Scleroderma “SOS”: Telangiectases a biomarker for pulmonary disease

There are significant associations between the increased numbers of telangiectases in patients with scleroderma and the presence of pulmonary arterial hypertension (PAH). Cutaneous telangiectases may be a manifestation of the vasculopathy of scleroderma that could serve as a clinical biomarker for pulmonary vascular disease.

Shah and colleagues scored matted, nonstellate telangiectases on 11 body areas of 147 adults who had scleroderma. Scores ranged from 0, indicating an absence of lesions, to 2, indicating the presence of 10 or more lesions. Patients also were evaluated for the presence of Raynaud phenomenon (RP) and cardiopulmonary disease.

Telangiectasia scores were strongly and positively correlated with several variables: disease duration, patient age, and right ventricular systolic pressure (RVSP). They were not linked with severe RP. For every 10-point increase in telangiectasia score, the average RVSP measurement rose by 10.9 mm Hg. Also, in patients with this magnitude of score increase, the adjusted relative odds of PAH (confirmed by right heart catheterization) were 12.4.

The authors noted that more studies are needed to determine whether the number of telangiectases may serve as an early clinical biomarker for the development of severe vascular disease, especially PAH.