Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), AP-HP, Hôpital Saint-Antoine, Sorbonne Universités, UPMC University Paris 06, F-75012, Paris, France.
Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), AP-HP, Hôpital Saint-Antoine, Sorbonne Universités, UPMC University Paris 06, F-75012, Paris, France.
Semin Arthritis Rheum. 2018 Feb;47(4):575-577. doi: 10.1016/j.semarthrit.2017.07.003. Epub 2017 Jul 10.
Adult onset Still's disease is a rare affection classified among non-hereditary autoinflammatory diseases. We here report a case of AOSD revealed during pregnancy with a life-threatening presentation along with a review of 19 cases from literature.
A 38-years old woman was treated in our department for diffuse systemic sclerosis and associated Sjögren syndrome. She was pregnant and presented with acute fever and arthralgias. Laboratory data revealed mild liver cytolysis but a large screening for infectious and auto-immune diseases was negative and hepato-biliar imaging was normal. Ferritin levels were at 41 000 ng/mL with glycosylated ferritin less than 5%. The diagnosis of AOSD was stated and because of persistent fever and polyarthralgias, after exclusion of active infection, steroids were started (prednisone 1 mg/kg) associated with colchicine, which allowed clinical remission and C-reactive protein significant decrease.
Pregnancy-revealed AOSD appears to be a specifical subset of the disease with a systemic course, flares on first and second trimester, obstetrical complications such as prematurity and IUGR sometimes leading to life-threatening situations requiring parenteral corticotherapy and intravenous immunoglobulins.
成人Still 病是一种罕见的疾病，归类于非遗传性自身炎症性疾病。我们在此报告一例妊娠期间出现的 AOSD 病例，其表现为危及生命，并对文献中的 19 例病例进行了回顾。
一名 38 岁女性因弥漫性系统性硬化症和相关干燥综合征在我科治疗。她怀孕了，出现急性发热和关节痛。实验室数据显示轻度肝细胞溶解，但广泛的感染和自身免疫性疾病筛查均为阴性，肝胆成像正常。铁蛋白水平为 41000ng/mL，糖化铁蛋白<5%。诊断为 AOSD，由于持续发热和多发性关节炎，在排除活动性感染后，开始使用类固醇（泼尼松 1mg/kg）联合秋水仙碱治疗，这使临床缓解和 C 反应蛋白显著降低。
妊娠诱发的 AOSD 似乎是该病的一个特定亚型，具有全身性病程，在第一和第二孕期出现发作，产科并发症如早产和 IUGR 有时会导致危及生命的情况，需要静脉内皮质激素治疗和静脉注射免疫球蛋白。
