重症成人斯蒂尔病一例

Severe adult-onset Still’s disease:a case report

成人斯蒂尔病(AOSD)是一种累及多系统、临床表现复杂多样且缺乏特异性诊断指标的临床综合征,极易被误诊或漏诊。其病因和发病机制尚未明确,有较多学者认为与感染、遗传和免疫异常有关,近年来认为AOSD属于“自身炎症反应综合征”。目前尚无统一诊断标准,我国推荐应用较多的是日本Yamaguchi标准。AOSD治疗上以NSAID和(或)糖皮质激素及改善病情抗风湿药为主,生物制剂也有一定效果。该文报道1例重症AOSD患者,以反复发热为首发症状,积极抗感染治疗无效,病情逐渐进展,后出现关节症状、水肿、休克、心力衰竭、急性呼吸窘迫、肺部感染、严重贫血等表现,最后临床确诊,经过糖皮质激素及改善病情抗风湿药、升压、抗心力衰竭、抗感染、连续肾脏替代疗法、呼吸机辅助呼吸及相关对症支持处理后病情好转出院,随访1年余患者健康存活。该例提示,应提高对重症AOSD患者的认识,减少误诊或漏诊,提高诊治致命性AOSD的能力。

Adult-onset Still’s disease(AOSD)is a clinical syndrome involving complex and diverse clinical manifestations of multiple systems and lacks of specific diagnostic indicators,which can be easily misdiagnosed.The etiology and pathogenesis of AOSD are still unclear.Many scholars believe that AOSD is related to infection,heredity and immune abnormality.In recent years,AOSD has been considered as an autoimmune inflammatory response syndrome.Currently,there is no unified diagnostic standard,and the Yamaguchi standard is recommended to be applied in China.Non-steroidal anti-inflammatory drug(NSAID)and/or glucocorticoids and even disease-modifying anti-rheumatic drugs(DMARD)are mainly utilized to treat AOSD.Biological agents can yield certain efficacy.In this article,we reported one case of severe AOSD presenting with recurrent fever as initial symptoms,who was untreated after antibiotic therapy and developed progressive symptoms,such as joint symptoms,edema,shock,heart failure,acute respiratory distress syndrome,pulmonary infection and severe anemia.Eventually,the clinical diagnosis was confirmed.Through corticosteroids and anti-rheumatic medicine/DMARDs,blood pressure treatment,heart failure management,anti-infection,CRRT,assisted ventilation and other supportive treatment,the patient was discharged and remained physically stable and healthy during follow-up for more than a year.This case prompts that we should deepen the understanding of severe AOSD,reduce the risk of misdiagnosis or missing diagnosis,and improve the diagnosis and treatment of this fatal condition.