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Screening for pulmonary arterial hypertension in patients with systemic sclerosis in the era of new pulmonary arterial hypertension definitions


1, 2, 3, 4, 5, 6, 7, 8

 

  1. Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
  2. Department of Cardiology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey. burcakdr@yahoo.com
  3. Department of Cardiology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
  4. Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
  5. Department of Cardiology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
  6. Department of Chest Diseases, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
  7. Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
  8. Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.

CER17054
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PMID: 38489342 [PubMed]

Received: 10/08/2023
Accepted : 18/01/2024
In Press: 15/03/2024

Abstract

OBJECTIVES:
This study compares the performance of three composite pulmonary arterial hypertension (PAH) screening tools in a real-life SSc cohort, according to both the previous 2015 ESC/ERS guideline and the recent 2022 ESC/ERS guideline haemodynamic criteria.
METHODS:
Consecutive SSc patients without a previous diagnosis of pulmonary hypertension (PH) were screened for PAH using the European Society of Cardiology/European Respiratory Society (ESC/ERS), DETECT, and Australian Scleroderma Interest Group (ASIG) algorithms. Right heart catheterisation (RHC) referral performances for PAH were compared according to the 2022 ESC/ERS PAH criteria.
RESULTS:
Thirty-five of the 81 patients required RHC; 15 (18.5%) according to ESC/ERS, 27 (33.3%) according to DETECT, and 25 (31%) according to ASIG. The final diagnoses were no-PH in 17 patients, WHO group 1 PH (PAH) in 8 patients, WHO group 2 PH in 8 patients, and WHO group 3 PH in 2 patients. When the hemodynamic criteria of the previous ESC/ERS guideline were applied, only one patient was diagnosed with PAH. The sensitivities of the algorithms for the diagnosis of PAH were 62.5% for ESC/ERS, 75% for DETECT, 87.5% for ASIG according to the 2022 ESC/ERS guideline definition, and 100% for all according to the previous ESC/ERS guideline.
CONCLUSIONS:
With the recent criteria, PAH diagnosis in patients with SSc increased by 1.8-fold. Current algorithms for screening PAH are less sensitive with these revised criteria. Although the ASIG algorithm seems more sensitive, it can still miss the diagnosis. The multimodal/algorithmic approach seems to be the best option for predicting PAH.

DOI: https://doi.org/10.55563/clinexprheumatol/gzo4r2

Rheumatology Article

Rheumatology Addendum