The prevalence and incidence rate of pulmonary arterial hypertension in systemic sclerosis: Systematic review and meta-analysis |
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| Authors: | Manuel Rubio-Rivas Narcís A. Homs Daniel Cuartero Xavier Corbella |
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| Affiliation: | 1. Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China;2. Department of Epidemiology and Bio-statistics, Institute of Basic Medical Sciences, China Academy of Medical Sciences & Peking Union Medical College, Beijing, China;3. Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Beijing, China;1. CHRU de Nancy, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Vascular Medicine Division, Nancy F-54000, France;2. Inserm, UMR_S 1116, Nancy F-54000, France;3. Université de Lorraine, Nancy F-54000, France;4. NYU Langone Medical Center, Division of Rheumatology, New York, NY, USA;5. CHRU de Nancy, Cardiology Department, Nancy F-54000, France;6. CHRU de Nancy, Hematology Laboratory, Nancy F-54000, France;7. CHU de Saint-Étienne, Service de Médecine Vasculaire et Thérapeutique and Université Jean-Monnet and Inserm U1059 and INNOVTE Network, Saint-Étienne F-42023, France;8. CHRU de Nancy, Department of Respiratory Medicine and Lorraine University, INGRES, EA 7298, Nancy F-54000, France;9. CHRU de Nancy, Contrat d''interface, Nancy F-54000, France;10. Weill Cornell Medicine, Division of Cardiology, New York, NY, USA;11. Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, and Weill Cornell Medicine of Cornell University, New York, NY, USA |
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| 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).ConclusionThe 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). |
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