SAT0263 MALIGNANT CONDITIONS IN SYSTEMIC SCLEROSIS PATIENTS, SINGLE CENTER EXPIRIENCE
Authors: Alexandra Balbir-Gurman, Menahem Nahir, Vika Shataylo, Doron Markovits, Yolanda Braun-Moscovici
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Internal organ complications (interstitial lung disease [ILD], pulmonary hypertension [PAH], renal crisis [SRC], gastrointestinal involvement [GIT], etc.) have significant influence on scleroderma (SSc) patients' morbidity and mortality; malignancy could contribute to SSc outcome. <h3>Objectives:</h3> We aim to assess the incidence of malignancy, clinical and laboratory factors associated with this complication. <h3>Methods:</h3> We identified 43 patients with biopsy and/or imaging confirmed malignant conditions (SSc-C); additionally, case-control study was performed with identifying SSc patients without cancer (All-NC, N=86) divided into two subgroups: matching age (Age-NC, N-43) and matching disease duration (DD-NC, N=43). <h3>Results:</h3> Since 2004, 424 SSc patients were assessed at our center (340 EUSTAR registry participants); 43 (10.1%) SSc-C patients (7 males [16.3%]; age [years, standard deviation, SD] 68.1[12.7]; age at SSc onset 52.9[14.9]; age at 1<sup>st</sup> cancer diagnosis 54.2[12.7]; smoking 12 [27.9] had various types of tumors: breast 9; lung 8; neck &amp; head 8 (brain-3, thyroid-3, parotis-1, laryngs-1); genito-urinary 8 (prostate-1, ovary-3, utery-4; GIT 6 (stomach 2, pancreas 2, colon 2); hematologic 3 (lymphoma 2, myeloma 1); skin 3; pheochromocytoma 1, carcinoid 2; sarcoma 1. Thirteen patients (30.2%) developed cancer three years before or after SSc onset; 7 patients had cancer long before SSc; 6 patients had two cancers. Comparison between SSc-C patients and subgroups Age-NC, DD-NC and All-NC patient's subgroup did not reveal any difference in term of disease subset, presence of antibodies to topoisomerase, centromere, RNA polymerase, and clinical features (digital ulcers, ILD, GIT &amp; heart involvement, PAH, myositis, polyautoimmunity); there were no cases of SRC in SSc-C patients compared to 8 (9.3%) patients in All-NC patients. Twenty-six (60.5%) SSc-C patients died compared to 16 (37.2) in Age-NC (P&lt;0.08), DD-NC (P&lt;0.001), All-NC (P&lt;0.001). SSc related death was reported in 22.1% of All-NC patients and 16.3% of SSc-C (P=NS); 19 patients dead from cancer (73.1%). There were no differences between cancer and non-cancer patients in term of treatment with methotrexate, mycofenolate, azathioprine, cyclophosphamide, calcium blockers and iloprost; 24.3% SSc-C patients were treated with bosentan compared to 44.5% (P,0.015). <h3>Conclusion:</h3> Malignancy is an often and variable scleroderma complication; it could preclude SSc or appeared during SSc course. Patients with malignancy had higher mortality rate and mostly dead from cancer. There no cases of tumor in those patients who developed SRC. Patients treated with bosentan has less incidence of tumor. Prompt screening for cancer should be consider as a reasonable approach in caring SSc patients; early diagnosis and treatment of tumor may improve SSc patients' survival. <h3>Disclosure of Interests:</h3> None declared