Pulmonary diseases emerge as leading causes of mortality in patients with anti-synthetase syndrome
Due to the heterogeneity of clinical presentations in anti-synthetase syndrome (ASyS), maintaining a high index of suspicion and early screening for specific autoantibodies could significantly contribute to the prompt diagnosis of ASyS and the timely detection of related complications, according to a study.
The study, conducted retrospectively, analyzed the clinical characteristics and complications in 205 patients with ASyS, seeking to identify factors influencing their survival.
Patients fulfilling either the Connor’s criteria or Solomon’s criteria for ASyS were recruited, and electronic health records were reviewed. The median follow-up time for the cohort was four years.
The results revealed a diverse array of autoantibodies among the participants, with anti-Jo-1 being the most prevalent at 49.3%, followed by anti-PL-7 (19.0%), anti-EJ (11.2%), anti-PL-12 (10.2%), and anti-OJ (3.4%). The study observed significant variations in the time from symptom onset to diagnosis, with non-anti-Jo1 patients experiencing a longer median duration (5 months compared to 3 months).
Common initial presentations included myositis (56.1%), arthritis (54.6%), and interstitial lung disease (ILD) (54.1%). Patients with anti-Jo-1 exhibited higher muscle enzyme levels and a higher incidence of arthritis. Patients with anti-EJ eventually developed ILD, while malignancy was noted in 28.6% of anti-OJ positive patients.
Overall, 15.6% of the patients included in the study died, with pulmonary diseases, such as ILD or pneumonia, identified as the primary causes of mortality. The study identified several factors independently associated with mortality, including age at diagnosis, malignancy, and rapidly progressive ILD, while joint manifestation emerged as a protective factor.
Reference
Tang HS, Tang IYK, Ho RTC, et al. Clinical heterogeneity and prognostic factors of anti-synthetase syndrome: a multi-centered retrospective cohort study. Rheumatology (Oxford). 2023;kead671. doi: 10.1093/rheumatology/kead671. Epub ahead of print. PMID: 38096583.
