1 Division of Cardiology, Stellenbosch University & Tygerberg Academic Hospital, Cape Town, South Africa.
2 Division of General Internal Medicine, Department of Medicine, Stellenbosch University & Tygerberg Academic Hospital, Cape Town, South Africa.
Lupus. 2019 Aug;28(9):1154-1157. doi: 10.1177/0961203319862867. Epub 2019 Jul 23.
Hypertrophic pachymeningitis (HP) is a relatively uncommon disease associated with focal or diffuse thickening of the dura mater secondary to underlying chronic inflammation. The link between systemic lupus erythematosus (SLE) and hypertrophic pachymeningitis (HP) is extremely rare, with only six other cases reported in the literature. We, however, report the first case of SLE pachymeningitis presenting with multiple cranial nerve palsies. The patient showed good response to steroids and cyclophosphamide therapy. One should maintain a high index of suspicion to make the diagnosis in patients with SLE presenting with neurological dysfunction. Prompt therapy prevents long-term neurological sequelae.
肥厚性硬脑膜炎(HP)是一种相对罕见的疾病,与潜在的慢性炎症引起的硬脑膜局灶性或弥漫性增厚有关。系统性红斑狼疮(SLE)与肥厚性硬脑膜炎(HP)之间的联系极为罕见,文献中仅报道了其他六例。然而,我们报告了首例 SLE 硬脑膜炎伴多发性颅神经麻痹的病例。患者对类固醇和环磷酰胺治疗反应良好。对于出现神经功能障碍的 SLE 患者,应保持高度怀疑以做出诊断。及时治疗可预防长期的神经后遗症。