Practical application and validation of the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines for the diagnosis of idiopathic pulmonary fibrosis.
Practical application and validation of the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines for the diagnosis of idiopathic pulmonary fibrosis.
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DOI：
10.1186/s12931-021-01670-7
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发表时间：
2021-04-26
影响因子：
5.8
通讯作者：
Hariri LP
中科院分区：
文献类型：
--
作者：
Shih AR;Nitiwarangkul C;Little BP;Roop BW;Nandy S;Szabari MV;Mercaldo N;Mercaldo S;Montesi SB;Muniappan A;Berigei SR;Lynch DA;Sharma A;Hariri LP
特发性肺纤维化（IPF）的准确诊断对于告知预后和治疗至关重要。2018年，ATS/ERS/JRS/ALAT和Fleischner Society发布了普通型间质性肺炎（UIP）/IPF的新诊断指南，基于既往研究证明该类别对组织病理学UIP/可能的UIP具有相对较高的阳性预测值（PPV），将可能的UIP添加为CT类别。本研究采用2018年ATS/ERS/JRS/ALAT和Fleischner Society指南，以确定学术临床实践中CT类别的测试特征。由三名胸部放射科医师和两名胸部病理科医师对CT和组织病理学进行评估。通过CT和组织病理学比较了2018年ATS和Fleischner Society指南的共识分类。在CT UIP患者中，87%（PPV，95% CI：60-98%）的组织病理学UIP具有97%（CI：90-100%）的特异性。在CT可能的UIP患者中，38%（PPV，CI：14-68%）有组织病理学UIP，46%（PPV，CI：19-75%）有组织病理学UIP或可能的UIP，特异性为88%（CI：77-95%）。CT不确定和替代诊断的患者在27%（PPV，CI：6-61%）和21%（PPV，CI：11-33%）的病例中具有组织病理学UIP，特异性为90%（CI：80-96%）和25%（CI：16-37%）。放射科医师之间的观察者间变异性（kappa）范围为0.32-0.81。CT UIP和Probable UIP对组织病理学UIP具有较高的特异性，CT UIP对组织病理学UIP具有较高的PPV。对于合并的组织病理学UIP/很可能UIP，CT很可能UIP的PPV为46%。我们的研究结果表明，需要进一步的研究，以进一步评估和完善的指导标准，以提高分类性能。在线版本包含补充材料，可通过10.1186/s12931-021-01670-7获得。
Accurate diagnosis of idiopathic pulmonary fibrosis (IPF) is essential to inform prognosis and treatment. In 2018, the ATS/ERS/JRS/ALAT and Fleischner Society released new diagnostic guidelines for usual interstitial pneumonitis (UIP)/IPF, adding Probable UIP as a CT category based on prior studies demonstrating this category had relatively high positive predictive value (PPV) for histopathologic UIP/Probable UIP. This study applies the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines to determine test characteristics of CT categories in academic clinical practice. CT and histopathology were evaluated by three thoracic radiologists and two thoracic pathologists. Comparison of consensus categorization by the 2018 ATS and Fleischner Society guidelines by CT and histopathology was performed. Of patients with CT UIP, 87% (PPV, 95% CI: 60–98%) had histopathologic UIP with 97% (CI: 90–100%) specificity. Of patients with CT Probable UIP, 38% (PPV, CI: 14–68%) had histopathologic UIP and 46% (PPV, CI: 19–75%) had either histopathologic UIP or Probable UIP, with 88% (CI: 77–95%) specificity. Patients with CT Indeterminate and Alternative Diagnosis had histopathologic UIP in 27% (PPV, CI: 6–61%) and 21% (PPV, CI: 11–33%) of cases with specificities of 90% (CI: 80–96%) and 25% (CI: 16–37%). Interobserver variability (kappa) between radiologists ranged 0.32–0.81. CT UIP and Probable UIP have high specificity for histopathologic UIP, and CT UIP has high PPV for histopathologic UIP. PPV of CT Probable UIP was 46% for combined histopathologic UIP/Probable UIP. Our results indicate that additional studies are needed to further assess and refine the guideline criteria to improve classification performance. The online version contains supplementary material available at 10.1186/s12931-021-01670-7.
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