Department of Pulmonary Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Department of Pathology, University of California, San Francisco, San Francisco, CA.
Chest. 2017 Sep;152(3):502-509. doi: 10.1016/j.chest.2017.02.011. Epub 2017 Feb 20.
The ability of specific histopathologic features to predict mortality or lung transplantation in patients with chronic hypersensitivity pneumonitis (HP) is unknown.
Patients with chronic HP diagnosed by surgical lung biopsy were identified from an ongoing longitudinal cohort. The surgical lung biopsy slides were evaluated prospectively by an experienced thoracic pathologist using a standardized checklist to differentiate the major pathologic patterns and score the presence of specific histopathologic features. Cox proportional hazard analysis was used to identify independent predictors of transplant-free survival, and Kaplan-Meier analysis was used to visualize outcomes.
One hundred nineteen patients were identified. Patients with a fibrotic nonspecific interstitial pneumonia (f-NSIP) pattern, bronchiolocentric fibrosis (BF) pattern, or usual interstitial pneumonia (UIP) pattern had significantly worse transplant-free survival than did those with a cellular NSIP (c-NSIP) pattern or peribronchiolar inflammation with poorly formed granulomas (PI-PFG) pattern. No survival difference among patients with an f-NSIP pattern, a BF pattern, or a UIP pattern was found. Fibroblastic foci were identified in a subset of biopsy samples from all pathologic patterns. Peribronchiolar fibrosis was noted in all UIP cases. Independent predictors of time to death or transplantation included the presence of fibroblast foci or dense collagen fibrosis.
Histopathologic patterns of c-NSIP and PI-PFG had a better transplant-free survival than did patterns of UIP, f-NSIP, and BF. The presence of fibroblast foci or dense collagen fibrosis correlated with progression to death or lung transplantation. Identification of fibroblast foci on biopsy samples, regardless of the underlying histopathologic pattern, may be a clinically useful predictor of survival in patients with HP.
特定组织病理学特征能否预测慢性超敏性肺炎（HP）患者的死亡率或肺移植尚不清楚。
从正在进行的纵向队列中确定了通过手术肺活检诊断为慢性 HP 的患者。一位经验丰富的胸病理学家使用标准化清单前瞻性评估手术肺活检切片，以区分主要病理模式并评分特定组织病理学特征的存在。Cox 比例风险分析用于确定无移植生存率的独立预测因子，Kaplan-Meier 分析用于可视化结果。
确定了 119 名患者。与具有细胞性非特异性间质性肺炎（c-NSIP）模式、细支气管中心纤维化（BF）模式或寻常间质性肺炎（UIP）模式的患者相比，具有纤维性非特异性间质性肺炎（f-NSIP）模式、支气管中心纤维化（BF）模式或寻常间质性肺炎（UIP）模式的患者无移植生存率明显更差。在 f-NSIP 模式、BF 模式或 UIP 模式的患者中未发现生存差异。在所有病理模式的活检样本中都发现了纤维母细胞灶。所有 UIP 病例均有细支气管周围纤维化。死亡或移植时间的独立预测因子包括成纤维细胞灶或致密胶原纤维化的存在。
c-NSIP 和 PI-PFG 的组织病理学模式比 UIP、f-NSIP 和 BF 的模式具有更好的无移植生存率。成纤维细胞灶或致密胶原纤维化的存在与死亡或肺移植进展相关。无论潜在的组织病理学模式如何，在活检样本中识别出成纤维细胞灶可能是预测 HP 患者生存的一种有用的临床预测因子。
