ReviewThe prevalence and incidence rate of pulmonary arterial hypertension in systemic sclerosis: Systematic review and meta-analysis
Introduction
Systemic Sclerosis (SSc) is an autoimmune systemic disease of unknown origin. Pulmonary involvement in the form of pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) constitute the main causes of death [1,2] and it seems its ratio as a cause of death is rising over time along with the decrease of other causes such as scleroderma renal crisis (SRC).
So far, a few reports from single or multicentre cohorts have reported PAH prevalence and incidence based on right heart catheterism (RHC) [[3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32]]. Unfortunately, quite a few articles released are based on echocardiography findings, which is not the gold standard for PAH diagnosis.
Our study aimed to perform a systematic review and meta-analysis of the prevalence and incidence of PAH worldwide in SSc.
Section snippets
Medical literature search
Eligibility criteria were observational studies providing PAH data from an entire SSc cohort. The search was carried out by two independent investigators (M.R-R. and N.A.H.) through MEDLINE and SCOPUS databases between June 1962 and May 2019, using the terms: (“Scleroderma, Systemic”[MesH]) AND “Hypertension, Pulmonary”[MesH]. The search was completed by the bibliography review of every paper selected for full-text examination. It was restricted to papers written in English, Spanish, French,
Search results
The literature search identified thirty observational studies providing PAH prevalence or incidence of an entire SSc cohort or their subsets (lcSSc and/or dcSSc) (Fig. 1).
PAH prevalence
Twenty-four studies were included in the global PAH prevalence study (Table 1 and Fig. 1). They comprised data from 9804 SSc patients. The overall PAH prevalence found was 6.4% (95%CI 5%–8.3%) (I2 = 91.3 Q = 265.5 tau2 = 0.3 p < 0.001). Begg's method tau = −0.254 p = 0.083. Excluding the studies by Coghlan et al. [22,32]
Discussion
We hereby present the largest and most accurate metanalysis focused on the prevalence and incidence of SSc-related PAH. The overall prevalence found was 6.4% (95%CI 5%–8.3%) and the overall PAH incidence 18.2 cases per 1000 person-years (95%CI 12–27.4 cases per 1000 person-years). We have to take into account are based on the current, and still applied, the definition of PAH. It has been recommended new cut-offs and thus new definitions of PAH, but it is only a suggestion to date [45]. In case
Competing interests and funding
The authors declare no conflicts of interest. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Acknowledgments
The authors would like to thank Dr. Launay and the RESCLE Registry for their help providing not published data.
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