Review
The prevalence and incidence rate of pulmonary arterial hypertension in systemic sclerosis: Systematic review and meta-analysis

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Highlights

  • The overall PAH prevalence is 6.4% (95%CI 5%–8.3%).

  • The PAH prevalence in lcSSc was 7.7% (95%CI 5.3–11.1) and in dcSSc 6.3% (95%CI 4.5–8.9).

  • The overall PAH incidence 18.2 cases per 1,000 person-years (95%CI 12–27.4).

  • The PAH incidence in lcSSc was 20.4 cases per 1,000 person-years (95%CI 10.1–41.1) and in dcSSc 16.6 cases per 1,000 person-years (95%CI 8.5–32.1).

Abstract

The present study aimed to assess the prevalence and incidence rate of pulmonary arterial hypertension (PAH) in Systemic Sclerosis (SSc). The review was undertaken using MEDLINE and SCOPUS from June 1962 to May 2019 and the terms: (“Scleroderma, Systemic”[MesH]) AND “Hypertension, Pulmonary”[MesH]. The Newcastle-Ottawa Scale (NOS) was used for the qualifying assessment. The inverse variance-weighted method was performed. Twenty-four studies were included in the global PAH prevalence study. They comprised data from 9804 SSc patients. The overall PAH prevalence found was 6.4% (95%CI 5%–8.3%). Fourteen studies were included in the PAH prevalence study for lcSSc. They comprised data from 4987 lcSSc patients. The PAH prevalence found in lcSSc was 7.7% (95%CI 5.3%–11.1%). Twelve studies were included in the PAH prevalence study for dcSSc. They comprised data from 1790 dcSSc patients. The PAH prevalence found in dcSSc was 6.3% (95%CI 4.5%–8.9%). Fifteen studies showed PAH incidence of an entire SSc cohort. They comprised data from 5926 SSc patients. The overall PAH incidence found was 18.2 cases per 1000 person-years (95%CI 12–27.4). Eight studies showed PAH incidence for lcSSc. They comprised data from 2721 patients. The overall PAH incidence found in lcSSc was 20.4 cases per 1000 person-years (95%CI 10.1–41.1). Seven studies showed PAH incidence for dcSSc. They comprised data from 942 dcSSc patients. The overall PAH incidence found in dcSSc was 16.6 cases per 1000 person-years (95%CI 8.5–32.1).

Conclusion

The overall PAH 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).

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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