TY - JOUR T1 - Early risk prediction in idiopathic <em>versus</em> connective tissue disease-associated pulmonary arterial hypertension: call for a refined assessment JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00854-2020 VL - 7 IS - 3 SP - 00854-2020 AU - Clara Hjalmarsson AU - Barbro Kjellström AU - Kjell Jansson AU - Magnus Nisell AU - David Kylhammar AU - Mohammad Kavianipour AU - Göran Rådegran AU - Stefan Söderberg AU - Gerhard Wikström AU - Dirk M. Wuttge AU - Roger Hesselstrand Y1 - 2021/07/01 UR - http://openres.ersjournals.com/content/7/3/00854-2020.abstract N2 - Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to patients with idiopathic/hereditary pulmonary arterial hypertension (IPAH). We aimed to investigate differences in clinical characteristics, outcome and performance of the European Society of Cardiology (ESC)/ European Respiratory Society (ERS) risk stratification tool in these patient groups. This retrospective analysis included incident patients with CTD-PAH (n=197, of which 64 had interstitial lung disease, ILD) or IPAH (n=305) enrolled in the Swedish PAH Register (SPAHR) 2008–2019. Patients were classified as low, intermediate or high risk at baseline, according to the “SPAHR-equation”. One-year survival, stratified by type of PAH, was investigated by Cox proportional regression. At baseline, CTD-PAH patients had lower diffusing capacity for carbon monoxide and lower haemoglobin but, at the same time, lower N-terminal prohormone-brain natriuretic peptide, longer 6 min walk distance, better haemodynamics and more often a low-risk profile. No difference in age, World Health Organisation functional class (WHO-FC) or renal function between groups was found. One-year survival rates were 75, 82 and 83% in patients with CTD-PAH with ILD, CTD-PAH without ILD and IPAH, respectively. The 1-year mortality rates for low-, intermediate- and high-risk groups in the whole cohort were 0, 18 and 34% (p&lt;0.001), respectively. Corresponding percentages for CTD-PAH with ILD, CTD-PAH without ILD and IPAH patients were: 0, 26, 67% (p=0.008); 0, 19, 39% (p=0.004); and 0, 16, 29% (p=0.001), respectively. The ESC/ERS risk assessment tool accurately identified low-risk patients but underestimated the 1-year mortality rate of CTD-PAH and IPAH patients assessed as having intermediate risk at diagnosis.ESC/ERS risk assessment accurately identifies low-risk patients but underestimates the 1-year mortality rate of CTD-PAH and IPAH patients assessed as having intermediate risk at diagnosis https://bit.ly/3dDl5x8 ER -