Krishnan Sathish, Ramalingam Vijaya, Adigopula Sashi, Gadeela Nitesh
Pulmonary and Critical Care Medicine, Community Health Network, Indianapolis, USA.
Pulmonary and Critical Care Medicine, Northeast Georgia Medical Center Gainesville, Gainesville, USA.
Cureus. 2025 Jul 31;17(7):e89111. doi: 10.7759/cureus.89111. eCollection 2025 Jul.
Pulmonary hypertension (PH) significantly affects prognosis in patients with pulmonary Langerhans cell histiocytosis (PLCH); however, reported prevalence varies substantially across studies. This systematic review and meta-analysis aimed to synthesize existing data to estimate the prevalence of PH in PLCH patients. A comprehensive literature search was performed in PubMed, Scopus, Web of Science, Cochrane Library, and Embase from inception to 2022, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and Meta-Analysis of Observational Studies in Epidemiology (MOOSE) guidelines. Observational studies reporting PH prevalence in PLCH were included. Data extraction and quality assessment were independently conducted by two reviewers. Pooled prevalence was calculated using a random-effects model with logit transformation, and subgroup analyses explored heterogeneity by diagnostic methods, study designs, and geographical region. Ten observational studies (one prospective cohort, seven retrospective cohorts, two cross-sectional studies; total participants = 14,302) met the inclusion criteria. The overall pooled prevalence of PH in PLCH was 26.3% (95% confidence interval (CI): 17.3%-33.3%; I = 100%). Subgroup analysis revealed a higher prevalence in studies using echocardiography (36.3%) compared with right heart catheterization (RHC) (25.2%), and a higher prevalence observed in cross-sectional studies (40.5%) compared to prospective (5.0%) and retrospective studies (38.0%). Geographic differences between Europe and America were not statistically significant. Sensitivity analysis confirmed the robustness of the pooled estimate, and funnel plots indicated mild publication bias. This meta-analysis demonstrates a high prevalence of PH among PLCH patients. Diagnostic modality and study design contribute to substantial prevalence variability. Early screening using RHC is recommended for accurate PH diagnosis and improved clinical outcomes.
肺动脉高压（PH）显著影响肺朗格汉斯细胞组织细胞增多症（PLCH）患者的预后；然而，各研究报告的患病率差异很大。本系统评价和荟萃分析旨在综合现有数据，以估计PLCH患者中PH的患病率。按照系统评价和荟萃分析的首选报告项目（PRISMA）以及流行病学观察性研究的荟萃分析（MOOSE）指南，从创刊至2022年在PubMed、Scopus、Web of Science、Cochrane图书馆和Embase中进行了全面的文献检索。纳入报告PLCH中PH患病率的观察性研究。由两名审阅者独立进行数据提取和质量评估。使用对数转换的随机效应模型计算合并患病率，并通过诊断方法、研究设计和地理区域进行亚组分析以探讨异质性。十项观察性研究（一项前瞻性队列研究、七项回顾性队列研究、两项横断面研究；总参与者 = 14302）符合纳入标准。PLCH患者中PH的总体合并患病率为26.3%（95%置信区间（CI）：17.3% - 33.3%；I² = 100%）。亚组分析显示，与右心导管检查（RHC）（25.2%）相比，使用超声心动图的研究中患病率更高（36.3%），与前瞻性研究（5.0%）和回顾性研究（38.0%）相比，横断面研究中的患病率更高（40.5%）。欧洲和美洲之间的地理差异无统计学意义。敏感性分析证实了合并估计值的稳健性，漏斗图表明存在轻度发表偏倚。这项荟萃分析表明PLCH患者中PH的患病率很高。诊断方式和研究设计导致患病率存在很大差异。建议采用RHC进行早期筛查以准确诊断PH并改善临床结局。
