ARRC – Autoimmune Resource & Research Centre

Stress Associated Pulmonary Hypertension in Rheumatoid Evaluation (SAPHIRE)

Study Findings:   SAPHIRE (PDF Download)

Study Objective

The stress associated pulmonary hypertension in rheumatoid evaluation (SAPHIRE) study was conducted with following aims:

  1. Explore the prevalence of pulmonary hypertension in individuals with Rheumatoid Arthritis (RA) using stress echocardiography as a screening tool.
  2. To correlate simple clinical markers of exercise capacity with pulmonary haemodynamics during exercise to determine if assessment of these markers alone is an adequate predictor of the presence of pulmonary hypertension.
  3. Determine whether abnormal pulmonary vascular responsiveness with exercise correlated with a history of Raynaud’s phenomenon.
  4. Establish whether abnormal pulmonary vascular responsiveness with exercise is common in rheumatoid arthritis.

Study Rationale

Pulmonary arterial hypertension (PAH) has been reported to be a common cause of morbidity in rheumatoid arthritis (R/A), presenting with dyspnoea, palpitations and atypical chest discomfort. The prevalence of pulmonary pressure elevation & PAH in RA is not well-established, however, with some authors stating it is rather rare and others finding prevalence rates as high as 21% .  The condition is often silent, with the development of clinical symptoms often signalling the presence of advanced disease, associated with poor survival.

This has prompted the need for methods of detecting PAH, including annual echocardiographic screening of asymptomatic individuals with systemic autoimmunity. Therapeutic nihilism no longer surrounds this entity, with a range of treatments (phosphodiesterase inhibitors, prostacyclin analogues, and dual-receptor or single-receptor (ET-A) endothelin blockade) improving exercise capacity and survival. With these therapeutic developments, and given the irreversibility of advanced pathology associated with PAH, a new impetus has been provided for earlier detection of PAH in at-risk populations, with exercise proposed as a useful stress tool, albeit a method yet to possess clear normal cut-off ranges.

This completed study was an observational prevalence study aimed to confirm the reported increase prevalence of PAH in people diagnosed with rheumatoid arthritis.  For further enquiries, please email

This study is sponsored by