Clinicoserologic factors predict PAH risk in SSc patients
medwireNews: An Australian research team has pinpointed several clinical and serologic parameters that identify systemic sclerosis (SSc) patients at risk of developing pulmonary arterial hypertension (PAH).
Among 1579 SSc patients without severe interstitial lung disease (ILD) enrolled in the Australian Scleroderma Cohort Study, the prevalence of PAH was 8.4%, while the annual incidence was 0.7%.
Multivariate analysis showed that the presence of anti-centromere antibody (ACA) significantly increased the PAH risk 1.6-fold, as did the occurrence of sicca symptoms and digital ulcers (p=0.03 for all). Calcinosis, esophageal stricture, and mild ILD were also significant predictors of PAH, raising the risk by a respective 1.9 (p=0.01), 2.0 (p=0.01) and 2.3 (p<0.001) times.
A model with these six variables predicted the development of PAH with 70% accuracy; the sensitivity, specificity, and positive and negative predictive values were 100%, 99.9%, 100%, and 91.8%, respectively.
Lead author Mandana Nikpour, from The University of Melbourne at St Vincent’s Hospital in Victoria, and colleagues point out that only two patients were positive for all six factors, but they found that the presence of any four or more of the variables “gave predictive properties similar to those presented for the complete model.”
Of interest, other than mild ILD, which was associated with increased PAH risk in patients with limited and those with diffuse cutaneous SSc, the factors predictive of PAH risk varied by SSc subtype.
Specifically, individuals with limited SSc had an elevated PAH risk in the presence of digital ulcers, calcinosis, and sicca symptoms, whereas among those with diffuse cutaneous disease, esophageal stricture and ACA were associated with an increased risk of developing PAH.
And using these variables in separate models for limited and diffuse cutaneous SSc predicted PAH development with an accuracy of 73% in each case.
“With the advent of potent pulmonary vasodilators and their associated impact on patients’ symptoms, quality of life and survival, it is imperative that we begin to detect PAH in its early stages to optimise care,” the study authors write in BMC Pulmonary Medicine.
They continue: “Whilst not a screening or diagnostic model, these models help to identify a subset of patients at an appreciably higher risk of developing PAH, who should be screened and would in future benefit from preventative therapies when these become available.”
medwireNews is an independent medical news service provided by Springer Healthcare Limited. © Springer Healthcare Ltd; 2016