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Introduction Major adverse cardiovascular events (MACE) are a leading cause of mortality in systemic sclerosis (SSc). However, early predictors of cardiovascular involvement remain unclear. This study aimed to evaluate whether the first non-Raynaud’s phenomenon (non-RP) sign/symptom can predict the future occurrence of MACE in SSc patients.Material and Methods We conducted a monocentric, retrospective, long-term longitudinal study on 161 SSc patients fulfilling ACR/EULAR 2013 criteria. Patients were stratified based on their first non-RP manifestation into four categories: vascular [puffy hands, telangiectasias, fingertip lesions, pulmonary arterial hypertension (PAH), scleroderma renal crisis (SRC)], musculo-cutaneous (skin thickening, muscle/joint symptoms, calcinosis), gastrointestinal (either upper or lower), and pulmonary [radiological signs of interstitial lung disease (ILD) at HRCT, ILD-related dyspnea, cough]. Demographic, clinical, and serological data were collected. Patients were followed from the first non-RP sign/symptom. Kaplan–Meier curves were employed to estimate time to event survival (MACE, defined as 2018 FDA definition) and a Cox proportional hazards analyses were implemented to evaluate the impact of first non-RP sign/symptom type on the risk of MACE.Results During a median follow-up of 10 years (IQR 6–16; max 30 years), 14 out of 161 patients (8.7%) developed MACE (11 myocardial infarction, 3 stroke). Compared to the non-MACE group, patients with MACE were older at enrollment, RP onset, first non-RP symptom, and SSc diagnosis ( table 1). They also had higher prevalence of telangiectasias (p=0.024), PAH (p=0.015), dyslipidemia, systolic hypertension, and rare scleroderma-specific antibodies (Th/To, fibrillarin).Notably, the distribution of first non-RP manifestations differed between groups. Vascular involvement was more frequent in the non-MACE group (52.4% vs 21.4%, p=0.047), while pulmonary manifestations were significantly more common in the MACE group (28.6% vs 8.2%, p=0.015) (figure 1). No significant differences were observed in gastrointestinal or musculo-cutaneous involvement.Kaplan-Meier curves revealed a shorter MACE-free survival for patients experiencing first non-RP pulmonary manifestations [mean 18.8 years (13-23)], followed by gastrointestinal [mean 22.6 years (19-26)], musculo-cutaneous [mean 26.2 years (23-29)] and vascular [mean 27.9 years (25-30)], with a statistically significant log-rank test (Mentel-cox) of p=0.042 (figure 2). In multivariate Cox analysis, pulmonary involvement as first non-RP manifestation (HR 4.465, p=0.028) and, male sex (HR 5.999, p=0.023) were independently associated with MACE occurrence (table 2).Conclusions The type of the first non-RP manifestation in SSc would have prognostic implications. In this analysis, onset with pulmonary signs/symptoms and male sex appear to be significant predictors of MACE. These findings could help to better tailor cardiovascular risk assessment and monitoring strategies in SSc patients.Abstract P.160 Figure 1First non-RP sigm/symptom distribution based on the presence or absence of MACEAbstract P.160 Figure 2Kaplan-Meier curves showing MACE-free survival by first non-RP manifestationAbstract P.160 Table 1Dusagrapais and disase relaind feattresAbstract P.160 Table 2Multivariable cox proportiomal hazards regression analysis assessing the asociation between elinieal variables and the oceurronee of MACE