Cluster analysis reveals three clinical phenotypes of pulmonary artery hypertension associated with connective tissue diseases: insights into inflammation and immunity

Arthritis Res TherPublished Retrospective cohort study

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:
  1. Inflammatory
  2. 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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