Abstract
Introduction/Objective In the present paper, we aimed to test the value of the red cell distribution width (RDW) coefficient of variation as a candidate biomarker for pulmonary arterial hypertension (PAH) in patients with connective tissue disorders (CTD), correlating it with the degree of cardiopulmonary impairment in these patients. Methods The study population included N = 141 patients with CTD and N = 59 patients affected by pulmonary hypertension of other etiologies, all referred to the Pulmonary Hypertension Clinic of the Cardiology Division of an Academic Hospital in Northern Italy for evaluation (including right catheterization). Clinical, instrumental, and laboratory data were collected and related to RDW and other full blood count indexes. Results Twenty out of 141 CTD patients (14%) received a diagnosis of PAH. In comparison to those without PAH, CTD patients with PAH displayed a larger RDW (14.9% (13.5-17.2) vs. 13.8% (13.1-15.0); p = 0.02) and a lower platelet count (205 (177‐240) × 109/l vs. 244 (197.5‐304.2) × 109/l; p = 0.005). Moreover, with respect to CTD patients without PAH, RDW was significantly larger also in PH of other etiologies. In contrast, the platelet count was significantly lower only in CTD-related PAH, with a value > 276 × 109/l being 100% sensitive in ruling out PAH. Finally, RDW, but not the platelet count, was related directly to systolic pulmonary arterial pressure (r = 0.381; p = 0.0008) and right ventricle diameter (r = 0.283; p = 0.015) and inversely to diffusing capacity of the lung for carbon monoxide (r = −0.325; p = 0.014). Conclusion RDW is a promising candidate biomarker for the screening and the prognostic stratification of PAH in CTD patients.
Highlights
Pulmonary hypertension (PH) is a clinical entity that comprises multiple conditions of different etiologies and pathophysiology, defined by the presence of a mean pulmonary arterial pressure equal to or greater than 25 mmHg, measured during invasive right heart catheterization (RHC) at rest [1]
The estimated prevalence and incidence of pulmonary arterial hypertension (PAH) are around 15-60 cases and 5-10 cases per million adult population, respectively [3]; roughly, half of these cases occur in association with connective tissue disorders (CTD), with a prevalence of 21-29% in mixed connective tissue disease (MCTD), up to 14% systemic lupus erythematosus (SLE) [4], and close to
As shown in the table, the patients with PAH were older and characterized by a significantly higher brain natriuretic peptide (BNP) and systolic pulmonary artery pressure (sPAP); the creatinine clearance and the DLCO were significantly lower in the case of PAH
Summary
Pulmonary hypertension (PH) is a clinical entity that comprises multiple conditions of different etiologies and pathophysiology, defined by the presence of a mean pulmonary arterial pressure (mPAP) equal to or greater than 25 mmHg, measured during invasive right heart catheterization (RHC) at rest [1]. PH (i.e., pulmonary arterial hypertension (PAH), characterized by a pulmonary artery wedge pressure ≤ 15 mmHg), on the other hand, is a rare disease that may complicate connective tissue diseases (CTD) [2]. The estimated prevalence and incidence of PAH are around 15-60 cases and 5-10 cases per million adult population, respectively [3]; roughly, half of these cases occur in association with CTD, with a prevalence of 21-29% in mixed connective tissue disease (MCTD), up to 14% systemic lupus erythematosus (SLE) [4], and close to Disease Markers. Lower rates have been reported in Sjogren’s syndrome (SS) [4] and in dermatomyositis/polymyositis (PM/DM) [6] Consensus exists that these high-risk patients should be screened for PAH, since CTD-related PAH has a severe prognosis, even poorer than the idiopathic form [7]; PAH is the leading cause of death in systemic sclerosis [8]. Despite the relatively good performance of the DETECT algorithm, no universally agreed approach by which to screen these patients exists, and novel biomarkers of PAH are actively pursued
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