Benign and Malignant Tumor Burden in Patients with Systemic Sclerosis: A Retrospective Single-Center Study from the United Arab Emirates.
TL;DR
The findings reveal a distinctive malignancy profile characterized by a higher preva-lence of genitourinary, head, and neck, and gastrointestinal tumors compared to international co-horts, and an earlier average age of tumor onset highlighting unique regional characteristics.
Abstract
INTRODUCTION Malignancy is a significant comorbidity for patients with systemic scle-rosis (SSc). Data regarding the prevalence and risks of developing malignancy in SSc, particular-ly in the United Arab Emirates (UAE) population, are scarce. This study aims to investigate the proportion of benign and malignant tumors in the UAE's SSc patients.
Materials And Methods
SSc patients from the UAE Scleroderma Registry from April 2015 to April 2023 were characterized according to the presence of a tumor, whether benign or malig-nant. Descriptive statistics were utilized to examine the study's variables.
Results
Out of 133 patients, 33 (25%) had a history of a tumor. Benign and malignant tumors were reported in 54.5% and 42.4% of the cohort, respectively. The remaining 3.1% were tumors of unknown nature. Among the 133 patients, seven (21%) had genitourinary tumors, followed by 5 (15%) head and neck tumors, and 4 (12%) gastrointestinal tumors. The mean age of SSc diag-nosis was 38.9 ± 13.7 years. Among patients with tumors, there was SSc autoantibody positivity with topoisomerase I (Scl-70) (36%), anticentromere (ACA) (27%), and anti-polymerase III (ARA) (6%, p < 0.00001). IF-ANA was positive in 88% of patients with a predominantly centromere pattern (p < 0.05) and elevated titers ranging between 1:640 and 1:1280 (p < 0.05). Tumors were diagnosed a mean of 3.2 years before SSc diagnosis in some patients and a mean of 11 years after SSc diagnosis in others.
Discussion
Our findings reveal a distinctive malignancy profile characterized by a higher preva-lence of genitourinary, head, and neck, and gastrointestinal tumors compared to international co-horts, and an earlier average age of tumor onset highlighting unique regional characteristics.
Conclusion
The study is the first to describe tumor burden in the UAE's SSc population and highlights the need to tailor malignancy screening and management.