PO:31:161 | First-line diagnostic tests to intercept primary heart involvement in systemic sclerosis: clinical associations from the SPRING-SIR registry

Antonio Tonutti^1|2, Francesca Motta^1|2, Rossella De Angelis³, Valeria Riccieri⁴, Silvia Laura Bosello⁵, Fabio Cacciapaglia⁶, Veronica Codullo⁷, Giacomo De Luca⁸, Francesca Ingegnoli⁹, Dilia Giuggioli¹⁰, Clodoveo Ferri¹⁰, Marco Matucci-Cerinic⁸, Carlo Selmi^1|2, Maria De Santis^1|2 | ¹Department of Biomedical Sciences, Humanitas University Pieve Emanuele, Milan, Italy; ²Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital Rozzano, Milan, Italy; ³Università Politecnica delle Marche Ancona, Italy; ⁴Università degli Studi di Roma La Sapienza Roma, Italy; ⁵Università Cattolica del Sacro Cuore Roma, Italy; ⁶Università degli Studi di Bari Aldo Moro Bari, Italy; ⁷Reumatologia, Fondazione IRCCS Policlinico San Matteo Pavia, Italy; ⁸Università Vita-Salute San Raffaele Milano, Italy; ⁹Università degli Studi di Milano, Italy; ¹⁰Università degli Studi di Modena e Reggio Emilia, Italy

Background. Primary heart involvement (pHI) is an overlooked, poorly characterized complication of systemic sclerosis (SSc), associated with worse prognosis. To fill the gap in the literature, this study evaluates the risk factors for first level assessment, specifically ECG and echocardiographic alterations suggestive of SSc-pHI in the nationwide SPRING cohort. Materials and methods This was a retrospective, multicenter, cohort study. Out of 1922 SSc patients in SPRING, we excluded those with confounding cardiac conditions according to WSF/HFA (arterial hypertension, diabetes, ischemic heart disease, pulmonary disease, PAH, echocardiographic PASP >/= 40mmHg, renal impairment, scleroderma renal crisis), and those with incomplete ECG/echocardiographic assessment. Cross-sectional and longitudinal analyses were performed to identify risk factors for SSc-pHI, defined as the presence of at least one of the following domains: non-ischemic hypokinesia; left ventricle (LV) dilatation; diastolic dysfunction; arrhythmias; conduction blocks.

Results. Of 600 eligible patients, 90% were females, with a median age at SSc onset of 45 (IQR 35-55) and disease duration of 8 years (4-14); 22% had dcSSc, 43% ILD, 24% digital ulcers, 16% muscle weakness, 6.5% muscle atrophy, 53% esophageal and 22% intestinal involvement. Anti-Scl70 were positive in 41%, ACA in 39%. At baseline, 149 (25%) had signs of SSc-pHI. Diastolic dysfunction was the most common alteration (62%), followed by conduction block (34%), arrhythmia (11%), LV dilation (9%), and hypokinesia (3%). The alterations were not mutually exclusive. Weak but significant correlations were found between increasing PASP and arrhythmia, increasing PASP and diastolic dysfunction, and LV dilation and non-ischemic hypokinesia; diastolic dysfunction negatively correlated with LV dilation and arrhythmia (Figure 1). In the multivariable logistic regression (adjusted for age, sex, and duration), the risk of SSc-pHI was increased with dcSSc (OR 1.85; 95%CI 1.05-3.26), intestinal symptoms (OR 1.79; 95%CI 1.03-3.08), and age at SSc onset (OR 1.04; 95%CI: 1.02-1.06) (Table 1). During follow-up (data available for 125 subjects), 31 new SSc-pHI cases were recorded (cumulative incidence 25%). Overall mortality did not differ between patients with or without incident SSc-pHI. Cox proportional hazards regression (adjusted for age, duration, and sex) revealed muscle atrophy as the only risk factor for new-onset SSc-pHI (HR 2.83, 95%CI 1.01-7.73) (Table 2). Exposure to immunosuppressive and vasoactive treatments did not differ between patients with or without SSc-pHI.

Conclusions. pHI is a protean and severe complication possibly affecting one-quarter of SSc cases. Early detection is crucial, and patients older at disease onset, with the diffuse form, muscular involvement, and intestinal manifestations can be those at higher risk.