Oehler E, Valour F, Hachulla E, Ghawche F
Service de médecine interne et polyvalente, centre hospitalier de Polynésie française, Tahiti, 98713 Pirae, Polynésie française.
Service de médecine interne et polyvalente, centre hospitalier de Polynésie française, Tahiti, 98713 Pirae, Polynésie française.
Rev Med Interne. 2014 Dec;35(12):827-30. doi: 10.1016/j.revmed.2013.06.014. Epub 2013 Dec 2.
Adult Still's disease may present with multiple non-specific clinical manifestations leading to diagnostic difficulties. Presentation as initial myopericarditis is rare but must be known.
We report a 49-year-old man who presented with the sudden onset of a high fever, arthralgia and myalgia, skin rash, dyspnea and chest pain related to a myopericarditis. Infectious, neoplastic and autoimmune diagnostic work-up was non-contributive. Adult Still's disease was discussed and confirmed by the decrease in the glycosylated fraction of ferritin. Corticosteroids followed by interleukin-1 receptor antagonist (anakinra) therapy allowed a rapid improvement in clinical and biological manifestations.
Adult Still's disease should be considered in any systemic inflammatory disease without a diagnosis, even with atypical manifestations, especially since it can be life-threatening in the absence of treatment.
成人斯蒂尔病可能表现出多种非特异性临床表现,导致诊断困难。以原发性心肌心包炎形式出现较为罕见,但必须有所了解。
我们报告一名49岁男性,突发高热、关节痛、肌痛、皮疹、呼吸困难以及与心肌心包炎相关的胸痛。感染性、肿瘤性和自身免疫性的诊断检查均无阳性发现。讨论后考虑成人斯蒂尔病,并通过铁蛋白糖基化分数降低得以确诊。给予糖皮质激素治疗,随后使用白细胞介素-1受体拮抗剂(阿那白滞素)治疗,使临床和生物学表现迅速改善。
对于任何未确诊的全身性炎症性疾病,即使表现不典型,也应考虑成人斯蒂尔病,尤其是因为未经治疗可能危及生命。