Application of the 2022 European Society of Cardiology (ESC) Risk Assessment Model in Australian and Singaporean Systemic Sclerosis Patients with Newly Diagnosed Pulmonary Arterial Hypertension (PAH)

Zoe Brown¹, Dylan Hansen², Wendy Stevens³, Nava Ferdowsi⁴, laura ross⁵, Alannah Quinlivan⁶, Joanne Sahhar⁷, Gene-Siew Ngian⁷, Diane Apostolopoulos⁷, Jennifer G Walker⁸, Susanna Proudman⁹, Gim Gee Teng¹⁰, Andrea Hsiu Ling Low¹¹, Kathleen Morrisroe¹² and Mandana Nikpour¹³, ¹The University of Melbourne, Armadale, Australia, ²Department of Rheumatology, St Vincent’s Hospital Melbourne, Fitzroy, Australia, ³Department of Rheumatology, St Vincent's Hospital Melbourne, Fitzroy, Australia, ⁴St Vincents Hospital, Kew, Australia, ⁵St. Vincent's Hospital Melbourne, Fitzroy, Australia, ⁶St Vincent's Hospital Melbourne, Highett, Australia, ⁷Monash Health and Monash University, Clayton, Australia, ⁸Royal Adelaide Hospital, Adelaide, Australia, ⁹Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia, ¹⁰Alexandra Hospital, Department of Medicine, Division of Rheumatology, National University Health System, Singapore, Singapore, ¹¹Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore, ¹²The University of Melbourne at St Vincent’s Hospital Melbourne, Fitzroy, Australia, ¹³The University of Melbourne at St. Vincent’s Hospital Melbourne, Departments of Medicine and Rheumatology, Melbourne, Australia

Meeting: ACR Convergence 2023

Keywords: Cardiovascular, prognostic factors, pulmonary, risk assessment, Scleroderma, Systemic

Session Information

Date: Sunday, November 12, 2023

Title: (0609–0672) Systemic Sclerosis & Related Disorders – Clinical Poster I: Research

Session Type: Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Pulmonary arterial hypertension (PAH) patients can be stratified as low, intermediate, or high risk of 1-year mortality based on clinical, biochemical and haemodynamic prognostic variables measured at diagnosis. In 2022, the European Society of Cardiology (ESC) and European Respiratory Society (ERS) updated their risk assessment method which is intended to guide therapeutic strategy over time.(1)

Methods: We evaluated incident systemic sclerosis-associated PAH (SSc-PAH) in a large combined cohort of Singaporean and Australian patients. We applied the 2022 ESC 3- and 4-strata risk assessment at baseline and first follow up (within 2 years), respectively. Kaplan-Meier survival analyses and Cox proportional hazards regression models were used to evaluate survival according to risk score.

Results: At baseline (n = 200), the majority of SSc-PAH (72.2%) were intermediate risk according to the 2022 ESC 3-strata risk assessment, based on ten variables. At follow-up, according to the 4-strata risk assessment (based on three variables: WHO functional class, serum N-terminal pro-brain type natriuretic peptide and six-minute walk distance), half (53.5%) of the cohort were classified as low or intermediate-low risk (Figure 2). The 2022 4-strata risk model at follow up demonstrated statistically significant differences in survival between risk groups with low risk having better survival, and a change in risk category from high or intermediate risk to low or lower risk was associated with an improvement in survival (Figure 1). All three individual parameters were significantly associated with mortality at baseline and/or follow up and included WHO functional class, serum N-terminal pro-brain type natriuretic peptide and six-minute walk distance (Table 1).

Conclusion: The 2022 ESC risk assessment strategy at baseline and follow up accurately predicts survival in SSc-PAH and is sensitive to change. Treatment decisions for SSc-PAH should include risk assessments, aiming to achieve low risk status according to the 2022 ESC guidelines as this is associated with improved outcome.

Figure 1. Kaplan-Meier survival curve according to 2022 ESC risk score from PAH diagnosis to mortality applied at i. PAH diagnosis, ii. First follow up and iii. Change in risk score:
a.) All-cause mortality b.) PAH-related mortality

Table 1. Hazard ratios for all-cause and cardiac-cause mortality according to individual risk variables in combined ASCS and SCORE cohort

Disclosures: Z. Brown: Janssen, 6, Novartis, 6; D. Hansen: None; W. Stevens: None; N. Ferdowsi: None; l. ross: None; A. Quinlivan: None; J. Sahhar: Boehringer Ingelheim, 1, 6, Janssen, 5; G. Ngian: None; D. Apostolopoulos: None; J. Walker: None; S. Proudman: None; G. Teng: None; A. Low: Boehringer-Ingelheim, 6, Janssen, 6; K. Morrisroe: None; M. Nikpour: AstraZeneca, 2, 6, Boehringer-Ingelheim, 2, 6, GSK, 2, 6, Janssen Pharmaceuticals, 2, 5, 6.

To cite this abstract in AMA style:

Brown Z, Hansen D, Stevens W, Ferdowsi N, ross l, Quinlivan A, Sahhar J, Ngian G, Apostolopoulos D, Walker J, Proudman S, Teng G, Low A, Morrisroe K, Nikpour M. Application of the 2022 European Society of Cardiology (ESC) Risk Assessment Model in Australian and Singaporean Systemic Sclerosis Patients with Newly Diagnosed Pulmonary Arterial Hypertension (PAH) [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/application-of-the-2022-european-society-of-cardiology-esc-risk-assessment-model-in-australian-and-singaporean-systemic-sclerosis-patients-with-newly-diagnosed-pulmonary-arterial-hypertension-pah/. Accessed .

« Back to ACR Convergence 2023

ACR Meeting Abstracts - https://acrabstracts.org/abstract/application-of-the-2022-european-society-of-cardiology-esc-risk-assessment-model-in-australian-and-singaporean-systemic-sclerosis-patients-with-newly-diagnosed-pulmonary-arterial-hypertension-pah/