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Phenotypic patterns and outcomes of connective tissue disease-associated pulmonary arterial hypertension: a Saudi

Noura Alturaif1, Saleh Alhammad2, Sameer Desai3

  • 1Lung Health Department, Organ Transplant Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Respiratory Medicine
|July 24, 2026
PubMed

Insights

Systemic lupus erythematosus-pulmonary arterial hypertension (SLE-PAH) is common in Saudi Arabia. Both SLE-PAH and other connective tissue disease-PAH (CTD-PAH) showed similar disease severity and treatment response, despite differing clinical features.

Area of Science:

  • Cardiology
  • Rheumatology
  • Pulmonology

Background:

  • Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is a serious condition with varied prognosis.
  • Systemic sclerosis is common in Western countries, while systemic lupus erythematosus (SLE) is more prevalent in Saudi Arabia and other non-Western regions.
  • Limited data exists on CTD-PAH phenotypes, outcomes, and risk stratification in the Middle East.

Purpose of the Study:

  • To characterize CTD-PAH in a Saudi cohort.
  • To identify predictors of adverse outcomes in CTD-PAH.
  • To compare SLE-PAH with other CTD-PAH subtypes.

Main Methods:

  • Retrospective analysis of adult CTD-PAH patients from the SAUDIPH registry (2004-2024).
  • Comparison of baseline characteristics and 2-year changes in REVEAL Lite 2 scores between SLE-PAH and other CTD-PAH subtypes.

Main Results:

  • Thirty-nine patients were included: 24 SLE-PAH and 15 other CTD-PAH.
  • SLE-PAH patients were younger at diagnosis and showed higher anti-dsDNA and lower complement levels.
  • Both groups had high-risk scores at baseline, with significant 2-year improvement in REVEAL Lite 2 scores regardless of SLE status or immunosuppression.

Conclusions:

  • SLE-PAH is the predominant CTD-PAH subtype in this Saudi cohort.
  • No statistically significant difference in disease severity or treatment response was observed between SLE-PAH and non-SLE-PAH.
  • Further research is needed to explore trends in multiorgan autoimmune involvement and complement levels.
Abstract

Keywords:
Connective tissue diseasePulmonary arterial hypertensionREVEAL risk scoreRisk stratificationSystemic lupus erythematosus

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