Reviews
The prevalence of pulmonary arterial hypertension in patients with mixed connective tissue disease: a systematic review and meta-analysis
A.B. Hassan1, R.F. Hozayen2, Z.S. Mustafa3, I.E. Lundberg4, H.A. Jahrami5
- College of Medicine and Medical Sciences, Arabian Gulf University (AGU), Bahrain; and University Medical Center (UMC), King Abdullah Medical City (KAMC), Bahrain. adla.hassan@gmail.com
- College of Medicine and Medical Sciences, Arabian Gulf University (AGU), Bahrain; and Internal Medicine Department, Salmaniya Medical Complex, Bahrain.
- College of Medicine and Medical Sciences, Arabian Gulf University (AGU), Bahrain; and Internal Medicine Department, Salmaniya Medical Complex, Bahrain.
- Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm; and Department of Gastro, Dermatology, Rheumatology, Karolinska University Hospital, Stockholm, Sweden.
- College of Medicine and Medical Sciences, Arabian Gulf University (AGU), Bahrain; and Ministry of Health, Bahrain.
CER16621
2023 Vol.41, N°11
PI 2301, PF 2311
Reviews
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PMID: 37497727 [PubMed]
Received: 25/02/2023
Accepted : 19/06/2023
In Press: 24/07/2023
Published: 14/11/2023
Abstract
OBJECTIVES:
The prevalence and outcome of mixed connective tissue disease-associated pulmonary arterial hypertension (MCTD-PAH) has not been well understood. Our aim was to review the current knowledge on the prevalence, severity, and mortality of MCTD-PAH. We also aimed to examine the prevalence trend of MCTD-PAH over the years.
METHODS:
PubMed/Medline, Embase, Scopus and Web of Science electronic databases were searched for the published randomised controlled clinical trials (RCTs) and observational/original studies on PAH in patients with MCTD from January 1972 to December 2020.
RESULTS:
The results were pooled using random-effects meta-analysis based on DerSimonian and Laird method. A total of 983 patients from eight studies were included in the meta-analysis (K=8, n=983). Pooled prevalence of PAH in MCTD patients was 12.53% [95% CI 8.30–18.48%] with significant level statistical heterogeneity (tau2=0.30, tau=0.55, i2 83.3%, H=2.13 Q(df,7)=31.90, p=0.001). There was no association between PAH and female gender or age. The percentage of deaths in MCTD patients due to PAH varied and reached up to 81.8%.
CONCLUSIONS:
This is the first systematic review and meta-analysis investigating the prevalence of PAH in patients with MCTD and it revealed an overall prevalence of PAH in patients with MCTD of 12.53%. Our results showed trends of reduced prevalence of MCTD-PAH over last four decade, reconfirmed the lower prevalence rate in recent studies, but revealed an increased mortality rate. We also determined the low impact of the age, gender, and interstitial lung disease on MCTD-PAH.
