Chintamaneni Sreekanth, Patel Aarat M, Pegram Samuel B, Patel Hirenkumar, Roppelt Heidi
Department of Rheumatology, Stony Brook University Medical Center, Stony Brook, NY, USA.
Ann Indian Acad Neurol. 2010 Jul;13(3):207-10. doi: 10.4103/0972-2327.70874.
Sarcoidosis is a systemic disease characterized by noncaseating granulomas in the involved organs. Neurologic manifestations involving the central and/or peripheral nervous system occur in about 5% of patients. Neurosarcoidosis is often refractory to conventional treatment and therefore more effective treatment options are needed. While the etiology of the disease is still unknown, there is now a better understanding of its pathogenesis on a molecular level. It is clear that tumor necrosis factor-α (TNFα) plays a pivotal role in the development of the granulomas and it is believed to be a key cytokine involved in the pathogenesis of the disease. Taking advantage of this better understanding of disease pathogenesis, anti-TNFα agents are being increasingly used to treat refractory sarcoidosis. We report a patient with refractory neurosarcoidosis who showed dramatic improvement in the clinical and radiological manifestations following treatment with infliximab; he suffered a relapse upon discontinuation of the medication.
结节病是一种全身性疾病,其特征是受累器官出现非干酪样肉芽肿。累及中枢和/或外周神经系统的神经表现见于约5%的患者。神经结节病通常对传统治疗无效,因此需要更有效的治疗选择。虽然该疾病的病因仍不清楚,但目前在分子水平上对其发病机制有了更好的理解。很明显,肿瘤坏死因子-α(TNFα)在肉芽肿的形成中起关键作用,并且被认为是参与该疾病发病机制的关键细胞因子。利用对疾病发病机制的这一更好理解,抗TNFα药物正越来越多地用于治疗难治性结节病。我们报告了一名难治性神经结节病患者,在用英夫利昔单抗治疗后,其临床和影像学表现有显著改善;停药后病情复发。