Background Cutaneous telangiectasia (CT) are visible macular dilated superficial blood vessels. CT are an item of the new 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) (1) and are part of the newly established detection algorithm for SSc-related pulmonary arterial hypertension (2). These recent achievements highlight the importance of CT both for the diagnosis and prognosis of the disease. The number and size of CT can strongly vary from a patient to another according to the severity and progression of SSc-related vasculopathy. Nailfold videocapillaroscopy (NVC) is an accurate tool to evaluate microangiopathy allowing to discriminate vascular disease activity.
Objectives The aim of our study was to determine whether the number and size of CT were associated with specific NVC features.
Methods We performed a cross-sectional study including consecutive SSc patients over a 9-month period. We considered three subset 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 (3).
Results 87 patients were included (69 females), with a median age of 58 years (range 19-89 years), and a median disease duration of 11 years (range 0-53 years). 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 4.93±1.11 vs. 7.05±2.03 capillaries/digit, p=0.002, respectively) and neoangiogenesis (p=0.009 and p=0.003 respectively). Disease characteristics associated with profuse and pseudotumoral CT were older age (p=0.018 and p=0.038, respectively), a systolic pulmonary artery pressure over 40 mmHg measured by echocardiography (p=0.012 and p=0.005, respectively) and increased plasma levels of high sensitive cardiac troponin (>14 ng/L) (p<0.001, and p=0.025 respectively). No association was found between the number and size of CT and the presence of giant capillaries or microhaemorrhages, disease duration, cutaneous subset, or digital ulcers.
In multivariate logistic regression analysis, we confirmed that pseudotumoral CT were independently associated with the late NVC pattern (odds ratio, OR: 5.22, 95% confidence interval, CI, 1.09-29.95, p=0.038) and especially with neoangiogenesis (OR: 7.77, 95% CI 1.30-46.36).
Conclusions Our study reveals an independent association between pseudotumoral CT and the late NVC pattern. Our findings also show a relationship between pseudotumoral CT and neoangiogenesis, both characterized by ramified and bushy capillaries. These results support the development of a more accurate classification and staging of CT. This simple clinical finding might improve risk stratification of the vascular risk of SSc patients.
Van den Hoogen et al, Ann Rheum Dis 2013
Coghlan et al, Ann Rheum Dis 2014
Cutolo et al, J Rheumatol 2000
Disclosure of Interest None declared