ILD Center of Excellence, Department of Pulmonology, St. Antonius Hospital, Nieuwegein.
Department of Pulmonology, Division of Heart & Lungs, University Medical Centre Utrecht, Utrecht.
Curr Opin Neurol. 2019 Jun;32(3):475-483. doi: 10.1097/WCO.0000000000000684.
Sarcoidosis is a complex disease with many faces, and the clinical manifestation and course of neurosarcoidosis are particularly variable. Although neurosarcoidosis occurs in up to 10% of sarcoidosis patients, it can lead to significant morbidity and some mortality.
Three criteria are usually required for a diagnosis of (neuro)sarcoidosis: clinical and radiologic manifestations, noncaseating granulomas, and no evidence of alternative disease. Recent guidelines have helped to clarify criteria for diagnosing neurosarcoidosis. No firm guidelines exist on whether, when, and how treatment should be started. Treatment depends on the presentation and distribution, extensiveness, and severity of neurosarcoidosis. As regards evidence-based treatment, only a few randomized controlled trials have been done. Hence, several aspects of (neuro)sarcoidosis management are not fully addressed by the current literature.
Significant advances have been made in the potential and accuracy of diagnostics for neurosarcoidosis. Treatment should be approached within the context of the patient's anticipated clinical course, avoidance of adverse drug effects, and, if necessary, from the perspective of the comprehensive management of a chronic disease. A multidisciplinary approach to the management of sarcoidosis is strongly recommended.
结节病是一种具有多种表现的复杂疾病，神经结节病的临床表现和病程尤其多变。尽管神经结节病可发生于高达 10%的结节病患者中，但它可导致显著的发病率和一些死亡率。
（神经）结节病的诊断通常需要符合以下三个标准：临床表现和影像学表现、非干酪样肉芽肿以及无其他疾病的证据。最近的指南有助于阐明神经结节病的诊断标准。关于何时以及如何开始治疗尚无明确的准则。治疗取决于神经结节病的表现、分布、广泛程度和严重程度。关于基于证据的治疗，仅进行了少数随机对照试验。因此，目前的文献并没有完全解决（神经）结节病管理的几个方面。
在神经结节病的诊断潜力和准确性方面已经取得了重大进展。治疗应根据患者预期的临床病程、避免药物不良反应以及必要时从慢性病的综合管理角度来进行。强烈建议采用多学科方法来管理结节病。
