Fink Ludger, Jonigk Danny
Institut für Pathologie, Zytologie und Molekularpathologie, ÜGP Wetzlar, Forsthausstraße 1, 35578, Wetzlar, Deutschland.
Institut für Pathologie, Medizinische Hochschule Hannover, Hannover, Deutschland.
Pathologe. 2021 Feb;42(1):40-47. doi: 10.1007/s00292-020-00894-6. Epub 2021 Jan 8.
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic progressive fibrosing nonreversible interstitial lung disease of largely unknown origin. In high-resolution computer tomography (HRCT) and histopathology it presents with a UIP pattern. To diagnose IPF, (i) an ILD of known origin must be excluded (e.g., hypersensitivity pneumonitis, lung involvement in autoimmune or other systemic disease, and drug-induced ILD) and either (ii) the presence of a UIP pattern in HRCT or (iii) specific combinations of HRCT and histopathology is necessary. The diagnosis of IPF requires interdisciplinary collaboration and a structured procedure. The updated S2k guideline focuses on the IPF diagnostic process and describes the criteria of a UIP pattern in HRCT and histopathology that are differentiated into the categories "UIP pattern," "probable UIP pattern," "indetermined for UIP," and "alternative pattern." Depending on the anamnestic, clinical and serologic findings, HRCT, and - if acquired - histomorphology features, an algorithm to diagnose the IPF is recommended. If a UIP pattern in HRCT is present, IPF can still be diagnosed without further bioptic examination. Additionally, recommendations for the use of surgical lung biopsy (SLB), transbronchial lung biopsy, and the relatively new transbronchial lung cryobiopsy (TBLC) procedure are provided. In contrast to the international guideline, the S2k guideline group evaluated TBLC based on recent studies to be advantageous compared to the SLB, as the diagnostic value and the side-effect rate was assessed to be acceptable and more patients with progressed ILD can be biopsied by TBLC. It is therefore expected that by using TBLC the rate of unclassifiable ILDs can be reduced.
特发性肺纤维化（IPF）是一种慢性进行性纤维化且不可逆转的间质性肺病的特殊形式，其病因大多不明。在高分辨率计算机断层扫描（HRCT）和组织病理学检查中，它呈现出一种寻常型间质性肺炎（UIP）模式。要诊断IPF，（i）必须排除已知病因的间质性肺病（如过敏性肺炎、自身免疫性或其他全身性疾病累及肺部以及药物性间质性肺病），并且（ii）HRCT中存在UIP模式或（iii）HRCT与组织病理学的特定组合是必要的。IPF的诊断需要多学科协作和结构化程序。更新后的S2k指南聚焦于IPF的诊断过程，并描述了HRCT和组织病理学中UIP模式的标准，这些标准被分为“UIP模式”“可能的UIP模式”“UIP不确定”和“替代模式”几类。根据既往史、临床和血清学检查结果、HRCT以及（如有）组织形态学特征，推荐了一种诊断IPF的算法。如果HRCT中存在UIP模式，即使不进行进一步的活检检查也可诊断IPF。此外，还提供了关于使用外科肺活检（SLB）、经支气管肺活检以及相对较新的经支气管肺冷冻活检（TBLC）程序的建议。与国际指南不同，S2k指南组根据近期研究评估认为，与SLB相比，TBLC具有优势，因为其诊断价值和副作用发生率被认为是可接受的，并且更多病情进展的间质性肺病患者可以通过TBLC进行活检。因此，预计通过使用TBLC可以降低无法分类的间质性肺病的发生率。
