Ab1209 Systemic Sclerosis and the Development of Neoplasms: A Single Center Experience

Background: Systemic Sclerosis (SSc) is an autoimmune connective tissue disease with multiorgan involvement. It has been suggested that patients with scleroderma have a higher incidence of malignancy than general population. However, various estimates in cohorts of scleroderma patients have been reported, with prevalence data up to 14.2% and incidence ranging from 3.6% to 10.7%. Objectives: We present our experience of patients with SSc and cancer development in Southern Italy (Campania). Methods: Patients who met the 2013 American College of Rheumatology/European League Against Rheumatism criteria for SSc and were followed since 2000 were included. Data on disease onset, disease duration, autoantibodies, age, pulmonary hypertension and type of malignancy were taken into account for the period 2000-2023. We calculated the prevalence of sex, diffuse cutaneous Systemic Sclerosis (dcSSc) and limited cutaneous Systemic Sclerosis (lcSSc), autoantibody subset of the patient cohort. We calculated the prevalence of malignant neoplasms, average age at diagnosis and average age of onset of Raynaud's phenomenon compared to malignancy. Results: Of the 550 patients (female prevalence 89% vs. male 11%; limited subset 54.5% vs. diffuse 46.5%, autoantibody pattern ACA vs. Scl-70 63.6% vs. 36.4%), 4.9% developed different types of malignancy, (lcSSc no. 22 and dcSSc no. 5). Eleven were breast neoplasms (11 with lcSSc), three patients developed neoplasms of the lung, (3 with dcSSc); three patients developed papilliferous carcinoma of the thyroid (2 with lcSSc, 1 with dcSSc), three a colorectal adenocarcinoma (3 with lcSSc), one a prostate adenocarcinoma (1 lcSSc). Twenty-six were female patients, one was male. The mean age at the time of cancer diagnosis was 55.6 years, Raynaud's phenomenon (RF) started at a mean age of 42.3 years, and SSc was diagnosed at a mean age of 47.1 years. The mean time to development of any type of cancer was approximately 15.1 years after the onset of RF. Three patients were smokers/ex-smokers, seventeen had gastroesophageal reflux disease, thirteen had pulmonary fibrosis and three had pulmonary hypertension. Fourteen patients had been treated with DMARDs over time. Conclusion: The present descriptive study on SSc and cancer development provides data showing a potential association between the two entities. In accordance with the literature finding of a prevalence of 14.2% in a cohort of approximately 1700 patients, we found a high frequency of cancer development in patients with SSc (4.9%). This data shows an increased prevalence even compared to national data (4%). Therefore, careful monitoring and screening is necessary during follow-up of scleroderma patients. REFERENCES: [1] Lepri G, et al. Systemic Sclerosis Association with Malignancy. Clin Rev Allergy Immunol. 2022 Dec;63(3):398 416. [2] Tyndall Aj, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010 Oct;69(10):1809-15. [3] Dati ISS. Acknowledgements: NIL. Disclosure of Interests: None declared.