Cluster analysis reveals three clinical phenotypes of pulmonary artery hypertension associated with connective tissue diseases: insights into inflammation and immunity
Authors' conclusion
Distinct clinical phenotypes in CTD-PAH correlate with inflammatory activity and hemodynamic status, influencing treatment responses and prognosis.
Study context
- Population
- CTD-PAH patients, single centre, 2009 to 202495.1% female
- Participants
- 184 patients
- Exposure
- Three clinical phenotype clusters of CTD-PAH defined by inflammatory and immune markers
- Outcome
- Clinical phenotypes of CTD-PAH identified by hierarchical cluster analysis of:
- Inflammatory
- Immune markers (neutrophil lymphocyte ratio (NLR), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement C3 and C4)
Methodological detailssingle-centre, hierarchical cluster analysis
- Single-centre
- Hierarchical cluster analysis
Main finding
As reported in the abstract
Clinical phenotype clusters
Three distinct clusters identified
3
Observational data: associations, not effects.
Further findings
Underlying CTD by phenotype
- Most vasculitic patients were SLE-PAH, with a shorter course and higher prevalence of autoantibodies
- Vasculopathic and mixed phenotypes common in SSc-PAH, UCTD-PAH and MCTD-PAH, associated with poorer treatment outcomes and survival rate
Original publication
Cluster analysis reveals three clinical phenotypes of pulmonary artery hypertension associated with connective tissue diseases: insights into inflammation and immunity. Arthritis Res Ther. Published 5 April 2025. doi:10. 1186/ s13075-025-03545-4
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