Q. Maloir, F. Gester, J. Corhay, R. Louis, J. Guiot
{"title":"抗合成酶综合征的影像学和临床特征:回顾性分析研究","authors":"Q. Maloir, F. Gester, J. Corhay, R. Louis, J. Guiot","doi":"10.1183/13993003.congress-2019.pa3673","DOIUrl":null,"url":null,"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.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"28 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Radiological and clinical specific features of anti-synthetase syndrome : a retrospective analytic study\",\"authors\":\"Q. Maloir, F. Gester, J. Corhay, R. Louis, J. Guiot\",\"doi\":\"10.1183/13993003.congress-2019.pa3673\",\"DOIUrl\":null,\"url\":null,\"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.\",\"PeriodicalId\":267660,\"journal\":{\"name\":\"Rare ILD/DPLD\",\"volume\":\"28 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-09-28\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Rare ILD/DPLD\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1183/13993003.congress-2019.pa3673\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rare ILD/DPLD","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1183/13993003.congress-2019.pa3673","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Radiological and clinical specific features of anti-synthetase syndrome : a retrospective analytic study
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.