Chiodera P
Servizio di Anatomia Patologica, Casa di Cura S. Anna, Brescia, Italy.
Rays. 1997 Jan-Mar;22(1):127-56.
Interstitial lung disease encompasses a large number of clinical disorders that affect the epithelium, the endothelium or both cell surfaces of alveolar wall and satellite structures including terminal and respiratory bronchioles. Causative factors are over 200 from bacteria, fungi, viruses, protozoans, to collagen disease, hypersensitivity and inorganic pneumoconiosis. Clinical and histological findings of open lung and transbronchial biopsies from 50 patients are reported, 18 patients were affected by diffuse alveolar damage (DAD), 15 patients by usual interstitial pneumonia (UIP), 8 patients by non specific interstitial pneumonia-fibrosis (NSIP-F), 9 patients by bronchiolitis obliterans organizing pneumonia (BOOP) correlated with conventional chest radiography in 30 patients and with HRCT in 31 patients. Interstitial lung disease other than histiocytosis X share anatomoradiologic features indicative for activity, chronic progression, chronic quiescence, chronic advanced and irreversible disease. In general, the histologic features correlate with radiographic patterns and even if radiologic findings do not always supply definitive diagnosis, some HRCT patterns are highly suggestive and usually classified into 4 categories: normal; with ground glass attenuation; linear, nodular or reticulo-nodular; honeycombing, suggestive for end-stage fibrosis. Correlation of HRCT with histologic findings in 31 patients with idiopathic interstitial fibrosis (IIF) allowed assessment of disease activity, follow-up and therapeutic result. HRCT definitely better than conventional radiology detects the presence, type and extent of parenchymal alterations, differentiating potential reversible lesions (inflammatory) from potentially irreversible (fibrotic) lesions. In IIF, for diagnostic accuracy the specimen of open lung (the gold standard), transbronchial or video-thoracoscopic biopsy must be preoperative, HRCT-assisted and centered on ground glass opacties (or nodules in suspected histiocytosis X) since a diagnostically reliable biopsy correlates with HRCT morphology of histologic specimen. Interstitial lung disease includes benign as well as malignant forms, thus only a multidisciplinary approach can prevent long term hazardous effects as severe cor pulmonale or a fatal outcome. The histologic HRCT-assisted assessment of "active" lesions is crucial for correct careful treatment of these patients.
间质性肺疾病涵盖了大量临床病症，这些病症会影响肺泡壁以及包括终末细支气管和呼吸性细支气管在内的卫星结构的上皮细胞、内皮细胞或两者的细胞表面。致病因素超过200种，包括细菌、真菌、病毒、原生动物，以及胶原病、超敏反应和无机尘肺。报告了50例患者经开胸肺活检和经支气管活检的临床及组织学结果，其中18例患者患有弥漫性肺泡损伤（DAD），15例患者患有寻常型间质性肺炎（UIP），8例患者患有非特异性间质性肺炎 - 纤维化（NSIP - F），9例患者患有闭塞性细支气管炎伴机化性肺炎（BOOP），30例患者的结果与传统胸部X线摄影相关，31例患者的结果与高分辨率CT（HRCT）相关。除组织细胞增多症X外，间质性肺疾病具有提示活动、慢性进展、慢性静止、慢性晚期和不可逆疾病的解剖放射学特征。一般来说，组织学特征与放射学模式相关，即使放射学结果并不总是能提供明确诊断，但一些HRCT模式具有高度提示性，通常分为4类：正常；磨玻璃样衰减；线性、结节状或网状结节状；蜂窝状，提示终末期纤维化。对31例特发性间质性纤维化（IIF）患者的HRCT与组织学结果进行相关性分析，有助于评估疾病活动度、随访及治疗效果。HRCT在检测实质改变的存在、类型和范围方面肯定优于传统放射学，能区分潜在可逆性病变（炎症性）和潜在不可逆性病变（纤维化性）。在IIF中，为了诊断准确性，开胸肺活检标本（金标准）、经支气管活检或电视胸腔镜活检必须在术前进行，且在HRCT辅助下，以磨玻璃影（或疑似组织细胞增多症X中的结节）为中心，因为诊断可靠的活检与组织学标本的HRCT形态相关。间质性肺疾病包括良性和恶性形式，因此只有多学科方法才能预防严重肺心病或致命结局等长期有害影响。对“活动性”病变进行组织学HRCT辅助评估对于正确、谨慎地治疗这些患者至关重要。
