Case reports and clinical analysis of 8 patients with primary Sj?gren's syndrome diagnosed as anti-synthase syndrome / 中华风湿病学杂志

ABSTRACT

Objective:Anti-synthase syndrome (ASS) is a rare autoimmune disease. To increase the understanding of the disease and reduce the rate of miss diagnosis.Methods:The clinical data of 8 patients with positive anti-synthase antibody afterprimary Sj?gren's syndrome (pSS) were retrospectively analyzed and descriptive statistical analysis was carried out.Results:The diagnosis of Sjogren's syndrome (SS) was in accordance with the revised European criteriaof SS issued by the US-Europe consensus Group in 2002 or the classification criteria of American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) SS in 2016, and the diagnostic ASS was in accordance with the diagnostic criteria of Conners in 2010 or Solomon in 2011. Eight(100%) patients had a history of interstitial lung disease, and 7 (88%) patients had fever (oral temperature >38.5 ℃). All patients were positive for anti-Ro-52 antibody, 4 patients were positive for anti-PL-7 antibody, 2 patients were positive for anti-EJ antibody, 1 patient was positive for both anti-PL-7 antibody and anti-EJ antibody, and 1 patient was positive for anti-PL-12.Conclusion:pSS patients with severe interstitial lung disease or high fever of unknown causes should be screened for anti-synthase antibodies and the possibility of ASS.