AB0656 ANTISYNTHETASE SYNDROME: CLINICAL VALUE OF SOLOMON’S AND CONNORS’ DIAGNOSIS CRITERIA

Background: Two antisynthetase syndrome (ASSD) diagnosis criteria sets have been proposed; both consider mandatory the presence of anti-aminoacyl transfer RNA synthetase (ARS) autoantibodies. Solomon’s criteria consider major clinical criterions (interstitial lung disease (ILD) and fulfillment of Bohan and Peter criteria for DM/PM) and minor criterions (arthritis, Raynaud’s phenomenon (RP) and mechanic’s hands (MH)) (1). In contrast, Connors criteria evaluate the presence of at least one of the previously mentioned clinical features except myositis, and includes the presence of fever without other cause (2). Objectives: 1) to evaluate the performance of Solomon’s and Connors’ criteria in patients with clinical suspicion of aSSD or myositis and positive aRS. 2) to describe their clinical characteristics. Methods: We performed an observational retrospective study in two centers. All patients with clinical suspicion of aSSD or myositis, and positive aRS in the myositis immunoblot (Euroimmun assay) were included. Results: We analyzed 37 patients; 70.3% woman, with a mean age at the moment of the aRS detection of 51.4 (SD±14.0) years, median time from the first symptom to the aRS detection of 4.0 (SD±5.8) years, and time of evolution of 7.69 (SD±6.51) years. The frequency of aRS was: anti-Jo1 (n=17), anti-PL-12 (n=8), anti-PL-7 (n=4), anti-EJ (n=4), and anti-OJ (n=4). Diagnosis criteria fulfillment and clinical manifestations: 1) Patients that met Solomon and Connors’ criteria (n=17, 45.9%): - at disease onset: ILD (n=6, 35.9%), muscle weakness (MW) (n=5, 29.4%), and arthritis (n=4, 23.5%). - During disease development: ILD (n=14, 82.3%); arthritis (n=13, 76.5%); MW (n=10, 58.8%); mechanic hands (n=10, 58.8%); Raynaud phenomenon (n=8, 47.0%); and fever (n=3, 17.5%). 2) Patients that only met Connors’ criteria (n=17, 45.9%): - at disease onset: ILD (n=5, 29.4%), MW (n=3, 17.6%), or arthritis (n=5, 29.4%). - During disease development: arthritis (n=8, 47.0%); ILD (n=6, 35.3%); MW (n=6, 35.3%); Raynaud phenomenon (n=6, 35.3%); fever (n=5, 29.4%); and mechanic hands (n=1, 5.9%). Relative risk (RR) of the different clinical manifestation for Solomon’s criteria fulfillment: - MH RR=2.98 (95%CI 1.5-5.6; P=0.002), ILD RR=3.2 (95%CI 1.1-9.2; P=0.013); other manifestations does not presented significant RR. Conclusion: More than three quarter of all patients presented as first clinical manifestation one of those included in the aSSD classic triad (ILD, MW and arthritis). These manifestations showed increasing rates during the disease development, being more frequent in patients that met Solomon’s criteria than in those who only met Connor’s criteria; more than twice as high in for ILD, and almost twice for MW and arthritis. This suggests that patients who met Solomon’s criteria, at disease onset presented incomplete clinical forms, and their clinical progression favored the criteria fulfillment. On the other hand, we cannot predict if the patients that only met Connors’ criteria are going to fulfill Solomon’s criteria; nevertheless, our results suggests it. To conclude, our results suggest that: 1) Connors criteria might be considered for screening of aSSD; 2) Solomon’s criteria can be considered at the moment the gold standard for the aSSD diagnosis; 3) the presence of mechanic hands or ILD indicates a high probability of Solomon’s criteria fulfillment in patients with positive aRS. References [1] Solomon J, et al. Jornal brasileiro de pneumologia. 2011;37(1):100-9. [2] Connors GR, et al. Chest. 2010;138(6):1464-74. Disclosure of interests: Martin Greco: None declared, Maria Jesus Garcia de Yebenes: None declared, inmaculada alarcon: None declared, anahy Brandy-Garcia: None declared, Inigo Rua-Figueroa: None declared, Estibaliz Loza Grant/research support from: Roche, MSD, Pfizer, abbvie, BMS, UCB, actelion, Celgene, Grunenthal and Sanofi, Teresa Oton: None declared, Juan Carlos Quevedo-Abeledo: None declared, Carlos Rodriguez-Lozano: None declared, Loreto Carmona Grant/research support from: abbvie, actelion, astellas, BMS, Eisay, Gebro Pharma, Grunenthal, Leo Pharma, Lilly, MSD, Novartis, Pfizer, Roche, Sanofi-Aventis and UCB Pharma, Paid instructor for: Novartis