outcomes by vigilance and having a low threshold for investigation - this will aid early diagnosis, appropriate PD discontinuation and prompt initiation of therapies for EPS, albeit empirical at present. In addition, ongoing research is needed to better understand the pathophysiology of EPS and to develop more effective treatment strategies. |