Benan Musellim, Nesrin Mogulkoc, Oguz Uzun, Fatma Tokgoz Akyil, Haluk Turktas, Ozlem Ozdemir Kumbasar, Gulfer Okumus, Candan Ogus, Hulya Dirol, Adil Zamani, Tulin Sevim, Nihat Annakkaya Ali, Akinci Ozyurek Berna, Ismail Hanta, Yusuf Aydemir, Ebru Cakir Edis, Bahar Kurt, Can Tertemiz Kemal, Levent Tabak, Onur Yazici, Yurdanur Erdogan, Gungor Ates, Hatice Turker, Banu Salepci, Armagan Hazar, Yelda Niksarlioglu Elif, Bilge Yılmaz Kara, Nurdan Kokturk, Fusun Kalpaklioglu, Isil Uzel, Savas Ozsu, Ersan Atahan, Zeynep Fendoglu Turkan, Sureyya Yilmaz, Ilknur Basyigit, Gungor Camsari, Esin Tuncay, Elif Ucar Yilmazel, Dilek Kanmaz, Aydanur Ekici, Fusun Topcu, Esra Uzaslan, Fulsen Bozkus, Serap Argun Baris, Serap Duru, Goksel Altinisik, Zuleyha Bingol, Atadan Tunaci, Recep Savas, Fatih Alper, Selen Bayraktaroglu, Tuba Selcuk Can, Arslan Demir Ali
Department of Chest Diseases, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Department of Chest Diseases, Ege University Medical Faculty, Izmir, Turkey.
Turk Thorac J. 2021 Mar;22(2):102-109. doi: 10.5152/TurkThoracJ.2021.20028. Epub 2021 Mar 1.
Differential diagnosis of idiopathic pulmonary fibrosis (IPF) is important among fibrotic interstitial lung diseases (ILD). This study aimed to evaluate the rate of IPF in patients with fibrotic ILD and to determine the clinical-laboratory features of patients with and without IPF that would provide the differential diagnosis of IPF.
The study included the patients with the usual interstitial pneumonia (UIP) pattern or possible UIP pattern on thorax high-resolution computed tomography, and/or UIP pattern, probable UIP or possible UIP pattern at lung biopsy according to the 2011 ATS/ERS/JRS/ALAT guidelines. Demographics and clinical and radiological data of the patients were recorded. All data recorded by researchers was evaluated by radiology and the clinical decision board.
A total of 336 patients (253 men, 83 women, age 65.8±9.0 years) were evaluated. Of the patients with sufficient data for diagnosis (n=300), the diagnosis was IPF in 121 (40.3%), unclassified idiopathic interstitial pneumonia in 50 (16.7%), combined pulmonary fibrosis and emphysema (CPFE) in 40 (13.3%), and lung involvement of connective tissue disease (CTD) in 16 (5.3%). When 29 patients with definite IPF features were added to the patients with CPFE, the total number of IPF patients reached 150 (50%). Rate of male sex (p<0.001), smoking history (p<0.001), and the presence of clubbing (p=0.001) were significantly high in patients with IPF. None of the women <50 years and none of the men <50 years of age without a smoking history were diagnosed with IPF. Presence of at least 1 of the symptoms suggestive of CTD, erythrocyte sedimentation rate (ESR), and antinuclear antibody (FANA) positivity rates were significantly higher in the non-IPF group (p<0.001, p=0.029, p=0.009, respectively).
The rate of IPF among patients with fibrotic ILD was 50%. In the differential diagnosis of IPF, sex, smoking habits, and the presence of clubbing are important. The presence of symptoms related to CTD, ESR elevation, and FANA positivity reduce the likelihood of IPF.
在纤维化间质性肺疾病（ILD）中，特发性肺纤维化（IPF）的鉴别诊断至关重要。本研究旨在评估纤维化ILD患者中IPF的发生率，并确定有或无IPF患者的临床实验室特征，以提供IPF的鉴别诊断依据。
本研究纳入了根据2011年美国胸科学会（ATS）/欧洲呼吸学会（ERS）/日本呼吸学会（JRS）/拉丁美洲胸科协会（ALAT）指南，胸部高分辨率计算机断层扫描显示为普通型间质性肺炎（UIP）模式或可能的UIP模式，和/或肺活检显示为UIP模式、很可能的UIP或可能的UIP模式的患者。记录患者的人口统计学资料以及临床和放射学数据。研究人员记录的所有数据均由放射科和临床决策委员会进行评估。
共评估了336例患者（男性253例，女性83例，年龄65.8±9.0岁）。在有足够诊断数据的患者（n = 300）中，诊断为IPF的有121例（40.3%），未分类的特发性间质性肺炎50例（16.7%），合并肺纤维化和肺气肿（CPFE）40例（13.3%），结缔组织病（CTD）累及肺16例（5.3%）。当将29例具有明确IPF特征的患者加入CPFE患者中时，IPF患者总数达到150例（50%）。IPF患者中男性比例（p<0.001）、吸烟史（p<0.001）和杵状指的发生率（p = 0.001）显著更高。年龄<50岁且无吸烟史的女性和男性均未被诊断为IPF。非IPF组中提示CTD的症状至少出现1种、红细胞沉降率（ESR）和抗核抗体（FANA）阳性率显著更高（分别为p<0.001、p = 0.029、p = 0.009）。
纤维化ILD患者中IPF的发生率为50%。在IPF的鉴别诊断中，性别、吸烟习惯和杵状指的存在很重要。与CTD相关的症状、ESR升高和FANA阳性会降低IPF的可能性。
