A 10-YEAR JOURNEY OF CARING FOR PATIENTS WITH SYSTEMIC SCLEROSIS: FOLLOW-UP DATA ON DISEASE DURATION OF THE LEIDEN CCISS COHORT.

Background: Combined Care in Systemic Sclerosis (CCISS) is a prospective cohort of patients referred to Leiden University Medical Center for Raynaud’s Phenomenon (RP), a suspicion of systemic sclerosis (SSc) or a connective tissue disease. This cohort is characterized by its standardized and extensive annual follow-up. Since initiation of the cohort in 2009, diagnostic criteria for SSc have been updated leading to a higher sensitivity for early SSc (ACR 2013 criteria). A recent Dutch study showed that there is a gap to time of diagnosis between men and women, despite of overall increase of SSc awareness (PMID: 31539063). Objectives: To determine whether 1. time between first disease symptoms and diagnosis has changed over time, and 2. disease characteristics of SSc at first presentation in an expert clinic have changed over time for the total cohort, and between male and female patients. Methods: Patients included in the CCISS cohort undergo annual evaluation and clinical, laboratory, and imaging variables are systematically recorded. For this study, patients fulfilling the ACR/EULAR 2013 SSc criteria were included, and categorized into three groups based on the cohort entrance year: 1) 2010 – 2013, 2) 2014 – 2017, and 3) 2018 – 2021. SSc patients with a baseline visit in 2009 (n=65) were excluded as these patients were often not newly referred. Disease duration was defined by months since first RP, since first non-RP symptom and months between first date of diagnosis by a physician and first non-RP symptom. Disease characteristics included presence of interstitial lung disease (ILD), pulmonary arterial hypertension, digital ulcers (DU), diffuse cutaneous SSc, anti-topoisomerase and anticentromere antibodies. At baseline, disease duration and disease characteristics were compared between the three groups using appropriate tests. In addition, disease duration was compared between males and females in the three groups. Results: In total, 643 SSc patients were included of whom 229 (36%) had their baseline visit from 2010 until 2013, 207 (32%) from 2014 until 2017, and 207 (32%) from 2018 until 2021. The proportion of female patients was significantly higher in the 2010 – 2013 group compared to the 2014 – 2017 and 2018 – 2021 group ( Table 1 ). Over time, disease duration defined by RP duration and non-RP duration decreased as well as time between diagnosis and first non-RP symptom ( Table 1 ). The proportion of patients presenting with ILD and DU was highest in the first group ( Table 1 ). Table 1. 2010 - 2013 N=229 2014- 2017 N=207 2018 – 2021 N=207 P-value BASELINE Age, mean (SD ) 53 (15) 57 (14) 55 (14) 0.003 Female, % 86 76 75 0.010 RP duration, months (IQR ) 122 (46 – 240) 93 (20 – 202) 67 (20 – 210) 0.003 Non RP duration, months (IQR ) 43 (16 – 227) 20 (5 – 112) 17 (6 – 54) <0.001 Diagnosis duration, months (IQR ) 116 (80 - 177) 65 (45 – 105) 25 (5 – 45) <0.001 ΔRP and Non-RP, months (IQR ) 24 (0 – 99) 18 (0 – 118) 22 (0 – 120) 0.337 Anti-centromere antibodies, % 38 43 49 0.092 Anti-topoisomerase antibodies, % 24 24 18 0.259 Diffuse cutaneous SSc, % 19 23 16 0.073 Interstitial lung disease, % 43 31 31 <0.001 Pulmonary arterial hypertension, % 3 2 4 0.746 Digital ulcers, % 20 13 11 0.041 In both male and female SSc patients, disease duration and time between diagnosis and first non-RP decreased over time with a longer time in females for all durations which was significantly different for time between first RP and non-RP in 2014-2017 and 2018-2021 ( Figure 1 ). For the 2018 – 2021 group, duration since diagnosis for female was 26 (4 - 46) and male 17 months (7 – 39; p=0.355), and time between RP and non-RP for female 24 (0 - 168) and male 12 months (0 – 48; p=0.029). Figure 1. Conclusion: Over time, we observe a decrease in disease duration and in SSc patients presenting with ILD or DU at cohort entrance. Our results indicate increased awareness of early SSc and identification of SSc patients before severe complications have occurred. At the same time our results show the urge for specific attention to improve timely diagnosis in female SSc patients. Disclosure of Interests: None declared Citation: , volume 81, supplement 1, year 2022, page 758Session: Scleroderma, myositis and related syndromes (POSTERS only)