@article{3117260,
    title = "Determinants of pulmonary arterial hypertension in scleroderma",
    author = "Plastiras, Sotiris C. and Karadimitrakis, Stylianos P. and Kampolis, and Christos and Moutsopoulos, Haralampos M. and Tzelepis, George E.",
    journal = "Seminars in Arthritis and Rheumatism",
    year = "2007",
    volume = "36",
    number = "6",
    pages = "392-396",
    publisher = "W B SAUNDERS CO-ELSEVIER INC",
    issn = "0049-0172",
    doi = "10.1016/j.semarthrit.2006.10.004",
    keywords = "systemic sclerosis; pulmonary arterial hypertension; prevalence;
echocardiography",
    abstract = "Objective To define risk factors associated with pulmonary arterial
hypertension (PAH) in a large cohort of patients with systemic sclerosis
(SSc).
Methods SSc patients undergoing screening for PAH by means of Doppler
echocardiography were identified and their charts were retrospectively
reviewed. In all patients, we recorded systolic pulmonary artery
pressure along with pulmonary function testing, clinical, and laboratory
data. PAH was defined as right ventricular systolic pressure equal or
greater than 40 mm Hg.
Results Of 114 SSc patients with echocardiographic measurements, PAH was
found in 33 (29%) patients. In a multiple logistic regression analysis,
the presence of pulmonary fibrosis on thoracic computed tomography (OR
6.78, CI 1.54 to 29.9), forced vital capacity less than 80% predicted
(OR 3.03, CI 1.1 to 8.35), and duration of Raynaud’s phenomenon
preceding the onset of skin changes for at least 3 years (OR 5.75, CI
1.9 to 17.41) were found to be independent predictors of PAH. Age,
disease duration, disease subtype, or autoantibodies were not associated
with PAH in our patients.
Conclusions The present analysis identified pulmonary fibrosis and
Raynaud’s phenomenon preceding SSc skin manifestations by at least 3
years as risk factors for PAH in our scleroderma cohort. Screening for
PAH in these high-risk patients may detect PAH at an earlier stage and
guide decisions on therapeutic interventions. (c) 2007 Elsevier Inc. All
rights reserved."
}