Idiopathic pulmonary fibrosis - Relationship between histopathologic features and mortality
Idiopathic pulmonary fibrosis - Relationship between histopathologic features and mortality
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DOI：
10.1164/ajrccm.164.6.2001056
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发表时间：
2001-09-15
影响因子：
24.7
通讯作者：
Cherniack, RM
中科院分区：
文献类型：
--
作者：
King, TE;Schwarz, MI;Cherniack, RM
据推测，肺活检中发现的纤维化和细胞结构的程度和严重程度决定了特发性肺纤维化（IPF）的预后和治疗反应。本研究的目的是确定哪些组织病理学特征可预测 IPF 的生存率。我们前瞻性研究了 87 名经手术肺活检确诊为普通型间质性肺炎 (UIP) 的患者。四位病理学家独立对特定组织病理学特征的范围和严重程度进行分级。我们使用 Cox 比例风险模型来评估组织病理学模式对患者生存的影响。年龄、性别和吸烟的影响也包括在分析中。在 17 年的研究期间，有 63 名患者死亡。肉芽/结缔组织沉积（成纤维细胞灶）程度较低的受试者的生存期较长。肺泡间隙细胞结构、肺泡壁纤维化和细胞结构的程度不影响生存。吸烟史、呼吸困难程度和就诊时肺部僵硬程度也被证明是预测生存的独立因素。肺活检中存在的成纤维细胞灶的范围可预测 IPF 的生存率。这些发现支持这样的假设：终末期纤维化的关键途径不是“肺泡炎”，而是与持续成纤维细胞增殖相关的持续上皮损伤和修复过程。控制这些过程，而不是阻止炎症，对于预防进行性疾病和 IPF 中常见的致命结果似乎最重要。
It is hypothesized that the extent and severity of fibrosis and cellularity found on lung biopsy determine the prognosis and response to therapy in idiopathic pulmonary fibrosis (IPF). The objective of this study was to determine which histopathologic features predict survival in IPF. We prospectively studied 87 patients with usual interstitial pneumonia (UIP) confirmed by surgical lung biopsy. Four pathologists independently graded the extent and severity of specific histopathologic features. We used Cox proportional-hazards models to assess the effect of histopathologic patterns on patients' survival, The effects of age, sex, and smoking were also included in the analysis. Sixty-three patients died during the 17-yr study period. Survival was longer in subjects with lesser degrees of granulation/connective tissue deposition (fibroblastic foci). The degree of alveolar space cellularity, alveolar wall fibrosis, and cellularity did not affect survival. A history of cigarette smoking, the level of dyspnea, and the degree of lung stiffness at presentation were also shown to be independent factors predicting survival. The extent of fibroblastic foci present on lung biopsy predicts survival in IPF. These findings support the hypothesis that the critical pathway to end-stage fibrosis is not "alveolitis" but rather the ongoing epithelial damage and repair process associated with persistent fibroblastic proliferation. Controlling these processes, rather than stopping inflammation, appears most important in preventing progressive disease and the fatal outcome common in IPF.