Abstract

Background Scleroderma or systemic sclerosis (SSc) is a systemic, chronic autoimmune disease characterized by great clinical heterogeneity. In recent years, studies have proven that there is a relationship between SSc and neoplasia. SSc is associated with an increased risk of certain types of cancer, particularly lung, liver, hematological, non-melanoma skin and urothelial cancer. Despite this increasement, the relative risk of developing cancer is still low in these patients. In the literature, neoplasms have been described in 3-11% of patients with SSc. Objectives Our objective is to analyze the epidemiological, clinical and analytical characteristics previously described as possibly linked to the development of a cancer in patients with systemic sclerosis (SSc) in the Vall d’Hebron Hospital cohort. Methods We analyzed 583 patients in the Vall d’Hebron Hospital cohort of SSc. The inclusion criteria were age > 18 years and the diagnosis of SSc limited, diffuse and SSc sine scleroderma. The different variables were analyzed by univariate statistical analysis with SPSS v21. Results We included 521 patients with diagnosis from January 1985 to August 2016. 88.7% were women, with an average age of 53. 94 patients were diagnosed with neoplasia. The analyzed variables and their statistical significance and the types of cancer developed by our cohort patients are shown in Fig 1. Conclusion In the analyzed cohort, a slow pattern in capillaroscopy, positivity for Pm/Scl and the use of ARBs is associated with the development of neoplasia in patients with SSc. It has an inverse association the positivity of Scl70 (ATA I), the active pattern in capillaroscopy and the use of azathioprine and CCBs. In our cohort 94 patients with SSc have developed cancer. The most prevalent types of cancer were breast cancer, hematologic malignancies and lung cancer, as shown in the literature.

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