Judson Marc A, Modi Aakash, Ilyas Furqan, Yucel Recai
Division of Pulmonary and Critical Care Medicine, Albany Medical College, Albany, New York.
Department of Epidemiology and Biostatistics, School of Public Health, State University of New York at Albany, Rensselaer, NY 12144.
Sarcoidosis Vasc Diffuse Lung Dis. 2018;35(3):192-197. doi: 10.36141/svdld.v35i3.6917. Epub 2018 Apr 28.
Vitamin D dysregulation may occur in sarcoidosis patients and result in hypercalciuria, hypercalcemia, nephrolithiasis, and renal impairment. We performed an open label pilot study of highly purified (H.P.) Acthar® Gel (repository corticotropin injection) (RCI) on patients with sarcoidosis-induced vitamin D dysregulation and hypercalciuria. Nine patients with sarcoidosis-induced vitamin D dysregulation and hypercalciuria on stable maintenance anti-sarcoidosis therapy received 80 units of RCI subcutaneously twice weekly for 12 weeks. 24-hour urinary calcium excretion was measured at baseline and at 12 weeks. Other parameters measured over 16 weeks (including 4 weeks post the last RCI dose) included the following serum values: calcium, 25-OH vitamin D, 1,25- diOH vitamin D and serum parathyroid hormone (PTH). In addition, the Sarcoidosis Health Questionnaire (SHQ) and Short Form-36 (SF-36) as well as a urinary symptom score were measured in all subjects. There was no significant change in the 24-hour urinary calcium excretion over 12 weeks of the study. However, there was evidence that RCI improved sarcoidosis-induced vitamin D dysregulation in that the serum 1,25- diOH vitamin D level significantly declined over 12 weeks. There was also improvement in most of the domains of the quality of life measures, although only a few of them reached statistical significance. There was also a trend toward improvement in urinary symptoms over the study period. The was evidence of the development corticosteroid side effects in the cohort, in that weight significantly increased over the study period. In this small pilot open label trial, 12 weeks of RCI did not significantly improve sarcoidosis-induced hypercalciuria. However, some statistically significant changes in serum vitamin D and PTH levels were demonstrated that were consistent with some amelioration of sarcoidosis-induced vitamin D dysregulation. Several corticosteroid-related side effects were demonstrated in this cohort. .
结节病患者可能会出现维生素D调节异常，进而导致高钙尿症、高钙血症、肾结石和肾功能损害。我们对患有结节病引起的维生素D调节异常和高钙尿症的患者进行了一项关于高纯度（H.P.）Acthar® Gel（促肾上腺皮质激素注射液）（RCI）的开放标签试点研究。9名在稳定维持性抗结节病治疗下患有结节病引起的维生素D调节异常和高钙尿症的患者，每周皮下注射80单位RCI，共12周，每周两次。在基线和第12周时测量24小时尿钙排泄量。在16周内（包括最后一剂RCI后4周）测量的其他参数包括以下血清值：钙、25-羟基维生素D、1,25-二羟基维生素D和血清甲状旁腺激素（PTH）。此外，在所有受试者中测量了结节病健康问卷（SHQ）和简明健康状况调查量表（SF-36）以及尿路症状评分。在研究的12周内，24小时尿钙排泄量没有显著变化。然而，有证据表明RCI改善了结节病引起的维生素D调节异常，因为血清1,25-二羟基维生素D水平在12周内显著下降。生活质量测量的大多数领域也有改善，尽管只有少数达到统计学意义。在研究期间，尿路症状也有改善的趋势。在该队列中有证据表明出现了皮质类固醇副作用，因为在研究期间体重显著增加。在这项小型开放标签试点试验中，12周的RCI并没有显著改善结节病引起的高钙尿症。然而，血清维生素D和PTH水平出现了一些具有统计学意义的变化，这与结节病引起的维生素D调节异常的一些改善是一致的。在该队列中还出现了几种与皮质类固醇相关的副作用。
