THU0635-HPR PREVALENCE AND CLINICAL CHARACTERISTICS OF NEOPLASIA AMONG A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS
Authors: J. L. Tandaipan Jaime, E. Riera Alonso, N. Gimenez Gomez, G. Ghio, L. Berbel Arcobe, S. Martinez Pardo
Journal: Annals of the Rheumatic Diseases (2020)

Background: Patients with Systemic Sclerosis (SSc) have increased risk of malignancy compared to general population. The specific risk factors and underlying physiopathological mechanisms are still unknown, although some studies suggest that a relationship between malignancies and certain antibodies can exist. Lung, breast and hematological cancers are the most frequently seen among these patients. Objectives: To describe the prevalence of malignancies in a cohort of SSc patients and analyze the epidemiological, clinical and immunological characteristics Methods: A retrospective observational study was conducted at a tertiary-level university hospital, including a cohort of patients with SSc (ACR/EURLAR 2013 criteria). The main variable was neoplasia prevalence and also, malignancy type, age, evolution of the SSc at the time of diagnosis and mortality were collected. Regarding SSc, demographic data, clinical and immunological characteristics, organ involvement, capillaroscopy findings and presence of other autoimmune diseases were collected. Results: A 15% of the 98 patients with SSc presented malignancies (80% women). The mean age at the time of diagnosis was 57±15 years old (table 1). The frequency of cancer was: 40% breast, 13% colon, 7% ovary and lung. 2 patients died (1 breast, 1 lung). The limited subtype (lSSc) was the most frequent (80%) and 33% showed overlap syndrome (26% Sjögren syndrome). Regarding clinical manifestations: 67% had telangiectasia, 33% pitting scars, joint and digestive involvement. Most frequently seen antibodies were: 67% anti centromere (ACA) and 20% anti topoisomerase (ATA). None of the patients presented anti-ARN polimerase III (ARN-pol), and 13% had none of them (triple negative). Active and early capillaroscopy patterns were seen in a 46% and 27%. SSc and cancer were diagnosed in less than 5 years difference among a 33% of the cohort. A relationship between age and cancer was detected (p=0,042). Patients with neoplasia were a mean of 10 years older than those without malignancies (IC95%: 1-19 years) Table 1. SSc with neoplasia n= 15(%) SSc without neoplasia n= 83(%) Female 12 (80) 76 (92) Mean age*(n; DE) 57(15) 52(17) Pre-scleroderma 1 (7) 11 (13) Limited 12 (80) 54 (65) Diffuse 2 (13) 12 (15) SINE 0 6 (7) Overlap syndrome 5 (33) 14 (17) Sjögren 4 (27) 10 (12) MCTD 1 (7) 2 (2) Rheumatoid Arthritis 1 (7) 3 (4) Myositis 1 (7) 0 Clinical m anifestations Telangiectasia 10 (67) 41 (49) Pitting Scars 5 (33) 11 (13) Joint 5 (33) 27 (33) Digestive 5 (33) 33 (40) Digital ulcers 4 (27) 11 (13) Calcinosis 4 (27) 12 (14) ILD 3 (20) 16 (19) PAH 3 (20) 8 (10) Cardiac 3 (20) 4 (5) Muscular 2 (13) 3 (4) Puffy Fingers 2 (13) 24 (29) Renal 0 2(2) Antibodies ACA 10 (67) 46 (55) ATA 3 (20) 12 (14) Anti-ARN 0 4 (5) Triple negative 2 (13) 23 (28) Capillaroscopy Early 4 (27) 19 (23) Active 7 (46) 39 (47) Late 0 3 (4) Treatment Calcium antagonists 11 (73) 52 (63) PPIs 7 (46) 33 (40) Corticosteroids 8 (53) 24 (29) DMARD 5 (33) 26 (31) *P&lt;0,05 test t-student MCTD (Mixed Connective Tissue Disease), ILD (Interstitial Lung Disease), PAH (Pulmonary Artery Hypertension), Triple negative (anti ARN, ACA and ATA negative antibodies), PPI (Proton Pump Inhibitor), ACE inhibitors (Angiotensin Converting Enzyme inhibitors), ARBs (Angiotensin II Receptor Blockers), DMARD (Disease-Modifying Anti-Rheumatic Drugs). Conclusion: Our study showed a similar prevalence of the most frequent neoplasia among patients with SSc compared to general population (around 15%). This prevalence is similar to other series. The only epidemiological factor related to neoplasia was the age; a major proportion of lSSc was detected but without statistical significance. In a third of the patients there were less than 5 years of difference between cancer and SSc diagnosis. No association was found between neoplasia and certain antibodies. We recommend further studies to evaluate the relationship between SSc and cancer. Disclosure of Interests: None declared