RT Journal Article SR Electronic T1 Early risk prediction in idiopathic versus connective tissue disease-associated pulmonary arterial hypertension: call for a refined assessment JF ERJ Open Research JO erjor FD European Respiratory Society SP 00854-2020 DO 10.1183/23120541.00854-2020 A1 Clara Hjalmarsson A1 Barbro Kjellström A1 Kjell Jansson A1 Magnus Nisell A1 David Kylhammar A1 Mohammad Kavianipour A1 Göran Rådegran A1 Stefan Söderberg A1 Gerhard Wikström A1 Dirk M. Wuttge A1 Roger Hesselstrand YR 2021 UL http://openres.ersjournals.com/content/early/2021/04/15/23120541.00854-2020.abstract AB 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 ESC/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 2008–2019. Patients were classified as low, intermediate, or high risk at baseline, according to the “SPAHR-equation”. 1-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 walking distance, better hemodynamics, and more often a low-risk profile. No difference in age, WHO-FC, or renal function between groups was found. 1-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<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.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Hjalmarsson reports personal fees from Actelion Pharmaceuticals Sweden AB , grants from MSD, other from United Therapeutics, other from Arena, other from Acceleron, personal fees from Vifor Pharma, outside the submitted work.Conflict of interest: Dr. Kjellström reports grants from MSD, grants from Actelion Pharmaceuticals Sweden AB , outside the submitted work.Conflict of interest: Dr. Jansson reports grants, personal fees and other from Actelion Pharmaceuticals Sweden AB , grants, personal fees and other from Glaxo-SmithKline, personal fees from Bayer Health Care, personal fees and other from MSD, outside the submitted work.Conflict of interest: Dr. Nisell reports personal fees from Actelion Pharmaceuticals Sweden AB , personal fees from Glaxo-SmithKline, personal fees from Bayer Health Care, personal fees from Pfizer, personal fees from Nordic Infu, other from United Therapeutics, outside the submitted work.Conflict of interest: Dr. Kylhammar reports grants and personal fees from Actelion Pharmaceuticals Sweden AB , personal fees from Glaxo-SmithKline, grants from Bayer Health Care, grants from Pfizer, outside the submitted work.Conflict of interest: Dr. Kavianipour has nothing to disclose.Conflict of interest: Dr. Rådegran reports grants, personal fees and other from Actelion Pharmaceuticals Sweden AB , grants, personal fees and other from Glaxo-SmithKline, personal fees and other from Bayer Health Care, personal fees from Nordic Infu, personal fees from Sandoz/Novartis, other from United Therapeutics, other from Pfizer, other from Arena, other from Sanofi-Aventis, other from Acceleron, other from Ely-Lilly, outside the submitted work .Conflict of interest: Dr. Söderberg reports personal fees and other from Actelion Pharmaceuticals Sweden AB , outside the submitted work.Conflict of interest: Dr. Wikström reports personal fees from Actelion Pharmaceuticals Sweden AB , personal fees and other from Astra Zeneca, personal fees and other from Vifor Pharma, personal fees from Orion Pharma, outside the submitted work;Conflict of interest: Dr. Wuttge has nothing to disclose.Conflict of interest: Dr. Hesseltrand reports personal fees and other from Actelion Pharmaceuticals Sweden AB , personal fees from Roche Sweden AB, personal fees and other from Boehringer-Ingelheim Sweden AB, other from United Therapeutics, outside the submitted work.