POS1270 THE IMPACT OF OVERLAP SYNDROMES ON THE SCLERODERMA CLINICAL TRIAL CONSORTIUM DAMAGE INDEX (SCTC-DI) IN SYSTEMIC SCLEROSIS

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Background Systemic sclerosis (SSc) is a systemic autoimmune disease leading to tissue atrophy, vascular damage and organ failures, and therefore causing severe disability. Coexistent autoimmune diseases are called overlap syndromes. Due to the distinct clinical picture[1], the extent of damage in SSc-overlap may be affected compared to pure-SSc. Objectives To identify differences in the time course and characteristics of damage between pure-SSc and SSc-overlap patients by evaluating the Scleroderma Clinical Trials Consortium-Damage Index (SCTC-DI)[2]. Methods Single tertiary care centre observational study with 160 enrolled SSc patients. Eighty-eight patients (55%) had diffuse cutaneous SSc (dcSSc), 86% were female and median disease duration was 9 years/4;16/. SSc-overlap was diagnosed based on the evaluation of the attending physicians. SCTC-DI was calculated. SCTC-DI score between 6-12 was considered as moderate and >12 points as severe damage. Damage profile of pure-SSc and SSc-overlap including subsets was compared. Results SSc-overlap was present in 24% of cases (n=39; 18 rheumatoid arthritis, 12 Sjögren, 12 myositis, 1 antiphospholipid syndrome). Age at enrolment and at disease onset, dcSSc/limited cutaneous (lcSSc) ratio, disease duration and gender distribution showed no difference between pure-SSc and SSc-overlap. Pure-SSc and SSc-overlap patients, including lcSSc/dcSSc subset comparison had the same damage burden (Kruskal-Wallis p>0.05). Median/IQR/ SCTC-DI was similar in pure-SSc and SSc-overlap (9/7;14/ and 9/6;13/ respectively). Moderate damage was found in around 53% and severe damage in 28% of patients in both pure-SSc and SSc-overlap. Gastrointestinal, cardiopulmonary, cardiac, vascular and renal domains of SCTC-DI did not differ between SSc-overlap and pure-SSc, whereas proximal muscle weakness was more prevalent in SSc-overlap compared to pure-SSc. ILD was scored similarly frequently in pure-dcSSc and dcSSc-overlap (82% vs 66.7%, χ² p>0.05), however, it was also present in 65% of pure-lcSSc and 60% of lcSSc-overlap patients. Severe damage was more prevalent in SSc-overlap patients at early stage of SSc (≤3 years duration, Fisher’s p=0.029) compared to pure-SSc. Early severe damage was more frequent in dcSSc-overlap compared to pure-dcSSc (Fisher’s p=0.042). Although in pure-SSc there were weak—to-moderate correlations between the SCTC-DI, age, and disease duration (Spearman’s rho:.360-.498;p<0.001), no such associations were found in SSc-overlap patients. Differences in SCTC-DI items in pure-SSc and SSc-overlap are shown in Table 1. Conclusion SCTC-DI is a relevant tool to assess damage in SSc-overlap. In this cohort damage was not related to ageing and disease duration in SSc-overlap. Early dcSSc-overlap patients had higher risk to develop severe damage compared to early pure-dcSSc patients based on the SCTC-DI. Pulmonary damage was less frequent in SSc-overlap compared to pure-dcSSc, but similar in pure-dcSSc and dcSSc-overlap patients. As expected, musculoskeletal damage, especially proximal muscle weakness was present in a remarkable proportion of SSc-overlap patients. References [1]Moinzadeh P et al.; Ann Rheum Dis. 2015 Apr;74(4):730-7. [2]Ferdowsi N et al.; Ann Rheum Dis. 2019 Jun;78(6):807-816. Acknowledgements Project no. TKP2021-EGA-10 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary, financed under the TKP2021-EGA funding scheme. Disclosure of Interests None Declared.

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