RÉSUMÉ
Objective:To analyze the clinical characteristics and predictive factors of SSc associated heart disease.Methods:The clinical data of patients with SSc from January 2016 to December 2021 in Ningbo Medical Center Lihuili Hospital were collected. Aight healthy controls come from the medicial examination center. They were divided into a positive group and a negative group based on whether heart involvement was present or not. The clinical manifestations of the two groups were compared by t test, Wilcoxon signed rank test and χ2 test and Logistic regression or ROC curve was used to analyze the prognostic risk of SSc associated heart disease. Then the transcriptome sequencing was used to analyze the differential gene expression. Results:①A total of 75 SSc patients were treated in our hospital, of which 6 patients with overlap syndrome and 1 patient with congenital heart disease were excluded. The clinical data of 68 patients were analyzed including 16 patients in the positive group and 52 patients in the negative group. Among the 16 patients with cardiac involvement, 12 patients (75.0%) had abnormal electrocardiogram, 9 patients (56.2%) with heart valve disease, 8 patients (50.0%) with abnormal cardiac structure and 8 patients (50.0%) with pericardial effusion. The biomarkers were elevated in 10 cases (83.3%). ②Univariate analysis showed that the positive group had a longer course of disease [120(11.2, 132) months vs 48(24, 90)months, Z=-2.08, P=0.037], and the rate of pulmonary arterial hypertension (50.0% vs 11.5%, χ2=11.07, P<0.001) and renal insufficiency(50.0% vs 3.8%, χ2=20.78, P<0.001) in the positive group were significantly higher than those in the negative group. Further Logistic regression analysis revealed that long course of disease [ OR (95% CI) =1.011 (1.001, 1.021), P=0.031], pulmonary arterial hypertension [ OR (95% CI) =5.431, 95% CI (1.065, 27.710), P=0.042] and renal insufficiency [ OR (95% CI) =30.444 (4.139, 223.938), P<0.001] were risk factors for SSc associated heart disease. ③Nail-fold videocapillaroscopy (NVC) was checked in 63 patients. The difference of abnormal NVC changes between the two groups was statistically significant (93.3% vs 58.3%, χ2=5.87, P=0.013). The total number of capillaries in the positive group was significantly less than that in the negative group [3.5(2, 4.8) vs 6 (5, 7), Z=-2.97, P=0.003]. Further ROC curve analysis showed that the total number of capillaries less than 4.5 predicted the occurrence of cardiac involvement (sensitivity was 80.0%, specificity was 83.8%), and the area under the ROC curve (95% CI) was 0.805 (0.061, 1.000, P=0.003).④The transcriptome of a total of 11 SSc patients (including 6 in the positive group and 5 in the negative group) and 8 healthy controls were analyzed to obtain the synchronously down regulated gene TNFRSF13B. The differences between the three groups were statistically significant ( χ2=11.88, P=0.003), especially in the positive group and the healthy controls( χ2=11.19, P=0.004). Conclusion:SSc patients with long course of disease accompanied by PAH and renal insufficiency are prone to have heart involvement. Early capillary endoscopy is also helpful to predict the risk of heart involvement. Moreover, TNFRSF13B genetic testing is helpful but further study is needed.