Abstract
IntroductionPatients with mean pulmonary artery pressures (mPAP) of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)—a condition thought to represent early-stage pulmonary arterial vasculopathy. Based on the DETECT study, this post-hoc analysis examined patient characteristics of systemic sclerosis (SSc) patients with normal mPAP, BoPAP and elevated mPAP, fulfilling pulmonary arterial hypertension (PAH) criteria.MethodsAdult patients with a duration of SSc more than 3 years, a diffusing capacity of the lung for carbon monoxide less than 60% predicted, and no previous diagnosis of any form of pulmonary hypertension (PH) underwent screening tests followed by right heart catheterization. Subjects were divided into three groups: normal mPAP, BoPAP, and PAH. Exploratory comparative and binary logistic regression analyses were performed for the BoPAP versus normal mPAP and PAH versus BoPAP groups.ResultsOf 244 patients evaluated, 148 (60%) had normal mPAP, 36 (15%) had BoPAP, and 60 (25%) had definite PAH. Univariable logistic regression (ULR) showed the mean tricuspid regurgitation velocity in patients with BoPAP to be intermediate between normal mPAP and PAH. In the ULR analyses BoPAP versus normal mPAP and PAH versus BoPAP, the statistically significant predictors were, amongst others: demographic, clinical, pulmonary function, echocardiographic and hemodynamic variables.ConclusionsIn this exploratory post-hoc analysis of the DETECT study population patients with BoPAP could be distinguished from patients with normal mPAP and PAH, and it appears that BoPAP may be an intermediate stage on the continuum between normal PA pressures and PAH.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-014-0493-1) contains supplementary material, which is available to authorized users.
Highlights
Patients with mean pulmonary artery pressures of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)—a condition thought to represent early-stage pulmonary arterial vasculopathy
Systemic sclerosis (SSc, scleroderma) is an autoimmune connective tissue disorder characterized by inflammation, fibrosis and vasculopathy
Patients were eligible for inclusion in DETECT if they were aged ≥18 years and had: 1) a definite diagnosis of systemic sclerosis (SSc) [16] of >3 years’ duration from first non-Raynaud’s symptom; 2) a diffusing capacity of the lung for carbon monoxide (DLCO)
Summary
Patients with mean pulmonary artery pressures (mPAP) of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)—a condition thought to represent early-stage pulmonary arterial vasculopathy. Based on the DETECT study, this post-hoc analysis examined patient characteristics of systemic sclerosis (SSc) patients with normal mPAP, BoPAP and elevated mPAP, fulfilling pulmonary arterial hypertension (PAH) criteria. Modern treatment has improved two-year survival estimates of SSc-associated PAH (SSc-PAH) from. Screening for SSc-PAH is capable of identifying earlier stages of the disease, resulting in earlier intervention that may improve survival [8]. DETECT was the first SScPAH detection study to evaluate all subjects with right heart catheterization (RHC), the gold standard test for the diagnosis of PAH [9]. Clinical, Visovatti et al Arthritis Research & Therapy (2014) 16:493 echocardiographic, serologic and functional testing data were collected
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call