Abstract
ABSTRACT OBJECTIVES: Anti-synthetase syndrome (ASS) is a rare autoimmune disorder combining autoantibodies and specific clinical manifestations. One of the particularities of ASS is the pleiomorphic radiological presentation seen at the initial work-up. Evaluating treatment response can also be challenging and requires specific clinical, functional, biological and radiological monitoring. For these reasons, it is fundamental to identify specific radiological and clinical features of ASS for improved diagnosis and therapeutic approaches. METHODS: We retrospectively studied all patients suffering from ASS in the CHU of Liège from 2008 to 2019. We analysed the clinical features, pulmonary function tests (PFTs), computed tomography (CT), and longitudinal evolution with regard to patient treatment. RESULTS: In the whole cohort of 30 patients, we identified 19 with anti-JO1 antibodies, 5 with anti-PL12 antibodies and 6 with anti-PL7 antibodies. The sex ratio was slightly in favour of males. Interestingly, PL-12 syndrome was more likely to be present in younger patients than those associated with other antibodies. Overall, 77% of the overall cohort exhibited specific pulmonary involvement without any significant difference with regard to the severity assessed by PFT at diagnosis. In contrast, the radiological presentation was pleomorphic for anti-JO1 syndrome, and anti-PL12 syndrome exhibited mainly ground-glass opacities (GGOs) and reticular abnormalities, while those with anti-PL7 antibodies showed reticulations and bronchiectasis. Longitudinal CT analysis mainly showed a reduction in consolidations and GGOs with specific therapies. CONCLUSION: In our single-centre retrospective study, we found different profiles for different autoantibodies according to age and radiological appearance.
Highlights
Anti-synthetase syndrome (ASS) is a rare auto-immune disorder described for the first time in 1990 [1]
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 < 0.01)). 77% of the overall cohort exhibited specific pulmonary involvement without any significant difference with regards to the severity assessed by pulmonary function test (PFT) at diagnosis
In our single center retrospective study, we found different profile of autoantibodies according to age and radiological appearance
Summary
Anti-synthetase syndrome (ASS) is a rare auto-immune disorder described for the first time in 1990 [1]. A systematic blood analysis focusing on the specific antibodies assessment must be considered in the presence of any ILD. Diagnosis of ASS associates a positive serologic testing for an anti-aminoacyl-transfer-RNA synthetase (anti-ARS) autoantibody and at least one pathognomonic clinical manifestation (Table 1) [6]. Anti-JO1 (anti-histidyl), anti-PL7 (anti-threonyl), anti-PL12 (anti-alanyl), anti-OJ (antiisoleucyl) and anti-EJ (anti-glycyl) represent more than 90 percent of the detected ones [7]. They are routinely tested in practice but clinician must consider that specific analysis in case of positive anticytoplasmic nuclear antibodies in patient suffering from ILD [8]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
