Department of Neurology, Auckland City Hospital, Auckland, New Zealand
Department of Medicine, Faculty of Medical and Health Sciences, The University of Auckland, Auckland, New Zealand.
Pract Neurol. 2022 Apr;22(2):145-153. doi: 10.1136/practneurol-2021-003031. Epub 2021 Oct 1.
Autoinflammatory syndromes result from a defective innate immune system. They are characterised by unexplained fever and systemic inflammation involving the skin, muscle, joints, serosa and eyes, along with elevated acute phase reactants. Autoinflammatory syndromes are increasingly recognised as a cause of neurological disease with a diverse range of manifestations. Corticosteroids, colchicine and targeted therapies are effective if started early, and hence the importance of recognising these syndromes. Here, we review the neurological features of specific autoinflammatory syndromes and our approach (as adult neurologists) to their diagnosis.
自身炎症性疾病是由先天免疫系统缺陷引起的。其特征为不明原因发热及全身性炎症，可累及皮肤、肌肉、关节、浆膜和眼睛，并伴有急性反应蛋白升高。自身炎症性疾病正日益被认为是导致神经疾病的一个病因，其临床表现多种多样。如果早期开始使用皮质类固醇、秋水仙碱和靶向治疗，这些疾病通常是有效的，因此识别这些综合征非常重要。在此，我们回顾了特定自身炎症性疾病的神经学特征，以及我们（作为成人神经科医生）对其诊断的方法。
