Guerrieri Aldo, Angeletti Giulia, Mazzolini Massimiliano, Bassi Ilaria, Nava Stefano
Department of Specialistic-Diagnostic and Experimental Medicine (DIMES), Respiratory and Critical Care Unit, S.Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
Respir Med Case Rep. 2017 Jul 3;22:91-94. doi: 10.1016/j.rmcr.2017.07.001. eCollection 2017.
Adult onset Still disease (AOSD) is a rare condition characterized by elevated fever along with arthritic symptoms, elevated polymorphonuclear neutrophil count, evanescent rash, and hyperferritinemia. Diagnosis can be made only after have ruled out more frequent conditions, and Yamagouchi or Fautrel criteria should be applied. Parenchimal lung involvement (PLI) is present in less than 5% of AOSD cases and ranges from aspecific reticular interstitial opacities to life threatening conditions, such as acute respiratory distress syndrome (ARDS).
We report the case of a 59 years old man who was referred to our ward because of high fever treated as a pneumonia with antibiotic but not responding to medical treatment, and findings suggestive of interstitial lung disease prevalent in the lower zone on high resolution computed tomography (HRCT). AOSD was diagnosed when the diagnostic Yamaguchi criteria were fulfilled and our suspect was confirmed by the rheumatologist; the patient was then successfully treated with corticosteroids.
PLI in AOSD is very rare but must be considered among differential diagnosis in patients with high fever and aspecific interstial parenchimal lung involvement.
成人斯蒂尔病(AOSD)是一种罕见疾病,其特征为发热伴关节炎症状、多形核中性粒细胞计数升高、一过性皮疹和高铁蛋白血症。只有在排除更常见的疾病后才能做出诊断,应采用山口或福特尔标准。实质性肺部受累(PLI)在不到5%的AOSD病例中出现,范围从非特异性网状间质混浊到危及生命的情况,如急性呼吸窘迫综合征(ARDS)。
我们报告一例59岁男性病例,该患者因高热被当作肺炎用抗生素治疗但无反应而转诊至我院病房,高分辨率计算机断层扫描(HRCT)显示下肺区存在提示间质性肺疾病的表现。当满足山口诊断标准且风湿科医生确认我们的怀疑后,诊断为AOSD;该患者随后接受糖皮质激素治疗成功。
AOSD中的PLI非常罕见,但在高热且有非特异性间质性肺实质受累的患者鉴别诊断中必须予以考虑。