Churg Andrew, Müller Nestor L, Silva C Isabela S, Wright Joanne L
Department of Pathology, University of British Columbia, Vancouver, BC, Canada.
Am J Surg Pathol. 2007 Feb;31(2):277-84. doi: 10.1097/01.pas.0000213341.70852.9d.
Acute exacerbation of usual interstitial pneumonia (UIP) is a condition in which patients with UIP, and occasionally other forms of fibrotic interstitial lung disease, develop rapid respiratory failure, accompanied by extensive radiologic infiltrates. The pathologic features of this condition are ill-defined in the literature and the outcome is unclear. We report 12 such patients, 9 with underlying UIP, 2 with underlying fibrotic nonspecific interstitial pneumonia, and 1 with underlying chronic hypersensitivity pneumonitis, who underwent surgical lung biopsy for diagnosis. High-resolution computed tomography data were available in 11 cases and showed the presence of extensive bilateral ground-glass opacities, sometimes accompanied by focal consolidation, superimposed on underlying fibrosis. Three microscopic patterns of acute lung injury were seen: diffuse alveolar damage (DAD), organizing pneumonia (OP), and a pattern of numerous very large fibroblast foci superimposed on underlying fibrosis. After the biopsy, all patients were treated with steroids, in some instances accompanied by cyclophosphamide or azathioprine. Ten patients survived the acute episode and were discharged with survival times of 1 to 11 months; of these cases, 6 showed a pattern of OP or OP plus extensive fibroblast foci; 2 a pattern of extensive fibroblast foci only; and 2 a pattern of DAD. Both patients who died had histologic DAD. We conclude that acute exacerbation of UIP and other fibrotic lung diseases produces a variety of pathologic patterns on biopsy, and that patients with OP or extensive fibroblast foci as the acute pattern seem to do better than those with DAD. Our data also imply that survival (of the acute episode) may be better than the literature suggests.
普通型间质性肺炎（UIP）急性加重是一种UIP患者（偶尔也包括其他形式的纤维化间质性肺疾病患者）出现快速呼吸衰竭并伴有广泛影像学浸润的病症。该病症的病理特征在文献中定义不明确，预后也不清楚。我们报告了12例此类患者，其中9例有潜在的UIP，2例有潜在的纤维化非特异性间质性肺炎，1例有潜在的慢性过敏性肺炎，他们均接受了外科肺活检以明确诊断。11例患者有高分辨率计算机断层扫描数据，显示存在广泛的双侧磨玻璃影，有时伴有局灶性实变，叠加在潜在的纤维化基础上。观察到三种急性肺损伤的微观模式：弥漫性肺泡损伤（DAD）、机化性肺炎（OP）以及大量非常大的成纤维细胞灶叠加在潜在纤维化基础上的模式。活检后，所有患者均接受了类固醇治疗，部分患者还联合使用了环磷酰胺或硫唑嘌呤。10例患者在急性发作期存活并出院，生存时间为1至11个月；在这些病例中，6例表现为OP或OP加广泛成纤维细胞灶的模式；2例仅表现为广泛成纤维细胞灶的模式；2例表现为DAD模式。死亡的2例患者组织学表现均为DAD。我们得出结论，UIP和其他纤维化肺疾病的急性加重在活检时会产生多种病理模式，以OP或广泛成纤维细胞灶为急性模式的患者似乎比以DAD为模式的患者预后更好。我们的数据还表明（急性发作期的）生存率可能比文献报道的要好。
