Systemic sclerosis & Raynaud's
Dynamic Prediction of Pulmonary Hypertension in Systemic Sclerosis Using Landmark Analysis
Authors' conclusion
Our findings strongly support the importance of PFT trends over time in identifying patients at risk of developing PH.
Study context
- Population
- Systemic sclerosis (SSc) patients with disease onset ≥10 years prior to data retrieval and available autoantibody specificity and PFT data16.3% male
- Participants
- 1,247 patients
- Outcome
- Short-term risk of PH predicted from serial pulmonary function tests (PFTs) and other disease features
Methodological detailslandmark analysis, mixed-effects modeling, stratified Cox proportional hazards regression with landmarks as strata
- Landmark analysis
- Mixed-effects modeling
- Stratified Cox proportional hazards regression with landmarks as strata
Main finding
As reported in the abstract
Association of PFT values and their change over the preceding year with risk of PH (multivariable models)
- HR for Kco
- 0.93
- HR for change in Kco
- 0.76
- HR for DLco
- 0.90
- HR for change in DLco
- 0.96
- HR for forced vital capacity (FVC)/DLco ratio
- 1.08
- HR for change in FVC/DLco ratio
- 2.01
- P for all HRs
- P < 0.001 for all
- Kco-based model discrimination, Harrell's C-statistic
- 0.903
Observational data: associations, not effects.
Further findings
Incidence of pulmonary hypertension (PH)
- PH developed
- 13.6%
Annual decline in DLco and Kco
- Decline in all SSc patients, per year
- up to 1.5%
- Annual decline in DLco in the 5-7 years preceding PH diagnosis
- up to 4.5%
- Annual decline in Kco in the 5-7 years preceding PH diagnosis
- 4.8%
Autoantibody prevalence
- Anticentromere
- 29.8%
- Antitopoisomerase
- 22.0%
- Anti-RNA polymerase
- 11.4%
- dcSSc
- 35.8%
Original publication
Dynamic Prediction of Pulmonary Hypertension in Systemic Sclerosis Using Landmark Analysis. Arthritis Rheumatol. Published 22 February 2023. doi:10.
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