Background Early diagnostics PAH, associated with SSc is very important, due to devastating nature of the disease. Borderline and exercise-induced PH is a condition thought to represent early-stage pulmonary arterial vasculopathy.
Methods We selected 21 patients with SSc (age 52+10 years, all female) with normal resting mean PAP (17 (14;21) mm Hg) who underwent graded bicycle supine exercise right heart catheterization (RHC). Patients were included in this study if they had 1) a pulmonary artery wedge pressure (PAWP) ≤15 mmHg by RHC; 2) no significant interstitial lung disease; 3) no left heart diseases like systemic hypertension, ischemic heart disease etc. Mean pulmonary artery pressure (mPAP), cardiac output (CO), and others were estimated invasively. Cut-off values of mean PAP ≥30 mm Hg at peak exercise were considered exercise-induced pulmonary hypertension (EI-PH), mean PAP 21-24 mm Hg so-called borderline pulmonary hypertension (Bo-PH). The risk of PAH development was calculated by DETECT PAH risk calculator.
Results 11 of 21 pts had risk factors by DETECT calculator. We didn't find PH at rest RHC in none of 21 pts. 6 SSc pts had mPAP level between 21-24 mm.Hg. 10 (48%) patients showed EI-PH (median level of mPAP =36 (32; 42) mm Hg), 7 hadn't changes in pressure follow exercises. 4 pts were excluded due to low exercise tolerance. We revealed significant correlation between the level of mPAP and DETECT total risk score (r=0,55, p<0,01). We found that patients with EI-PH had significantly higher DETECT PAH risk score than patients without EI-PH (38 (27; 42) vs 25 (16; 35), p<0,05). Calculated level of PAH risk in both DETECT steps were also different depending on existence Bo-PH (41 (34; 43) vs 28 (25; 36), p<0,02).
Conclusions Our small study assume that DETECT PAH risk calculator also may help to recognize SSc patients who have preclinical stage of PAH.
Coghlan JC, Denton CP, Grünig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014;73:1340–1349.
Saggar R. et al. Exercise-Induced Pulmonary Hypertension Associated With Systemic Sclerosis. Arthritis & Rheumatism, 2010, v.62(12), pp.3741-3750.
Disclosure of Interest None declared