Radiological and clinical specific features of anti-synthetase syndrome : a retrospective analytic study

Q. Maloir, F. Gester, J. Corhay, R. Louis, J. Guiot
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Abstract

Introduction: Anti-synthetase syndrome (ASS) is a rare auto-immune disorder combining autoantibodies and specifics clinical manifestations. One of the particularities of the ASS is the pleiomorphic radiological presentation seen at the initial work-up. Evaluating treatment response can also be challenging and requires a specific clinical, functional, biological and radiological monitoring. Aim: To identify specific radiological and clinical features of ASS. Methods: We retrospectively studied all patients suffering from ASS in CHU of Liege until now. The diagnosis of ASS was made according to ERS’s criteria. We analyzed clinical features, pulmonary function test (PFT), computed tomography (CT), and longitudinal evolution with regard to their treatments. Results: In the whole cohort of 31 patients, we identified 19 anti-JO1, 5 anti-PL12 & 7 anti-PL-7. The sex-ratio is slightly in favor of male. Interestingly PL-12 syndrome was present in younger patients than those with others antibodies (mean age 39,8 vs 53,1 (JO1) & 73,3 (PL7) (p Conclusion: In our single center retrospective study, we found different profile of auto-antibodies according to age and radiological apparence.
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抗合成酶综合征的影像学和临床特征:回顾性分析研究
抗合成酶综合征(Anti-synthetase syndrome, ASS)是一种罕见的自身免疫性疾病,结合了自身抗体和特殊的临床表现。ASS的特点之一是在最初的检查中看到的多形性放射学表现。评估治疗反应也具有挑战性,需要进行具体的临床、功能、生物学和放射学监测。目的:探讨急性胆管炎的放射学及临床特点。方法:回顾性分析列日市所有急性胆管炎患者。根据ERS的诊断标准进行诊断。我们分析了临床特征、肺功能检查(PFT)、计算机断层扫描(CT)和治疗的纵向演变。结果:在整个队列的31例患者中,我们发现了19个抗jo1, 5个抗pl12和7个抗pl -7。性别比例稍微偏向男性。有趣的是,PL-12综合征出现在较年轻的患者中,而非其他抗体患者(平均年龄39,8岁vs 53,1 (JO1)和73,3 (PL7)) (p结论:在我们的单中心回顾性研究中,我们发现根据年龄和放射学表现不同的自身抗体谱。
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