Prevalence of Sarcoidosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis
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ABSTRACT: Background
Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with poor prognosis, conferring up to a 10-fold increase in mortality in patients with sarcoidosis, but the actual prevalence of SAPH is unknown. Methods
The PubMed, Embase, and Cochrane Library databases were systematically searched for epidemiological studies reporting the prevalence of SAPH up to July 2021. Two reviewers independently performed the study selection, data extraction, and quality assessment. Studies were pooled using random-effects meta-analysis. Results
This meta-analysis included 25 high-quality studies from 12 countries, with a pooled sample of 632,368 patients with sarcoidosis. The prevalence of SAPH by transthoracic echocardiography in Europe, the United States and Asia was 18.8% [95% confidence interval (CI): 11.1–26.5%], 13.9% (95% CI: 5.4–22.4%) and 16.2% (95% CI: 7.1–25.4%) separately, and the overall pooled prevalence was 16.4% (95%CI: 12.2–20.5%). By right heart catheterization (RHC), the pooled prevalence of SAPH was 6.4% (95% CI: 3.6–9.1%) in general sarcoidosis population, and subgroup analyses showed that the prevalence of SAPH was 6.7% (95% CI: 2.4–11.0%) in Europe and 8.6% (95% CI: −4.1 to 21.3%) in the United States. Further, the prevalence of pre-capillary PH was 6.5% (95% CI: 2.9–10.2%). For the population with advanced sarcoidosis, the pooled prevalence of SAPH and pre-capillary PH by RHC was as high as 62.3% (95% CI: 46.9–77.6%) and 55.9% (95% CI: 20.1–91.7%), respectively. Finally, the pooled prevalence of SAPH in large databases with documented diagnoses (6.1%, 95% CI: 2.6–9.5%) was similar to that of RHC. Substantial heterogeneity across studies was observed for all analyses (I2 > 80%, P < 0.001). Conclusions
The sarcoidosis population has a relatively low burden of PH, mainly pre-capillary PH. However, as the disease progresses to advanced sarcoidosis, the prevalence of SAPH increases significantly.
SUBMITTER: Zhang S
PROVIDER: S-EPMC8801498 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Despite the recent increasing worldwide attention towards pulmonary hypertension (PH), its epidemiology remains poorly described in Africa. Accordingly, we performed a systematic review and meta-analysis of PH prevalence, incidence and etiologies in Africa.We searched PubMed, EMBASE, African Journals Online, and Africa Index Medicus. Published observational studies until September 20, 2017, including adult participants residing in Africa were considered. Two review authors independently selected studies, assessed included studies for methodological quality, and extracted data. A random-effects model was used for meta-analysis. Heterogeneity was evaluated by the ? 2 test on Cochrane's Q statistic which is quantified by I2 values. Using Newcastle-Ottawa Scale, we considered a score of 0-4, 5-7, and 8-10 as indicative of high, moderate, and low risk of bias in included studies, respectively.Of 1611 entries, 25 studies were retained. Twelve (48%), seven (28%), and six (24%) papers had respectively a low, moderate and high risk of bias. The prevalence of PH widely varied across different populations: 9.8% (95% confidence interval: 3.2-19.3; I2?=?99.4%; 6 studies) in 11,163 people presenting with cardiac complaints; 10.6% (4.3-19.1; I2?=?90.3%; 4 studies) in 937 HIV-infected people; 32.9% (17.6-50.4; I2?=?97.2%; 3 studies) in 2077 patients with heart failure; 23.2% (15.2-32.2; I2?=?59.4%; 3 studies) in 248 patients on hemodialysis; 12.9% (11.8-14.0; I2?=?79.7%; 2 studies) in 3750 patients with rheumatic heart disease; 36.9% (29.7-44.3; I2?=?79.7; 2 studies) in 79 patients with sickle cell disease; 62.7% (49.0-74.7; 1 study) in 51 patients with chronic obstructive pulmonary disease; 25.4% (16.3-37.3; 1 study) in 63 patients with systemic lupus erythematous; 68.7% (62.8-74.1; 1 study) in 259 patients with cardiac surgery; and 7.4% (4.6-11.9; 1 study) in 202 patients with systemic sclerosis. No study reported PH incidence. From one international study (n?=?209), PH etiologies were: left heart disease (68.9%), pulmonary arterial hypertension (15.8%), lung disease and/or hypoxia (12.0%), chronic thromboembolic PH (1.9%) and unclear/multifactorial PH (15.8%).The prevalence of PH is relatively high in some populations in Africa, perhaps mainly driven by left heart diseases, highlighting the need for context-specific interventions.
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Project description:Background Recent studies have demonstrated a continuum in clinical risk related to mean pulmonary artery pressure that begins at >19 mm Hg, which is below the traditional threshold used to define pulmonary hypertension ( PH ) of 25 mm Hg. Because of the implications on patient diagnosis and prognosis, the generalizability and validity of these data need further confirmation. Methods and Results Databases were searched from inception through January 31, 2018, to identify studies comparing all-cause mortality between patients with mildly elevated mean pulmonary artery pressure near but <25 mm Hg versus the referent group. The meta-analysis included 15 nonrandomized studies and 16 482 patients (7451 [45.2%] with measured or calculated mean pulmonary artery pressure of 19-24 mm Hg by right heart catheterization [n=6037] and echocardiography [n=1414] [mild PH ]). The mean duration of follow-up was 5.2 years. Compared with the referent group, mild PH was associated with an increased risk of mortality (risk ratio, 1.52; 95% confidence interval, 1.32-1.74; P<0.001; I2=47%). Secondary analysis using risk-adjusted time-to-event estimates showed a similar result (hazard ratio, 1.19; 95% confidence interval, 1.09-1.31; P<0.001; I2=42%). The findings were consistent between subgroups of right heart catheterization and echocardiography studies ( Pinteraction>0.05). There was evidence of publication bias; however, this did not influence the risk estimate (Duval and Tweedie's trim and fill adjusted risk ratio, 1.34; 95% confidence interval, 1.15-1.56). Conclusions The risk of mortality is increased in patients with mild PH , defined as measured or calculated mean pulmonary artery pressure >19 mm Hg. These data emphasize a need for diagnosing patients with mild PH with consideration to enrollment in PH clinical studies investigating pharmacological and nonpharmacological interventions to attenuate clinical risk and improve outcomes.
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Project description:BackgroundPulmonary hypertension (PH) is a severe complication of sarcoidosis in a minority of patients. Several genetic defects are known to cause hereditary or sporadic PH, but whether variants in PH-associated genes are also involved in sarcoidosis-associated PH (SAPH) is unknown.Methods40 patients with SAPH were individually matched to 40 sarcoidosis patients without PH (SA). Whole exome sequencing was performed to identify rare genetic variants in a diagnostic PH gene panel of 13 genes. Additionally, an exploratory analysis was performed to search for other genes of interest. From 572 genes biologically involved in PH pathways, genes were selected in which at least 15% of the SAPH patients and no more than 5% of patients without PH carried a rare variant.ResultsIn the diagnostic PH gene panel, 20 different rare variants, of which 18 cause an amino-acid substitution, were detected in 23 patients: 14 SAPH patients carried a variant, as compared to 5 SA patients without PH (p = 0.018). Most variants were of yet unknown significance. The exploratory approach yielded five genes of interest. First, the NOTCH3 gene that was previously linked to PH, and furthermore PDE6B, GUCY2F, COL5A1, and MMP21.ConclusionsThe increased frequency of variants in PH genes in SAPH suggests a mechanism whereby the presence of such a genetic variant in a patient may increase risk for the development of PH in the context of pulmonary sarcoidosis. Replication and studies into the functionality of the variants are required for further understanding the pathogenesis of SAPH.
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Project description:IntroductionThere are no data summarising the epidemiology of pulmonary hypertension (PH) among adults residing in Africa. Establishment of accurate epidemiological data on PH in this region may guide decision-making toward interventions to curb the burden of PH in Africa. The aim of this systematic review is to determine the prevalence, incidence and aetiologies of PH among people residing in Africa.Methods and analysisThis systematic review and meta-analysis will follow the MOOSE guidelines for reporting. Relevant abstracts published until 30 September 2016 will be searched in PubMed/Medline, EMBASE (Excerpta Medica Database), African Journals Online and Africa Index Medicus. Full texts of eligible studies will then be accessed through PubMed, Google Scholar, HINARI and the respective journals' websites. Relevant unpublished papers and conference proceedings will also be checked. Data will be analysed using STATA version 13 software. The study-specific estimates will be pooled through a random-effects meta-analysis model to obtain an overall summary estimate of the prevalence/incidence and aetiologies of PH across studies. Heterogeneity of studies will be evaluated by the ?2 test on Cochrane's Q statistic. Funnel plot analysis and Egger's test will be done to detect publication bias. Results will be presented by geographical region (central, eastern, northern, southern and western Africa).Ethics and disseminationThe current study is based on published data; ethical approval is, therefore, not required. This review will guide policy, practice and research by providing information on the magnitude of PH among people residing in Africa. Findings will be presented in evidence tables of individual studies as well as in summary tables. The final report of this systematic review, in the form of a scientific paper, will be published in a peer-reviewed journal. Furthermore, findings will be presented at conferences and submitted to relevant health authorities.Review registration numberPROSPERO CRD42016049351.
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Project description:Sarcoidosis-associated pulmonary hypertension (SAPH) is estimated to occur in at least 5% or more of sarcoidosis patients, and it contributes to significant morbidity and mortality. Optimal therapy for SAPH is not well established. We performed a 24-week open-label trial of tadalafil for SAPH at 2 academic medical centers. Subjects were required to have confirmed sarcoidosis plus a right heart catheterization within 12 months of enrollment showing a mean pulmonary artery pressure ≥ 25 mmHg, a pulmonary artery wedge pressure ≤ 15 mmHg, and a calculated pulmonary vascular resistance ≥ 3 Wood units. Subjects received 20 mg/day of tadalafil for the first 4 weeks and then 40 mg/day for the subsequent 20 weeks. Sixteen patients were screened, 12 of whom met criteria for enrollment. At 24 weeks, there was no overall improvement in 6-minute walk distance (6MWD). Five of the 12 subjects dropped out of the study early (2 for social reasons, 3 for medical reasons). There was no significant change in short form 36, St. George's respiratory questionnaire, or maximum Borg dyspnea scores over the 24 weeks. There were no significant adverse events or laboratory abnormalities clearly related to tadalafil in the cohort. The study did not meet the primary end point of change in 6MWD because of the small sample size. Tadalafil was generally safely administered in this cohort of SAPH patients. There was a relatively high dropout rate but no major adverse events and no clinical worsening. Larger studies are needed to explore this question further. (Trial registration: ClinicalTrials.gov identifier: NCT01324999).
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Project description:ImportanceReliable estimates of the prevalence of childhood hypertension serve as the basis for adequate prevention and treatment. However, the prevalence of childhood hypertension has rarely been synthesized at the global level.ObjectiveTo conduct a systematic review and meta-analysis to assess the prevalence of hypertension in the general pediatric population.Data sourcesPubMed, MEDLINE, Embase, Global Health, and Global Health Library were searched from inception until June 2018, using search terms related to hypertension (hypertension OR high blood pressure OR elevated blood pressure), children (children OR adolescents), and prevalence (prevalence OR epidemiology).Study selectionStudies that were conducted in the general pediatric population and quantified the prevalence of childhood hypertension were eligible. Included studies had blood pressure measurements from at least 3 separate occasions.Data extraction and synthesisTwo authors independently extracted data. Random-effects meta-analysis was used to derive the pooled prevalence. Variations in the prevalence estimates in different subgroups, including age group, sex, setting, device, investigation period, BMI group, World Health Organization region and World Bank region, were examined by subgroup meta-analysis. Meta-regression was used to establish the age-specific prevalence of childhood hypertension and to assess its secular trend.Main outcomes and measuresPrevalence of childhood hypertension overall and by subgroup.ResultsA total of 47 articles were included in the meta-analysis. The pooled prevalence was 4.00% (95% CI, 3.29%-4.78%) for hypertension, 9.67% (95% CI, 7.26%-12.38%) for prehypertension, 4.00% (95% CI, 2.10%-6.48%) for stage 1 hypertension, and 0.95% (95% CI, 0.48%-1.57%) for stage 2 hypertension in children 19 years and younger. In subgroup meta-analyses, the prevalence of childhood hypertension was higher when measured by aneroid sphygmomanometer (7.23% vs 4.59% by mercury sphygmomanometer vs 2.94% by oscillometric sphygmomanometer) and among overweight and obese children (15.27% and 4.99% vs 1.90% among normal-weight children). A trend of increasing prevalence of childhood hypertension was observed during the past 2 decades, with a relative increasing rate of 75% to 79% from 2000 to 2015. In 2015, the prevalence of hypertension ranged from 4.32% (95% CI, 2.79%-6.63%) among children aged 6 years to 3.28% (95% CI, 2.25%-4.77%) among those aged 19 years and peaked at 7.89% (95% CI, 5.75%-10.75%) among those aged 14 years.Conclusions and relevanceThis study provides a global estimation of childhood hypertension prevalence based on blood pressure measurements in at least 3 separate visits. More high-quality epidemiologic investigations on childhood hypertension are still needed.
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Project description:Sarcoidosis has been a well-recognised risk factor for pulmonary hypertension (PH) for a long time, but still, the knowledge about this concatenation is incomplete. Sarcoidosis-associated PH (SAPH) is an uncommon but serious complication associated with increased morbidity and mortality among sarcoidosis patients. The real epidemiology of SAPH remains unknown, and its pathomechanisms are not fully explained. Sarcoidosis is a heterogeneous and dynamic condition, and SAPH pathogenesis is believed to be multifactorial. The main roles in SAPH development play: parenchymal lung disease with the destruction of pulmonary vessels, the extrinsic compression of pulmonary vessels by conglomerate masses, lymphadenopathy or fibrosing mediastinitis, pulmonary vasculopathy, LV dysfunction, and portal hypertension. Recently, it has been recommended to individually tailor SAPH management according to the predominant pathomechanism, i.e., SAPH phenotype. Unfortunately, SAPH phenotyping is not a straightforward process. First, there are gaps in our understanding of undergoing processes. Second, the assessment of such a pivotal element as pulmonary vasculature on a microscopic level is non-feasible in SAPH patients antemortem. Finally, SAPH is a dynamic condition, multiple phenotypes usually coexist, and patients can switch between phenotypes during the course of sarcoidosis. In this article, we summarise the basic knowledge of SAPH, describe SAPH phenotypes, and highlight some practical problems related to SAPH phenotyping.
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