SAT0348 CLINICAL SPECTRUM TIME COURSE OF ANTISYNTHETASE SYNDROME PATIENTS POSITIVE FOR ANTICENTROMERE ANTIBODIES
Authors: G. Zanframundo, G. Sambataro, V. Codullo, A. Biglia, E. Bozzalla Cassione, E. Bravi, F. Iannone, M. Fornaro, K. Triantafyllias, A. Pesci, P. Tomietto, Ø. Molberg, S. Scarpato, R. Voll, M. Matucci-Cerinic, M. A. González-Gay, C. Montecucco, L. Cavagna
Journal: Annals of the Rheumatic Diseases (2020)

Background: ASSD is characterized by antisynthetase antibodies (ARS) and the triad arthritis/myositis/Interstitial Lung Disease (ILD). ASSD and systemic sclerosis (SSc) may share features, like Raynaud’s phenomenon (RP), capillaroscopic alterations, and also some SSc specific autoantibodies. Objectives: To evaluate the characteristics of ASSD + for anticentromere antibodies (ACA). Methods: Retrospective analysis of clinical and laboratory characteristics of ACA + ASSD. Patients were identified in an established international cohort, randomly matched 1:1 for sex, age, disease duration and ARS positivity with a group of ACA - ASSD. Results: 18 ACA + ASSD (15 females, 83%, 15 anti-Jo1, 2 anti-PL7, 1 anti-PL12 ARS) patients were identified. In comparison to ACA - group, no differences were observed in disease clinical presentation and evolution. Though, 9 ACA + patients (50%) satisfied the ACR/EULAR 2013 classification criteria for SSc and only 1 in ACA - group (p=0.007) (Table 1). An incomplete ASSD (lack of at least one triad finding) was observed in 15 patients in both ACA + and – group (p=1). Among these patients, 13 ACA + and 11 ACA – developed de-novo triad finding during disease course (p=0.651). In ACA + group, a de-novo arthritis was observed in 4 patients (vs 1, p=0.565), a de-novo myositis in 8 (vs 5, p=1), and a de-novo ILD in 7 (vs 10, p=1). The prevalence of complete forms was similar between ACA + and – group at both disease onset (3 vs 3, 17%, p=1) and last follow-up, (10 vs 11, 56% vs 61%, p=1). Of note, only 1 patient (6%) for each group died (p=1). Conclusion: The clinical spectrum time course of ACA+ and - ASSD is similar, even when ACA + patients could be classified as SSc. By considering the high prevalence of arthritis and myositis we observed, we suggest that ACA+ patients with arthritis and myositis, should be tested for ARS antibodies even when an ASSD is not clearly suspected. References: [1]Mirrakhimov AE. Curr Med Chem 2015;22:1963–75 [2]Cavagna L. J Clin Med 2019;8:E2013 [3]Sebastiani M. J Rheum 2019:46:279-84 [4]van den Hoogen F. Ann Rheum Dis 2013;72:1747-55 Table 1. Patients characteristics. IQR, interquartile range; ILD, interstitial Lung Disease; SSc, systemic sclerosis ACA+ (18) ACA - (18) p Age (years) at disease onset (median, IQR ) 47 (37-63 ) 47 (39-63 ) 0.834 Disease duration (months) (median, IQR ) 81 (62-169 ) 77 (58-165 ) 0.486 anti Ro52 antibody (% ) 12 (67 ) 11 (61) 1 Arthritis onset 10 (56) 13 (72) 0.489 Arthritis last follow-up (%) 14 (78) 14 (78%) 1 Myositis onset (%) 7 (39) 11 (61) 0.318 Myositis last follow-up (%) 15 (83) 16 (89) 1 ILD onset (%) 9 (50) 6 (33) 0.5 ILD last follow-up (%) 16 (89) 16 (89) 1 Complete form onset (%) 3 (17) 3 (17) 1 Complete form last follow-up (%) 10 (56) 11 (61) 1 Raynaud phenomenon (%) 13 (72) 9 (50) 0.305 Mechanic’s hands (%) 6 (33) 7 (38) 1 Teleangectasias (%) 2 (11) 0 (0) 0.486 Cutaneous sclerosis (%) 5 1 0.177 Acral ulcers (%) 1 (6) 0 (0) 1 Scleroderma pattern at NVC 8 (44) 7 (39) 1 Pulmonary arterial hypertension (%) 3 (17) 2 (11) 1 2013 ACR/EULAR SSc classification criteria 9 (50) 1 (6) 0.007 Disclosure of Interests: Giovanni Zanframundo: None declared, Gianluca Sambataro: None declared, Veronica Codullo: None declared, Alessandro Biglia: None declared, Emanuele Bozzalla Cassione: None declared, Elena Bravi: None declared, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Marco Fornaro: None declared, Konstantinos Triantafyllias: None declared, Alberto Pesci: None declared, Paola Tomietto: None declared, Øyvind Molberg: None declared, Salvatore Scarpato: None declared, Reinhard Voll: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Carlomaurizio Montecucco: None declared, Lorenzo Cavagna: None declared