Abstract
BackgroundThere is no optimal screening tool for the assessment of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). A decreasing transfer factor of the lung for CO (TLCO) is associated with the development of PAH in SSc. TLCO can be partitioned into the diffusion of the alveolar capillary membrane (Dm) and the capillary blood volume (Vc). The use of the partitioned diffusion to detect PAH in SSc is not well established yet. This study evaluates whether Dm and Vc could be candidates for further study of the use for screening for PAH in SSc.MethodsEleven SSc patients with PAH (SScPAH+), 13 SSc patients without PAH (SScPAH-) and 10 healthy control subjects were included. Pulmonary function testing took place at diagnosis of PAH. TLCO was partitioned according to Roughton and Forster. As pulmonary fibrosis in SSc influences values of the (partitioned) TLCO, these were adjusted for fibrosis score as assessed on HRCT.ResultsTLCO as percentage of predicted (%) was lower in SScPAH+ than in SScPAH- (41 ± 7% vs. 63 ± 12%, p < 0.0001, respectively). Dm% in SScPAH+ was decreased as compared with SScPAH- (22 ± 6% vs. 39 ± 12%, p < 0.0001, respectively), also after adjustment for total fibrosis score (before adjustment: B = 17.5, 95% CI 9.0–25.9, p = < 0.0001; after adjustment: B = 14.3, 95% CI 6.0–21.7, p = 0.008). No difference was found in Vc%. There were no correlations between pulmonary hemodynamic parameters and Dm% in the PAH groups.ConclusionSScPAH+ patients have lower Dm% than SScPAH- patients. There are no correlations between Dm% and hemodynamic parameters of PAH in SScPAH+. These findings do not support further study of the role of partitioning TLCO in the diagnostic work- up for PAH in SSc.
Highlights
There is no optimal screening tool for the assessment of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc)
In this study we evaluate whether the components of the transfer factor of the lung for carbonmonoxide (TLCO), the conductance of the alveolar capillary membrane (Dm) and the pulmonary capillary blood volume (Vc) as assessed by the Roughton-Forster method [7], could be candidates for further studies in the search for tools for the diagnostic work-up for PAH in SSc patients
We investigated the relation between the two components of the lung for carbon monoxide (CO) (TLCO) and PAH, by calculating correlations between diffusion of the alveolar capillary membrane (Dm) and vascular level (Vc) and hemodynamic parameters obtained during right heart catheterisation
Summary
There is no optimal screening tool for the assessment of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). The use of the partitioned diffusion to detect PAH in SSc is not well established yet. This study evaluates whether Dm and Vc could be candidates for further study of the use for screening for PAH in SSc. Systemic sclerosis (SSc) is an autoimmune disease characterised by degenerative and fibrotic changes of skin, vasculature and internal organs. Patients with LcSSc are at higher risk of pulmonary arterial hypertension (PAH), which is the leading cause of death in this group of patients[2,3]. As therapeutic intervention implemented at an earlier phase might modify the disease course in SScPAH, new tools that assess PAH in patients with SSc are warranted[6]. Patient charts were reviewed from February 2004 onward, as since that date partitioned membrane diffusion measurements were consistently implemented according to the method described below
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