From the Department of Pathology, Vancouver General Hospital, Vancouver, British Columbia, Canada.
Arch Pathol Lab Med. 2020 Dec 1;144(12):1509-1516. doi: 10.5858/arpa.2019-0628-RA.
CONTEXT.—: Various pulmonary diseases can produce centrilobular (peribronchiolar) fibrosis, which may be isolated or associated with other patterns of more diffuse fibrosis. The major forms of interstitial lung disease in which centrilobular fibrosis is found are fibrotic (chronic) hypersensitivity pneumonitis, connective tissue disease-associated interstitial lung disease, and (a disputed issue) usual interstitial pneumonia/idiopathic interstitial fibrosis.
OBJECTIVE.—: To review recent literature that addresses separation of these entities.
DATA SOURCES.—: Data comprised recent publications.
CONCLUSIONS.—: In a specially constructed multidisciplinary discussion exercise, it was found that peribronchiolar metaplasia affecting more than half the bronchioles or more than 2 foci of peribronchiolar metaplasia per square centimeter of biopsy area was strongly associated with a confident diagnosis of fibrotic hypersensitivity pneumonitis. Giant cells or granulomas were only found in cases with a greater than 50% diagnostic confidence in hypersensitivity pneumonitis. Conversely, greater numbers of fibroblast foci per square centimeter and increasing measured amounts of subpleural fibrosis favored a diagnosis of usual interstitial pneumonia. Recent data also suggest that centrilobular fibrosis can be found in usual interstitial pneumonia, although the presence of centrilobular fibrosis statistically favors an alternate diagnosis. Connective tissue disease is a major confounder because many patterns are very similar to fibrotic hypersensitivity pneumonitis or usual interstitial pneumonia. Genetic abnormalities, such as the MUC5B minor allele overlap, in these conditions and at this point cannot be used for discrimination. Thus, the separation of fibrotic hypersensitivity pneumonitis and usual interstitial pneumonia remains a difficult problem. Accurate biopsy diagnosis of all of these diseases requires correlation with imaging and clinical findings, and is crucial for treatment.
各种肺部疾病均可导致中央性（细支气管周围）纤维化，这种纤维化可以是孤立性的，也可以与其他弥漫性纤维化模式同时存在。在伴有中央性纤维化的各种间质性肺疾病中，主要包括纤维化（慢性）过敏性肺炎、结缔组织病相关间质性肺病，以及（存在争议）普通型间质性肺炎/特发性间质性纤维化。
综述近期关于这些实体疾病鉴别的文献。
数据来源于近期出版物。
在一项特别构建的多学科讨论实践中发现，超过半数的细支气管或每平方毫米活检面积超过 2 个细支气管周围化生灶的支气管周围化生与纤维化过敏性肺炎的明确诊断具有强烈相关性。只有在过敏性肺炎的诊断置信度大于 50%的情况下才能发现多核巨细胞或肉芽肿。相反，每平方毫米的成纤维细胞灶数量越多，胸膜下纤维化程度越高，越支持普通型间质性肺炎的诊断。最近的数据还表明，中央性纤维化可存在于普通型间质性肺炎中，尽管中央性纤维化的存在从统计学上支持其他诊断。结缔组织病是一个主要的混杂因素，因为许多模式与纤维化过敏性肺炎或普通型间质性肺炎非常相似。这些疾病中存在的遗传异常，如 MUC5B 次要等位基因重叠，目前无法用于鉴别。因此，纤维化过敏性肺炎和普通型间质性肺炎的鉴别仍然是一个难题。所有这些疾病的准确活检诊断都需要与影像学和临床发现相关联，这对于治疗至关重要。
