SAT0348 CLINICAL SPECTRUM TIME COURSE OF ANTISYNTHETASE SYNDROME PATIENTS POSITIVE FOR ANTICENTROMERE ANTIBODIES

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 (+6 others)
2020 Annals of the Rheumatic Diseases  
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
more » ... 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;72:1747-55Table 1.Patients characteristics. IQR, interquartile range; ILD, interstitial Lung Disease; SSc, systemic sclerosisACA+ (18)ACA - (18)pAge (years) at disease onset (median, IQR)47 (37-63)47 (39-63)0.834Disease duration (months) (median, IQR)81 (62-169)77 (58-165)0.486anti Ro52antibody (%)12(67)11 (61)1Arthritis onset10 (56)13 (72)0.489Arthritis last follow-up (%)14 (78)14 (78%)1Myositis onset (%)7 (39)11 (61)0.318Myositis last follow-up (%)15 (83)16 (89)1ILD onset (%)9 (50)6 (33)0.5ILD last follow-up (%)16 (89)16 (89)1Complete form onset (%)3 (17)3 (17)1Complete form last follow-up (%)10 (56)11 (61)1Raynaud phenomenon (%)13 (72)9 (50)0.305Mechanic's hands (%)6 (33)7 (38)1Teleangectasias (%)2 (11)0 (0)0.486Cutaneous sclerosis (%)510.177Acral ulcers (%)1 (6)0 (0)1Scleroderma pattern at NVC8 (44)7 (39)1Pulmonary arterial hypertension (%)3 (17)2 (11)12013 ACR/EULAR SSc classification criteria9 (50)1 (6)0.007Disclosure 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
doi:10.1136/annrheumdis-2020-eular.3760 fatcat:vdcgvgdz4bbypkriynrbfyf2ke