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P.153 Differentiating active vs. chronic systemic sclerosis-primary heart involvement: clinical and prognostic implications

jsrd · 2026-06-05 · canonical JSON source

10 visible annotations · policy: published · automated confidence ≥ 75.00%

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Introduction Primary heart involvement (pHI) is a key driver of mortality in systemic sclerosis (SSc), underscoring the need for accurate stratification to improve outcomes. Cardiac MRI (CMR) is a valuable diagnostic tool, but its prognostic role is not fully established. A recent CMR-based classification proposed to distinguish SSc-pHI into ‘active’ and ‘chronic’ phenotypes 1: we aimed to assess its clinical and prognostic implications.Material and Methods SSc patients (ACR/EULAR 2013 criteria) followed in our center since January 2018 were screened for pHI 2; those with clinically suspected pHI underwent CMR. SSc-pHI was defined per current consensus3 and confirmed on CMR by >/= 1 feature: non-ischaemic late gadolinium enhancement, myocardial edema on T2 STIR, elevated extracellular volume, abnormal native T1 or T2 mapping.1 CMR-positive cases were classified as ‘active’ (edema +/elevated T2 +) or ‘chronic’ (edema -/ elevated T2-).1 Demographic, clinical and serological features at pHI diagnosis were recorded.Results Of 196 SSc patients screened, 51 underwent CMR, revealing pHI in 36 (18%). Compared to those without pHI, patients with SSc-pHI were more often male (p<0.001), had dcSSc (p=0.002) and shorter disease duration (p<0.001), more frequent ILD (p=0.05) and arthritis (p<0.001), and higher serum CRP (p=0.02) and hs-TnI levels (p<0.001). Mortality was higher in SSc-pHI patients (27% vs. 3%, p<0.001).Among pHI patients, CMR classified 50% as ‘active’ and 50% as ‘chronic’. The two groups were comparable in terms of demographics, skin subset, autoantibodies and structural CMR findings. Active pHI was associated with ILD (89% vs 50%, p=0.027), higher mRSS (p=0.045) and higher hs-TnI levels (p=0.048), compared with chronic pHI. Hs-TnI showed moderate diagnostic value for detecting active pHI at ROC analysis [AUC 0.69, 95% CI 0.51–0.86 (p=0.05); optimal cut-off > 32.25 ng/L (78% sensitivity, 56% specificity)]. At 12 months, active pHI had a lower survival [Log-rank test p=0.024, HR 0.13, 95% (CI 0.022-0.765)], though the difference was not significant at 24 months (p=0.112). Active pHI was more frequently treated with glucocorticoids (72%) and high-intensity immunosuppression (61%), while most chronic pHI (67%) received mycophenolate monotherapy.Conclusions Active SSc-pHI is associated with worse short-term survival, supporting the need for aggressive treatment and close monitoring. Concomitant ILD, higher mRSS and TnI levels may indicate active disease, with a significant role of hs-TnI in its detection.Abstract P.153 Table 1Differences between patients with chronic vs active SS-pHIAbstract P.153 Figure 1Survival analysis: patients with chronic vs active pHIReferences Batani V, et al, Rheumatology 2025.Bissell LA, et al, Rheumatology 2017.Bruni C, et al. J Scleroderma Relat Disord. 2022.