Unità Operativa di Reumatologia, Arcispedale S. Maria Nuova, Reggio Emilia, Italy.
Clin Exp Rheumatol. 2012 May-Jun;30(3):424-8. Epub 2012 Jun 26.
We present the case of a 48-year-old male with an acute respiratory distress syndrome which later proved to be an unexpected and initial manifestation of antisynthetase syndrome. Recognising this as a rare combination of an acute respiratory failure and a connective tissue disease in a previously asymptomatic subject is possible only by means of diagnostic exclusion. Based on similar case reports, the only way to reverse the disease and minimise the sequelae is to begin long-term immunosuppressive therapy as soon as possible once the diagnosis has been made. A review of similar cases with antibody anti-Jo-1 is presented with the aim of providing clinicians with useful indications for promptly recognising this poorly-defined and life-threatening emergency.
我们报告了一例 48 岁男性,患有急性呼吸窘迫综合征,后来证明这是抗合成酶综合征的意外和初始表现。只有通过诊断排除法,才能认识到这种急性呼吸衰竭和结缔组织疾病在以前无症状的患者中的罕见组合。基于类似的病例报告,一旦确诊,逆转疾病和最小化后遗症的唯一方法是尽快开始长期免疫抑制治疗。我们回顾了类似的抗 Jo-1 抗体病例,旨在为临床医生提供及时识别这种定义不明确且危及生命的急症的有用指标。