POS1230 ASSOCIATION OF CANCER AND ANTISYNTHETASE SYNDROME: A RETROSPECTIVE MULTICENTER STUDY

Abstract

BackgroundIdiopathic 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].ObjectivesThe 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.MethodsIn 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.ResultsAmong 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 or18FDG-positron emission tomography), systematic cancer screening (n= 3) and physical examination (n= 2). Patients with cancer were older (63.5 ± 8.27vs.52.1 ± 14.7 years-old, p = 0.0001), had lower CPK levels (541 ± 962vs.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 ± 0.25vs.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).ConclusionIn 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.0b013e31817d116fFigure 1: Flow chartTable 1:Factors associated with Cancer-Associated Myopathy in patients with antisynthetase syndrome.CharacteristicsEventyes/no (%)Bivariate analysisMultivariate analysisOR (95%CI)pOR (95%CI)pAge > 55 yearsNo3/61 (5%)Yes12/46 (21%)5.3 (1.41 to 19.89)0.017.3 (1.72 to 50.6)0.015FemaleMale7/32 (18%)Female8/75 (9.6%)2.1 (0.68 to 6.13)0.2FeverNo8/94 (7.8%)Yes7/13 (35%)6.32 (1.96 to 20.35)0.0034.12 (1.03 to 16.6)0.042CPK < 500 U/LNo29/181 (16.5)Yes2/8 (25.0)4.42 (1.15 to 16.9)0.034.2 (1.1 to 21.1)0.049Inflammatory rheuamtismNo8/45 (15.1%)Yes7/62 (10.1%)0.63 (0.21 to 1.9)0.4Interstitial lung diseaseNo1/22 (4.3%)Yes14/85 (14.1%)3.62 (0.45 to 29.1)0.22Skin involvementNo9/70 (11.4%)Yes6/37 (14%)0.78 (0.26 to 2.39)0.7Muscular involvementNo8/76 (9.5%)Yes7/31 (18.4%)0.47 (0.15 to 1.39)0.2Cardiac involvementNo14/103 (12%)Yes1/5 (16.6%)1.83 (0.19 to 17.6)0.5Acknowledgements:NIL.Disclosure of InterestsNone Declared.