Povýsil Ctibor
Ustav patologie 1. LF UK a VFN a Katedra patologické anatomie IPVZ, Praha.
Cesk Patol. 2010 Jan;46(1):3-7.
The classification scheme of interstitial lung diseases has undergone numerous revisions. The criteria for distinguishing seven distinct subtypes of idiopathic interstitial pneumonias are now well defined by consensus in the recently published ATS/ERS classification of these lung diseases. In our present review the histological patterns of the different types are described and the differential diagnosis of idiopathic interstitial pneumonias is discussed. Surgical lung biopsy remains the gold standard for the diagnosis of interstitial pneumonias, and sampling from at least 2 sites is recommended. Video-assisted thoracoscopic surgical biopsy is the preferred method for obtaining lung tissue as this procedure offers a similar yield as an open thoracotomy The most common histological subtype of chronic interstitial lung disease is the usual interstitial pneumonia [UIP] which makes up 47-71% of cases. The key histologic features include patchy subpleural and paraseptal distribution of remodeling lung architecture with dense fibrosis, frequent honeycombing, and large fibroblastic foci. Temporal and spatial heterogeneity are the hallmarks. Nonspecific interstitial pneumonia [NSIP] occurs primarily in middle-aged women who have never smoked, with more than 5-years survival rate in 80% of patients. The major feature of NSIP is a uniform interstitial thickening of alveolar septa by a fibrosing or cellular process. The cardinal histological feature in respiratory bronchiolitis and desquamative pneumonia is an excess of intraalveolar histiocytes. In both patterns, there is variable interstitial fibrosis and chronic inflammation, and a strong association with a history of smoking. Organizing pneumonia (idiopathic bronchiolitis obliterans-organizing pneumonia [BOOP]) is not strictly an interstitial process, because the alveoli and bronchioles are filled by intraluminal polyps of fibroblastic tissue and the expansion of the interstitium is mild. Lymphocytic interstitial pneumonia [LIP] is currently viewed as a pattern of diffuse reactive pulmonary hyperplasia associated in most cases with EB virus, immunosuppression, or a connective tissue disorder. Malignant transformation may rarely occur. A dense mixed interstitial lymphoid infiltrate is a typical histological finding. Diffuse alveolar damage [DAD] from unknown causes is termed acute interstitial pneumonia [AIP], and is synonymous with cases of Hamman-Rich disease. Hyaline membranes in the exsudative phase and marked expansion of the interstitium later are present.
间质性肺疾病的分类方案已经历了多次修订。在最近发表的美国胸科学会（ATS）/欧洲呼吸学会（ERS）关于这些肺部疾病的分类中，区分特发性间质性肺炎七种不同亚型的标准现已通过共识明确界定。在我们目前的综述中，描述了不同类型的组织学模式，并讨论了特发性间质性肺炎的鉴别诊断。外科肺活检仍然是诊断间质性肺炎的金标准，建议从至少两个部位取材。电视辅助胸腔镜手术活检是获取肺组织的首选方法，因为该手术的取材成功率与开胸手术相似。慢性间质性肺疾病最常见的组织学亚型是普通型间质性肺炎（UIP），占病例的47 - 71%。关键的组织学特征包括重塑的肺结构呈斑片状胸膜下和间隔旁分布，伴有致密纤维化、频繁的蜂窝状改变和大的成纤维细胞灶。时间和空间异质性是其特点。非特异性间质性肺炎（NSIP）主要发生在从不吸烟的中年女性中，80%的患者5年生存率较高。NSIP的主要特征是肺泡间隔通过纤维化或细胞过程均匀增厚。呼吸性细支气管炎伴间质性肺病和脱屑性间质性肺炎的主要组织学特征是肺泡内组织细胞过多。在这两种模式中，均存在不同程度的间质纤维化和慢性炎症，且与吸烟史密切相关。机化性肺炎（特发性闭塞性细支气管炎伴机化性肺炎[BOOP]）并不严格属于间质性病变，因为肺泡和细支气管腔内充满了成纤维组织息肉，间质扩张较轻。淋巴细胞性间质性肺炎（LIP）目前被视为一种弥漫性反应性肺增生模式，在大多数情况下与EB病毒、免疫抑制或结缔组织疾病相关。很少发生恶性转化。密集的混合性间质淋巴浸润是典型的组织学表现。原因不明的弥漫性肺泡损伤（DAD）被称为急性间质性肺炎（AIP），与Hamman - Rich病病例同义。渗出期可见透明膜，后期可见间质明显扩张。
