ASSOCIATION OF CANCER AND ANTISYNTHETASE SYNDROME: A RETROSPECTIVE MULTICENTER STUDY

L. Bucy, H. Devilliers, P. Decker, B. Bonnotte, J. F. Chabot, P. Bonniaud, J. F. Maillefert, A. Meyer, A. Servettaz, J. Campagne, N. Magy-Bertrand, R. Jaussaud, T. MoulinetNancy University Hospital, Internal Medicine and Clinical Immunology Department, Vandoeuvre les nancy, France Dijon University Hospital, Department of Internal Medicine and Systemic Diseases, Dijon, France Dijon University Hospital, Internal Medicine and Clinical Immunology Department, Dijon, France University of Bourgogne-Franche Comté, INSERM U1098, Dijon, France Nancy University Hospital, Department of Pneumology, Nancy, France Lorraine University, INSERM UMR S1116, Nancy, France Dijon University Hospital, Reference Center for Rare Pulmonary Diseases, Pulmonary Medicine and Intensive Care Unit, Dijon, France University of Bourgogne-Franche Comté, INSERM, LNC UMR1231, LipSTIC LabEx Team, Dijon, France Dijon University Hospital, Rheumatology Department, Dijon, France Bourgogne-Franche Comté University, INSERM UMR 1093-CAPS, Dijon, France Strasbourg University Hospital, Rheumatology Department, Muscular Fonctionnal Exploration Unit, Strasbourg, France Strasbourg University, EA3072, Strasbourg, France Reims University Hospital, Internal Medicine, Clinical Immunology and Infectious Diseases Department, Reims, France University of Reims Champagne-Ardenne, Laboratory of Immunology, EA7509 IRMAIC, Reims, France Uneos, Department of Internal Medicine, Metz, France Besançon University Hospital, Internal Medicine Department, Besançon, France Lorraine University, UMR 7365 CNRS-Université de Lorraine IMoPA, Vandœuvre Les Nancy, France  Background Idiopathic inflammatory myopathies (IIM) are rare and heterogeneous conditions, associated with cancer in 10 to 30% of cases. While some features have been associated with cancer (age, male sex, dermatomyositis, anti-TIF1-γ antibodies), the association with cancer is more debated in antisynthetase syndrome (ASS). Few studies have assessed the prevalence of cancer during ASS meeting the criteria of cancer-associated myopathy (CAM)(cancer diagnosed within 3 years before or after the diagnosis of ASS) [1]. Objectives The main objective was to assess the prevalence of CAM in patients with ASS. Secondary objectives were to describe clinical features, biological features and prognosis associated with CAM in ASS patients. Methods In this retrospective multicenter study, we included patients of the main tertiary care centers of Grand-Est and Bourgogne-Franche Comté regions (MyositEst network). Inclusion criteria were age ≥ 18 years old, and Connors criteria fulfilment. Patients with low antisynthetase antibodies levels were excluded. Results Among 212 patients screened, 122 patients were included, with a median age of 53.5 ± 14.5 years old, and a female predominance (68%) (Figure 1). The most frequent manifestations of ASS were interstitial lung disease, myositis, skin involvement and inflammatory rheumatism (81%, 69%, 65% and 57%, respectively), followed by fever and cardiac involvement (16% and 4%, respectively). The most frequent specific antibodies were anti-JO1, anti-PL12 and anti-PL7 antibodies (61%, 17% and 10%, respectively), followed by anti-EJ and anti-OJ antibodies (7% and 1.6%, respectively). Four patients were positive for both anti-JO1 and anti-PL12 antibodies, and 1 was positive for both anti-JO1 and anti-PL7 antibodies. Among patients included, 15 (12.3%) met CAM criteria. The diagnosis of cancer was made following whole-body imaging (n=10, either CT-scan or FDG-positron emission tomography), systematic cancer screening (n= 3) and physical examination (n= 2). Patients with cancer were older (63.5 ± 8.27 vs. 52.1 ± 14.7 years-old, p = 0.0001), had lower CPK levels (541 ± 962 vs. 1628 ± 2404 U/L, p = 0.004), less frequently myalgia (20% vs. 46.7%, p = 0.05) and a higher MRC muscle scale (4.93 ± 0.25 vs. 4.6 ± 0.7, p = 0.0014). CAM patients had more frequent history of cancer (46.6% vs. 2.7%, p = 0.004) and had a higher mortality rate (33% vs. 5%, p = 0.003). Age above 55 years-old, fever and CPK below 500 U/L were associated with CAM both on bivariate and multivariate analysis (logistic regression model, entry p valule threshold of 0.2, OR = 7.3 [1.72 – 50.6], p = 0.01; OR = 4.12 [1.03 – 16.6], p = 0.04; and OR = 4.2 [1.1 – 21.1], p = 0.04, respectively)(Table 1). Conclusion In our study, CAM in ASS were more prevalent compared to literature data. CAM were independently associated with age, fever and low CPK levels. CAM patients had higher mortality. Careful physical examination, age and sex-based cancer screening, and whole-body imaging could be a relevant screening strategy. Reference [1]Rozelle A, Trieu S, Chung L. Malignancy in the setting of the anti-synthetase syndrome. J Clin Rheumatol 2008;14:285–8. doi:10.1097/RHU.0b013e31817d116f Image/graph:Figure 1 : Flow chart Table 1: Factors associated with Cancer-Associated Myopathy in patients with antisynthetase syndrome. Characteristics Eventyes/no (%) Bivariate analysis Multivariate analysis OR (95%CI) p OR (95%CI) p Age > 55 years No 3/61 (5%) Yes 12/46 (21%) 5.3 (1.41 to 19.89) 0.01 7.3 (1.72 to 50.6) 0.015 Female Male 7/32 (18%) Female 8/75 (9.6%) 2.1 (0.68 to 6.13) 0.2 Fever No 8/94 (7.8%) Yes 7/13 (35%) 6.32 (1.96 to 20.35) 0.003 4.12 (1.03 to 16.6) 0.042 CPK < 500 U/L No 29/181 (16.5) Yes 2/8 (25.0) 4.42 (1.15 to 16.9) 0.03 4.2 (1.1 to 21.1) 0.049 Inflammatory rheuamtism No 8/45 (15.1%) Yes 7/62 (10.1%) 0.63 (0.21 to 1.9) 0.4 Interstitial lung disease No 1/22 (4.3%) Yes 14/85 (14.1%) 3.62 (0.45 to 29.1) 0.22 Skin involvement No 9/70 (11.4%) Yes 6/37 (14%) 0.78 (0.26 to 2.39) 0.7 Muscular involvement No 8/76 (9.5%) Yes 7/31 (18.4%) 0.47 (0.15 to 1.39) 0.2 Cardiac involvement No 14/103 (12%) Yes 1/5 (16.6%) 1.83 (0.19 to 17.6) 0.5 Acknowledgements: NIL. Disclosure of Interests None Declared. Keywords: Myositis, Malignancy DOI: 10.1136/annrheumdis-2023-eular.1450Citation: , volume 82, supplement 1, year 2023, page 951Session: Scleroderma, myositis and related syndromes (Poster View)