期刊
JOURNAL OF RHEUMATOLOGY
卷 37, 期 1, 页码 98-104出版社
J RHEUMATOL PUBL CO
DOI: 10.3899/jrheum.090697
关键词
TELANGIECTASES; SYSTEMIC SCLEROSIS; PULMONARY HYPERTENSION
类别
资金
- Scleroderma Research Foundation
- American College of Rheumatology Research and Education Foundations
- NIH [IK23AR052742, IP50 HL084946-01]
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [P50HL084946] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES [K23AR052742, T32AR048522] Funding Source: NIH RePORTER
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, I 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% Cl 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). Conclusion. 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. (First Release Dec 1 2009: J Rheumatol 2010:37:98-104: doi:10.3899/jrheum.090697)
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