迟发性系统性红斑狼疮合并癫痫及弥漫性 肺泡出血的临床分析

Clinical Analysis of Late-Onset Systemic Lupus Erythematosus Complicated by Epilepsy and Diffuse Alveolar Hemorrhage

摘要: 迟发性系统性红斑狼疮(late-onset systemic lupus erythematosus, Lo-SLE)是指在50岁后诊断出系统性红斑狼疮(systemic lupus erythematosus, SLE)的一种罕见的自身免疫性疾病，Lo-SLE起病隐匿、临床表现不典型，易被感染或其他老年常见疾病掩盖。我们回顾性分析了一例我院收治的85岁高龄老人初诊为SLE并发癫痫及弥漫性肺泡出血(diffuse alveolar hemorrhage, DAH)的病例。患者早期以感染性疾病为主要诊断方向，经抗感染治疗效果欠佳后，完善自身免疫学检查，明确诊断为SLE。激素治疗过程中患者出现新发癫痫发作，并迅速进展为难治性癫痫持续状态(refractory status epilepticus, RSE)，随后病情急剧恶化，突发大量咯血及呼吸循环衰竭，临床高度考虑暴发性DAH，最终抢救无效死亡。因此，对老年不明原因多系统受累患者，应及早进行自身免疫性疾病筛查，一旦出现神经精神症状或危及生命的并发症，应在严格评估感染风险的前提下，尽早考虑强化免疫抑制治疗及多学科协作干预，以改善预后。

Abstract: Late-onset systemic lupus erythematosus (Lo-SLE) refers to a rare autoimmune disease diagnosed after age 50. Lo-SLE has an insidious onset with atypical clinical manifestations, often masked by infections or other common geriatric conditions. We retrospectively analyzed a case of an 85-year-old patient admitted to our hospital who developed epilepsy and diffuse alveolar hemorrhage (DAH) following an initial SLE diagnosis. The patient was initially managed for infectious disease, but after inadequate response to antimicrobial therapy, comprehensive immunological testing confirmed SLE. During corticosteroid therapy, the patient developed new-onset seizures that rapidly progressed to refractory status epilepticus (RSE). The condition then deteriorated precipitously, manifesting as massive hemoptysis and respiratory-circulatory failure. Clinically, fulminant DAH was strongly suspected, and the patient ultimately succumbed despite resuscitative efforts. Therefore, for elderly patients with unexplained multisystem involvement, early screening for autoimmune diseases is essential. Upon the emergence of neuropsychiatric symptoms or life-threatening complications, intensive immunosuppressive therapy and multidisciplinary collaborative intervention should be considered promptly, while rigorously assessing infection risks, to improve prognosis.