Systemic sclerosis (scleroderma, SSc) is a chronic multisystem autoimmune disease characterised by fibrosis of the skin and internal organs and vasculopathy. One of the major contributors to mortality in patients with SSc is pulmonary arterial hypertension (PAH). International recommendations advise annual screening for the early detection of PAH in asymptomatic patients with SSc.
To evaluate by systematic review current measures employed for screening for PAH. To summarise by meta-analysis the current evidence for long-term outcomes of screening for PAH in SSc.
Manuscripts published until 12th March 2019 were identified through searching Medline, Embase and Cochrane Central Register of Controlled Trials and Database of Systematic Reviews. Eligible studies included abstracts or full reports investigating patients with SSc undergoing screening by any protocol to detect PAH. Risk of bias was assessed with reference to the QUADAS-2 tool.
The review resulted in 580 unique citations with 15 manuscripts included for final systematic review of screening methods, and six for meta-analysis. The systematic review demonstrated that there are varying protocols for screening for PAH. Screened populations were reported to have better risk stratification parameters at PAH diagnosis. Meta-analysis showed improved survival in patients with SSc-PAH diagnosed as a result of screening. There were trends towards having better risk stratification parameters at PAH diagnosis in those screened, although not all of these were statistically significant.
There are no randomised controlled trials of screening for PAH in patients with SSc and the evidence presented in this review is derived from publications of registry data, cross-sectional and cohort studies.
This review demonstrates long-term benefit through the systematic screening of patients with SSc of varying disease duration for the early detection of PAH. Screened cohorts had improved survival, and were more likely to have better prognostic factors at the time of diagnosis with PAH.