Scleroderma Study Identifies Risk Factors for PAH Development

Researchers have identified risk factors of pulmonary arterial hypertension (PAH) in patients with scleroderma, showing that the risk profiles differ between patients with limited and diffuse cutaneous scleroderma.

The study, “Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study,” was published in the journal BMC Pulmonary Medicine.

It suggests that the findings should be implemented in screening programs, allowing early identification of patients who are at risk, especially since PAH is the leading cause of mortality among patients with scleroderma.

In an effort to improve the ability of PAH screening programs to correctly identify scleroderma patients at risk, researchers at the University of Melbourne in Australia followed 1,579 scleroderma patients in the Australian Scleroderma Cohort Study (ASCS) for an average of 3.2 years. Patients who had pulmonary hypertension caused by severe interstitial lung disease were not included in the study.

During the study, 8.4 percent of patients were diagnosed with PAH, and the majority of these patients (69 percent) had limited cutaneous scleroderma. Researchers noted that PAH-associated scleroderma patients more often had a range of symptoms, including stomach problems and digital ulcers, but an extensive analysis showed that only some of these symptoms could predict the development of PAH.

Scleroderma patients who had anti-centromere antinuclear antibodies (ACA); esophageal stricture; calcium tissue deposits (calcinosis); digital ulcers; sicca symptoms (also known as Sjogren syndrome); or mild interstitial lung disease had an increased risk of developing PAH, ranging from 1.6 to 2.3-fold higher than patients without such symptoms. Patients who had between four and six of the symptoms were almost certain to develop PAH.

Looking separately at patients with limited and diffuse scleroderma, researchers found that calcinosis, sicca symptoms, mild interstitial lung disease, and digital ulcers best predicted the development of PAH in limited scleroderma, and that 35 percent of patients with PAH had all four symptoms.

In diffuse scleroderma patients, it was instead esophageal stricture, mild interstitial lung disease, and the presence of ACA which were linked to PAH development. Only two out of 30 patients had all three symptoms.

Mild interstitial lung disease, in particular, which is not believed to lead to PAH, was a risk factor in patients with both limited and diffuse disease, indicating that the two disease processes may be linked.

The team concluded in its report that the study “identifies a subset of patients at an appreciably higher risk of developing PAH, who should be screened and would in future benefit from preventative therapies.”