Thu0368 rapid Skin Thickness Progression Rate Is Associated With High Incidence Rate of Cardiopulmonary Involvement and Mortality in Patients With Early Diffuse Cutaneous Systemic Sclerosis (Dcssc)

Abstract

Background:There has been no prior inception cohort study regarding the incidence rate of cardiopulmonary involvement in patients with early dcSSc comparing among different groups of skin thickness progression rate.Objectives:The aims of this study were to compare differences in early dcSSc patients, which were classified into 3 subgroups; (a) rapid skin progression [RPsp], (b) intermediate skin progression [IMsp], and (c) slow skin progression [SLsp]; regarding: (1) clinical characteristics,(2) incidence rate of left ventricular ejection fraction (LVEF) < 50 %, interstitial lung disease (ILD),and pulmonary hypertension (PH). In addition, to compare mortality rate between skin improved and non-improved skin group.Methods:We used an inception cohort of early dcSSc patients seen at the Rheumatology clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and December 2017. All patients were assessed for demographic data, clinical manifestations, modified Rodnan Skin Score (mRSS) and underwent echocardiography, and HRCT at the study entry and then annually.Results:One hundred and two dcSSc patients (55 females and 89 anti-Scl 70 antibody positivity) with a mean±SD age of 53.2 ± 8.8 years and mean disease duration of 11.2 ± 8.7 months were enrolled, during a mean observation period of 54.0 ± 24.0 months. Mean±SD of baseline mRSS was 21.9 ± 9.1. Patients were classified into 3 groups: (a) 41 (40.2 %) patients with RPsp; (b) 37 (36.3 %) IMsp and; (c) 24 (23.5 %) SLsp. At enrollment, the RPsp group had significantly differences in clinical manifestations compared to IMSp and SLsp group as the followings: mean disease duration (5.4 ± 2.3 vs. 11.5 ± 6.1 and 20.7 ± 10.3 months, p < 0.001), anti-Scl 70 antibody- positive (95.1 % vs. 75.7 % and 91.7 %, p= 0.035), mRSS (26.4 ± 7.9 vs. 22.1 ± 8.4 and 13.8 ± 6.5, p < 0.001), dry mouth (48.8 % vs. 37.8 % and 8.3 %, p= 0.005), LVEF < 50 % (26.8 % vs. 21.6 % and 0, p= 0.010), pericardial effusion (0 vs. 10.8 % and 0, p= 0.018), arrhythmia (7.3 % vs. 29.7 % and 8.3 %, p= 0.018), muscle weakness (26.8 % vs. 27.0 % and 0, p= 0.009), creatine kinase ≥ 500 U/L (34.1 % vs. 18.9 % and 4.2 %, p= 0.016, and NT-proBNP (935.7 ± 2300.3 vs. 746.4 ± 1385.2 and 164.0 ± 225.3 ng/L, p= 0.001). During the observation period, the RPsp group had a significantly higher incidence rate of LVEF < 50 % compared with the SLsp group (6.06 vs. 0 per 100 person-years, incidence rate ratio= 21.27, p= 0.001). The RPsp group had a significantly higher incidence of ILD compared with the SLsp group (69.69 vs. 34.66 per 100 person-years, incidence rate ratio= 2.01, p= 0.012). Skin non-improved group had higher mortality rate compared with the skin improved group (28.6 % vs. 5.9 %, p= 0.004).Conclusion:Our study cohort found that early dcSSc patients with rapid skin progression had higher incidence rate of LVEF < 50 % and ILD compared to those with slow skin progression. In addition, skin non-improved patients had significantly higher mortality rates than those with skin improved.Disclosure of Interests:None declared

Clinical Features and Risk Factors of Rapidly Progressive Systemic Sclerosis in a Single Center in China: Anti-RNA Polymerase III Antibodies as a Predictor

“…In 2011, Robyn T Domsic et al [5] reported that rapid skin fibrosis could be a predictor of mortality and early internal organ involvement in dsSSc. While 2020 S. Wangkaew et al [13] found that rapid skin fibrosis was associated with high cardiopulmonary complications incidence in patients with early dsSSc. Rapid skin fibrosis and internal organ involvement severity and rate determines the optimal therapeutic interventions for SSc [7].…”