Encapsulating-peritoneal-sclerosis (EPS) is a rare, but serious and life-threatening complication of peritoneal dialysis (PD). Treatment of EPS consists of discontinuation of PD and maintenance of nutritional status, whereas the role of corticosteroids, tamoxifen and other immunosuppresive agents is not yet fully elucidated. We report the case of a 28-year-old patient, who developed a severe form of calcifying EPS after a 6-year-long therapy with automated PD. The clinical presentation was severe with repeated episodes of total bowel obstruction, weight loss and malnutrition that mandated his prolonged hospitalization. Initial treatment included corticosteroids and tamoxifen (20 mg/day) with a clinically meaningful improvement in gastrointestinal function and nutritional status over the first 6–12 months. Corticosteroids were discontinued at 18 months, but owing to persistence of calcifying lesions and peritoneal thickening in repeated computed-tomography (CT) scans, tamoxifen remained unmodified at a low-dose of 20 mg/day for a 10-year-long period. During follow-up, the patient remained symptoms-free in an excellent clinical condition and the CT findings were unchanged. Long-term administration of tamoxifen was not accompanied by any drug-related adverse effects and potentially exerted a beneficial action on down-regulation of inflammatory and fibrotic processes and improvement of gastrointestinal function, nutritional status and overall health-related quality of life.

中文翻译：

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他莫昔芬治疗严重钙化包膜性腹膜硬化 PD 患者 10 年生存期：病例报告

包裹性腹膜硬化 (EPS) 是腹膜透析 (PD) 的一种罕见但严重且危及生命的并发症。EPS 的治疗包括停止 PD 和维持营养状态，而皮质类固醇、他莫昔芬和其他免疫抑制剂的作用尚未完全阐明。我们报告了一名 28 岁患者的案例，他在接受 6 年的自动化 PD 治疗后出现了严重的钙化性 EPS。临床表现非常严重，反复出现完全肠梗阻、体重减轻和营养不良，因此需要长期住院治疗。初始治疗包括皮质类固醇和他莫昔芬（20 毫克/天），在前 6-12 个月内胃肠功能和营养状况有临床意义的改善。皮质类固醇在 18 个月时停用，但由于重复计算机断层扫描 (CT) 扫描中钙化病变和腹膜增厚的持续存在，他莫昔芬在 20 毫克/天的低剂量下保持 10 年不变。随访期间，患者无症状，临床状况良好，CT表现无变化。他莫昔芬的长期给药不伴随任何药物相关的不良反应，并且可能对炎症和纤维化过程的下调以及胃肠功能、营养状况和整体健康相关生活质量的改善发挥有益作用。他莫昔芬在 20 毫克/天的低剂量下保持 10 年不变。随访期间，患者无症状，临床状况良好，CT表现无变化。他莫昔芬的长期给药不伴随任何药物相关的不良反应，并且可能对炎症和纤维化过程的下调以及胃肠功能、营养状况和整体健康相关生活质量的改善发挥有益作用。他莫昔芬在 20 毫克/天的低剂量下保持 10 年不变。随访期间，患者无症状，临床状况良好，CT表现无变化。他莫昔芬的长期给药不伴随任何药物相关的不良反应，并且可能对炎症和纤维化过程的下调以及胃肠功能、营养状况和整体健康相关生活质量的改善发挥有益作用。