Background/Purpose: A higher prevalence of cancer has been described in patients with systemic sclerosis (SSc), but the magnitude of this risk and the type of cancer vary among reports. Risk factors predisposing the development of malignancies in SSc patients are not well defined. The purpose of our study was to analyze the frequency and risk factors associated with cancer in a large single cohort of patients with SSc.

Methods: All cases of cancer diagnosed in a cohort of 662 patients classified as SSc according to the ACR/EULAR criteria, attended in a single referral scleroderma outpatient clinic in Brazil between 2010 and 2017, were reviewed and evaluated regarding demographic, clinical and laboratory features. We used the Brazilian National Cancer Institute database to compare the cancer prevalence in SSc patients with the expected rate of cancer in the general population, and to estimate the risk of each cancer in this group, pairing by age. Taking into account that all sample proportions have an observed incidence higher than the general population, the one-tail proportion test was used to compare the cancer incidence in the sample and the population. A logistic regression model was performed to find risk factors for cancer. Statistical significance was considered when p ≤ 0.05.

Results: ±± 13.91 years; p ≤±±±±±± 9.24 years; p = 0.029). When the frequency of cancer by gender in the SSc patients was compared with the predicted frequency of cancer in the Brazilian general population, it was found a significant association among female gender and cancer in general (p<0.001), as well as with breast (p<0.001), uterus (p=0.023), lung (p=0.013), colon (p=0.007), esophagus (p=0.012), stomach (p = 0.042), lymphoma/leukemia (p=0.007), thyroid (p=0.004) and melanoma (p=0.022); male gender was only associated with cancer in general (p=0.013). Multivariate logistic regression identified non diffuse SSc (OR 10.7, 95%CI 2.1-54.8, p=0.004), Sjogren syndrome (OR 3.4, 95%CI 1,4-8.5, p=0.007), peripheral neuropathy (OR 4.4, 95%CI 1.5-13.2, p=0.009) and anti-RNApolIII positivity (OR 5.1, 95%CI 1.2-22.7, p=0.032) as significant risk factors for cancer occurrence.

Conclusion: This study confirmed a higher frequency of cancer in a large SSc cohort and pointed out that non diffuse SSc, peripheral neuropathy, concomitant Sjogren syndrome and anti-RNA polymerase III positivity represent risk factors for the development of cancer in SSc.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/non-diffuse-ssc-peripheral-neuropathy-concomitant-sjogren-syndrome-and-anti-rna-polymerase-iii-represent-risk-factors-for-the-higher-frequency-of-cancer-in-a-large-single-cohort-of-patients-with-sys/