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Early risk prediction in idiopathic versus connective tissue disease-associated pulmonary arterial hypertension : call for a refined assessment

Hjalmarsson, Clara ; Kjellström, Barbro LU ; Jansson, Kjell ; Nisell, Magnus ; Kylhammar, David ; Kavianipour, Mohammad ; Rådegran, Göran LU ; Söderberg, Stefan ; Wikström, Gerhard and Wuttge, Dirk M. LU , et al. (2021) In ERJ open research 7(3).
Abstract

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... (More)

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<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.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
in
ERJ open research
volume
7
issue
3
article number
00854-2020
publisher
European Respiratory Society
external identifiers
  • scopus:85136097922
  • pmid:34350280
ISSN
2312-0541
DOI
10.1183/23120541.00854-2020
language
English
LU publication?
yes
id
10431ce7-d1aa-40d4-8d79-e8f872fa894b
date added to LUP
2022-11-01 12:18:10
date last changed
2024-02-17 23:45:38
@article{10431ce7-d1aa-40d4-8d79-e8f872fa894b,
  abstract     = {{<p>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.</p>}},
  author       = {{Hjalmarsson, Clara and Kjellström, Barbro and Jansson, Kjell and Nisell, Magnus and Kylhammar, David and Kavianipour, Mohammad and Rådegran, Göran and Söderberg, Stefan and Wikström, Gerhard and Wuttge, Dirk M. and Hesselstrand, Roger}},
  issn         = {{2312-0541}},
  language     = {{eng}},
  month        = {{07}},
  number       = {{3}},
  publisher    = {{European Respiratory Society}},
  series       = {{ERJ open research}},
  title        = {{Early risk prediction in idiopathic versus connective tissue disease-associated pulmonary arterial hypertension : call for a refined assessment}},
  url          = {{http://dx.doi.org/10.1183/23120541.00854-2020}},
  doi          = {{10.1183/23120541.00854-2020}},
  volume       = {{7}},
  year         = {{2021}},
}