Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension.
OBJECTIVE
Clinical markers are needed to identify scleroderma patients at risk for pulmonary arterial hypertension (PAH) since early therapy may improve survival. We investigated whether increased numbers of telangiectases in scleroderma associate with measures of pulmonary vascular disease.


METHODS
One hundred forty-seven consecutive adult patients with scleroderma were enrolled in this cross-sectional study and scored for the presence of matted telangiectases on 11 body areas. Per body area, telangiectases were scored as 0 if none were present, 1 if there were fewer than 10 telangiectases, and 2 if 10 or more telangiectases were counted. Linear regression analysis was performed to assess the association between right ventricular systolic pressure (RVSP) and telangiectasia score, adjusted for age, race, smoking status, scleroderma subtype, disease duration, and autoantibody status. Logistic regression analysis was performed with PAH by right-heart catheterization (RHC) as the dependent variable.


RESULTS
The mean telangiectasia score was 6.0 (SD 4.5, range 0-20). RVSP and telangiectasia score were positively correlated (r = 0.271, p = 0.001). The mean RVSP increased by 10.9 mm Hg for every 10-point increase in telangiectasia score (95% CI 3.6-18.3 mm Hg, p = 0.004), adjusted for potential confounders. The adjusted relative odds of PAH by RHC were 12.4 for patients with a 10-point increase in telangiectasia score (95% CI 1.78-85.9, p = 0.01).


CONCLUSIONS
Increased numbers of telangiectases strongly associate with the presence of pulmonary vascular disease. Telangiectases may be a clinical marker of more widespread aberrant microvascular disease in scleroderma.
Shah
Ami A
AA
Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Mason F. Lord Building Center Tower, Suite 4100, Baltimore, MD 21224, USA. ashah32@jhmi.edu
Wigley
Fredrick M
FM
Hummers
Laura K
LK
D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000378:metabolism*; D001794:Blood Pressure / Q000502:physiology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology; D008875:Middle Aged; D011651:Pulmonary Artery / Q000503:physiopathology*; D012595:Scleroderma, Systemic / Q000150:complications / Q000473:pathology; D013684:Telangiectasis / Q000209:etiology / Q000473:pathology
D015415:Biomarkers