Telangiectasia (TA), one of the diagnostic criteria for systemic sclerosis (SSc), could be a clinical marker for the severity of vasculopathy, including pulmonary arterial hypertension (PAH). We designed a cross-sectional study: (i) to describe the whole-body distribution of TA, (ii) to assess the associations between the whole-body number of TA and the characteristics of patients, (iii) to determine whether the number of TA may be useful to discriminate SSc-PAH patients.

Patients were included in our National Referral Centre for Rare Systemic And Autoimmune Diseases if they fulfilled the 2013 ACR/EULAR criteria for SSc. They were excluded if they had received laser treatment. The whole-body number and distribution of TA were recorded at inclusion. The associations with patients’ characteristics were studied using univariate and multivariate linear regressions.

A total of 106 patients were enrolled, including 12 with PAH. The median (interquartile range) number of TA/patient was 30 (82.7). Their distribution was: 37.2% on the face, 33.2% on the upper limbs including 26.4% on the hands, 28.1% on the trunk including 17.1% for the upper part of the trunk, and 1.5% on the lower limbs. Using multivariate model, the whole-body number of TA was independently associated with PAH (P=0.038), pulmonary embolism (P=0.012), decreased glomerular filtration rate (P=0.038), increased soluble Endoglin (P=0.039) and male gender (P=0.033). The ROC analyses assessing the ability of TA to discriminate the presence of PAH revealed that the area under the curve was significant for the number of TA on the whole body [0.77 (0.57; 0.88)], on the hands and face [0.81 (0.57; 0.91)] and on the hands alone [0.77 (0.57; 0.89)].

In SSc-patients, TA were predominantly located on the face, hands and the upper part of the trunk. They may reflect the vasculopathy of SSc and could be a useful clinical biomarker for vascular disease, particularly for PAH, the most severe vascular complications of the disease.