Background/Purpose: Cutaneous Telangiectasia (CT) are common in systemic sclerosis (SSc) patients but their input to risk stratify the patients is poorly known. The aims of our study were to determine whether the number and size of CT i) were related to the pattern of microvascular lesions assessed by nailfold videocapillaroscopy (NVC) and ii) were associated with other disease characteristics, and more particularly with markers reflecting the severity of SSc-related vasculopathy.

Methods: We performed a cross-sectional study including consecutive SSc patients over a 6-month period. We considered three subsets of patients according to the number of hand or face CT: absence of CT, ≤10 hand or face CT (minor to moderate CT) or >10 CT (profuse CT). Pseudotumoral CT were also taken into account and were defined as CT with > 5mm diameter. NVC was performed and classified as early, active and late patterns.

Results: 87 patients were included (69 females), with median age of 57 years (range 19-89 years), and median disease duration of 11 years (range 0-53 years). 52 patients (60%) had the limited cutaneous subset. 75 patients (86%) had CT: 27 (36%) had profuse CT, and 19 (25%) pseudotumoral CT.

In univariate analysis, patients with profuse and pseudotumoral CT were more likely to have the late NVC pattern (p=0.003 and p=0.001 respectively). Profuse and pseudotumoral CT were also associated with capillary loss (5.09±1.25 vs. 7.73±2.1 capillaries/digit, p<0.001, and 5.34±1.11 vs. 7.34±2.03 capillaries/digit, p=0.002, respectively) and neoangiogenesis (p=0.009 and p=0.003 respectively).

Regarding disease characteristics, patients with profuse or pseudotumoral CT were significantly older than patients without these lesions (p=0.018 and p=0.037, respectively); patients with profuse or pseudotumoral CT were also more likely to have increased sPAP on echocardiography (>40mmHg, p=0.007 and p=0.004, respectively) and increased plasma HS-cTnT levels (>14 ng/L, p<0.001 and p=0.025, respectively). The likelihood of pseudotumoral CT was significantly higher in patients with precapillary pulmonary arterial hypertension (PAH) (p=0.008). A trend was observed for an association between profuse CT and past or current digital ulcers (DU) (p=0.055).

Then, a logistic regression analysis was performed including profuse or pseudotumoral CT as outcomes together with covariates reflecting the severity of vascular involvement. This analysis revealed that profuse CT were independently associated with past or current DU (odds ratio, OR: 2.95, 95% confidence interval, CI: 1.09-16.93, p=0.034), and above the cut-off value of HS-cTnT (OR: 7.66. 95% CI: 1.07-54.85, p=0.043). Pseudotumoral CT were independently associated with late NVC pattern (OR: 4.84, 95% CI 1.32-26.19, p=0.018) and precapillary PAH (OR: 12.60, 95% CI 1.68-94.53, p=0.014).

Conclusion: We demonstrate that the number and size of CT are associated with the most severe NVC pattern. In addition, profuse and pseudotumoral CT identify a subset of patients with a more severe vascular phenotype. Thus, the number and size of CT may reflect the vasculopathy of SSc and could represent a clinical biomarker for vascular disease. 

ACR Meeting Abstracts - https://acrabstracts.org/abstract/skin-telangiectasia-identify-a-subset-of-systemic-sclerosis-patients-with-severe-vascular-phenotype/