Skin Telangiectasia and the Identification of a Subset of Systemic Sclerosis Patients With Severe Vascular Disease

Abstract

Objective. Cutaneous telangiectasia (CT) are common in systemic sclerosis (SSc) patients, but their ability to stratify patients by risk is poorly known. We aimed to determine whether the number and size of CT were associated with the pattern of microvascular lesions assessed by nailfold videocapillaroscopy (NVC) and 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 also considered 3 predefined subsets of patients according to the number of hand or face CT: absence, £10, or >10 hand or face CT (profuse CT). Pseudotumoral CT were defined as CT with >5 mm diameter. Results. A total of 87 patients were included, of whom 75 (86%) had CT (27 with profuse and 19 with pseudotumoral CT). Profuse and pseudotumoral CT were both associated with capillary loss (P < 0.001 and P 5 0.002, respectively) and severe neoangiogenesis (P 5 0.015 and P 5 0.041, respectively), 2 hallmarks of the late NVC pattern. In multivariate analysis, profuse CT were independently associated with past or current digital ulcers (odds ratio [OR] 2.95 [95% confidence interval (95% CI) 1.09-19.63]), and pseudotumoral CT were independently associated with the late NVC pattern ) and with precapillary pulmonary hypertension . 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. Further prospective studies should determine whether CT number and size could serve as an early clinical biomarker for the development of severe vascular disease.

Combination of Capillaroscopic and Ultrasonographic Evaluations in Systemic Sclerosis: Results of a Cross‐Sectional Study

“…The proliferative obliterative vasculopathy characterizing Cutolo's late NFC pattern is especially associated with UAO in our study, supporting the hypothesis that micro-and macrovascular damages could be linked in SSc. These results are consistent with other studies considering skin telangiectasia as a marker of severe vascular disease in SSc (14). Our cross-sectional study reveals associations of macrovascular damages with digital ulcers but also with other digital manifestations of SSc such as acroosteolysis and calcinosis, both supposed to be the consequences of microvascular damages (2).…”

“…In our study, the association of UAO with the presence of skin telangiectasia also supports the hypothesis of a link between macrovascular obliterative damages and neoangiogenesis. These results are consistent with other studies considering skin telangiectasia as a marker of severe vascular disease in SSc (14). As suggested by L€ uders et al (5), a further characterization of other macrovessels of the hand such as proper palmar digital arteries would also be relevant to support this hypothesis.…”

Review: Defining a Unified Vascular Phenotype in Systemic Sclerosis

“…No multivariable analysis was performed, but it is of interest to point out that carotid atherosclerosis was not more common in SSc patients with digital ulcers than in those without digital ulcers. Regarding SSc characteristics, digital ulcers were more common in male patients and in those with dcSSc, and the MRSS was higher in patients with a history of digital ulcers, confirming previously reported findings in patient populations in other geographic regions . An evaluation of heart disease showed that the incidence of PAH was not higher in SSc patients with digital ulcers than in those without digital ulcers (9% and 10%, respectively).…”

Diagnostic value of nailfold videocapillaroscopy in systemic sclerosis secondary pulmonary arterial hypertension: a meta‐analysis

“…Recent studies predict that NVC has demonstrated vital results in the early identification of SSc-PAH. Hurabielle et al 17 reported that the echocardiography was more likely to demonstrate increased systolic pulmonary artery pressure (>40 mmHg, P = 0.007 and P = 0.004 respectively) when profuse or was assessed by NVC. Therefore, combining NVC with the DETECT algorithm may provide more accurate evidence for the early diagnosis of SSc-PAH.…”

“…Kim et al 16 also demonstrated that there was no difference in nailfold capillaroscopy parameters of pulmonary fibrosis, renal diseases, arthralgia and arthritis in SSc, but the number of capillaries in 3 mm and the loss of capillaries had been statistically and significantly lower in pulmonary hypertension and gastrointestinal dysfunction. Hurabielle et al 17 reported that the echocardiography was more likely to demonstrate increased systolic pulmonary artery pressure (>40 mmHg, P = 0.007 and P = 0.004 respectively) when profuse or was assessed by NVC. In summary, NVC, alone or in combination with other forms, such as the DETECT algorithm and echocardiography, is very significant for the early diagnosis of SSc-PAH.…”