肺のランゲルハン細胞ヒスティオサイトーシスの患者における肺高血圧の流行: 系統的レビューとメタ解析
Sathish Krishnan1, Vijaya Ramalingam2, Sashi Adigopula3
1Pulmonary and Critical Care Medicine, Community Health Network, Indianapolis, USA.
Cureus
|September 2, 2025
まとめ
肺高血圧 (PH) は,肺のランゲルハンズ細胞ヒスティオサイトーシス (PLCH) で一般的であり,患者の4分の1以上に影響します. 正確な診断とよりよい結果のために,右心経導管 (RHC) の早期スクリーニングが推奨されます.
科学分野:
- 肺病と呼吸器医学
- 心臓病科
- 医療 統計
背景:
- 肺高血圧 (PH) は,肺のランゲルハンズ細胞ヒスティオシトーシス (PLCH) の患者の予後に影響を及ぼします.
- 既存の研究では,PLCH患者におけるPHの流行率が大きく変化していることが報告されています.
- PLCHにおけるPHの流行を正確に推定するには,現在のデータの包括的な合成が必要である.
研究 の 目的:
- PLCH患者におけるPHの流行を推定するために,既存の観察研究を体系的にレビューし,メタ分析する.
- 診断方法と研究設計を含む報告されたPHの流行に寄与する要因を調査する.
主な方法:
- 2022年までの主要なデータベース (PubMed,Scopus,Web of Science,Cochrane Library,Embase) で体系的な文献検索が行われました.
- 合計14,302人の参加者を含む10件の観察研究は,含める基準を満たした.
- ランダム効果モデルを用いて,サブグループ分析によって異質性を評価した.
主要な成果:
- PLCHにおけるPHの総合的な流行率は26. 3% (95%CI:17. 3% - 33. 3%) であった.
- 罹患率は,診断方法 (エコーカーディオグラフィー:36. 3% vs. RHC:25. 2%) と研究設計 (横断: 40. 5% vs. コホート: 5. 0% - 38. 0%) によって著しく変化した.
- ヨーロッパとアメリカの間で有意な地理的差異は観察されなかった.
結論:
- このメタ解析は,PLCH患者におけるPHの高い罹患率を示しています.
- 診断方法と研究設計は,報告されたPHの流行の変動に影響を与える重要な要因です.
- 正確なPH診断と改善された患者のアウトカムのために,右心経導管 (RHC) を使用して早期スクリーニングを推奨します.
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