EULAR 2016 | Daily Highlights
RISK FACTORS FOR MALIGNANCIES SYNCHRONOUS TO THE ONSET OF SYSTEMIC SCLEROSIS IN PATIENTS POSITIVE FOR ANTI- RNA POLYMERASE III ANTIBODIES: A EUSTAR MULTICENTRE STUDYAbstract: OP0031
Authors: M. G. Lazzaroni1,*, I. Cavazzana2, E. Colombo3, O. Distler4, R. Dobrota4, J. Hernandez4, R. Hesselstrand5, L. Czirjak6, C. Varju6, G. Nagy6, V. Smith7, P. Caramaschi8, V. Riccieri9, E. Hachulla10, K. Romanowska-Prochnicka11, A. Balbir-Gurman12, E. Chatelus13, A. C. Araùjo14, Y. Allanore15, P. Airò2
Co Authors: 1The DANBIO registry and the Danish Departments of Rheumatology, Copenhagen, Denmark
Patients with anti-RNA polymerase III antibodies (anti-RNAP) have an increased risk of malignancies synchronous to the onset of Systemic Sclerosis (SSc). In a multicentre EUSTAR case-control study, however, only moderate odd ratio was observed (OR 3.85, 95%CI 1.3-10.9), except than for breast cancer (OR 20.2, 95%CI 1.4-355) (1). Defining clinical and demographic features characteristic of anti-RNAP+ SSc patients who suffer from simultaneous malignancy would be useful to individuate risk factors who can guide the clinicians in every-day practice to screen anti-RNAP+ SSc patients for cancer.
To individuate risk factors for malignancies synchronous to the onset of SSc in anti-RNAP+ patients.
EUSTAR centers were asked to participate to this study, providing data on malignancy history of their anti-RNAP+ patients in a dedicated form.
Among 158 anti-RNAP+ SSc patients collected from 13 EUSTAR centers, 14 cases of malignancies synchronous to the onset of SSc were identified. When compared with the other 144 anti-RNAP+ patients, patients with synchronous malignancies had an older mean age at SSc onset (65.3 yrs (SD 10.0) vs 49.3 (SD13.3); p <0.001) and an increased proportion of diffuse cutaneous subset (96% vs 56%; p:0.008; OR: 10.5 95%CI 1.33-82.6). A trend for increased proportion of males was also observed (43% vs 21%; p:0.06). In particular, the risk of non-breast cancer synchronous with SSc was greatly higher in male than in female patients (43% vs. 0.8%; p<0.001; OR 95.2 95%CI 10.2-890).
Anti-RNAP+ SSc patients of older age, and with diffuse cutaneous subset, are particularly at risk for malignancies synchronous to the onset of SSc. The risk of malignancies other than breast cancers is greatly increased in male patients. These risk factors may help in the screening for cancer in these patients.
1) M.G. Lazzaroni et al. Anti-RNA Polymerase III Antibodies in Patients with Systemic Sclerosis: A Eustar Multicenter Collaborative Study. Ann Rheum Dis 2015;74:Suppl 2 88 doi:10.1136/annrheumdis-2015-eular.1728
Disclosure of Interest
Systemic inflammatory diseases are often associated with an increased prevalence of malignancies. For example, the association of Sjögren syndrome and lymphomas is well established. Dermatomyositis can occur as a paraneoplastic syndrome, e.g., the onset of dermatomyositis is closely linked to the onset of the malignancy. The current study adds anti RNA Polymerase III positive systemic sclerosis (SSc) to the potentially paraneoplastic connective tissue diseases. This finding is confirmatory to recently published analysis from other cohorts. Because this analysis is also done on a large number of patients from the EUSTAR database and in addition supported by mechanistic pathophysiological studies, this only recently detected association is likely to be a true finding. For clinical practice, this would mean that patient with newly diagnosed anti-RNA Polymerase III positive SSc would require screening for malignancies as often done for patients with dermatomyositis. Guidelines are currently developed to support clinicians in this approach.
Prof. Dr. Oliver Distler
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