September 3, 2019 Idiopathic Pulmonary Fibrosis 2020. 6. 4.¢  idiopathic pulmonary fibrosis (IPF)

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  • 688 Copyrights © 2020 The Korean Society of Radiology

    Original Article J Korean Soc Radiol 2020;81(3):688-700 pISSN 1738-2637 / eISSN 2288-2928

    Acute Exacerbation of Idiopathic Pulmonary Fibrosis with Lung Cancer: A Comparative Analysis of the Incidence, Survival Rate, and CT Findings with the Patients without Lung Cancer 폐암을 동반한 특발성 폐섬유증 환자에서 폐섬유증의 급성 악화: 폐암 비동반군과의 발병률, 생존율 및 전산화단층촬영 소견의 비교

    Bumsang Cho, MD1 , Hee Kang, MD1* , Je Hun Kim, MD2 , Jung Gu Park, MD1 , Sekyoung Park, MD1 , Jong Hyouk Yun, MD1

    Departments of 1Radiology, 2Pulmonary and Critical Care Medicine, Kosin University Gospel Hospital, Kosin University College of Medicine, Busan, Korea

    Purpose To compare the incidence, survival rate, and CT findings of acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) between patients with and without lung cancer. Materials and Methods From June 2004 to July 2018, 89 consecutive patients diagnosed with IPF were included. Among them, 26 patients had IPF with lung cancer (IPF-LCA), and 63 pa- tients had IPF alone. The clinical characteristics and CT findings associated with IPF, lung can- cer, and AE were reviewed. Surgery and chemotherapy were performed for 6 and 23 cases of lung cancer, respectively, as the first- or second-line anticancer treatment. The overall survival, CT findings, disease-free period before AE, and duration from the onset of AE to death were compared. Results The incidence of AE was 61.5% in the IPF-LCA group and 58.7% in the IPF group (p = 0.806). The mean overall survival in the IPF-LCA and IPF groups were 16.8 and 83.0 months, respectively (p < 0.001). The mean durations from the start of the lung cancer treatment to the onset of AE were 16.0 and 4.6 months in cases of surgical treatment and chemotherapy, respectively. In com- parison of death from AE, the survival rate was significantly lower in the IPF-LCA group than in the IPF group (p = 0.008). In the CT findings associated with AE, the IPF-LCA group tended to have a peribronchial (p < 0.001) or asymmetric distribution (p = 0.016).

    Received May 28, 2019 Revised August 14, 2019 Accepted September 3, 2019

    *Corresponding author Hee Kang, MD Department of Radiology, Kosin University Gospel Hospital, Kosin University College of Medicine, 262 Gamcheon-ro, Seo-gu, Busan 49267, Korea.

    Tel 82-51-990-6341 Fax 82-51-255-2764 E-mail

    This is an Open Access article distributed under the terms of the Creative Commons Attribu- tion Non-Commercial License ( licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduc- tion in any medium, provided the original work is properly cited.

    ORCID iDs Bumsang Cho https:// Hee Kang https:// Je Hun Kim https:// Jung Gu Park https:// Sekyoung Park https:// Jong Hyouk Yun https://

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    J Korean Soc Radiol 2020;81(3):688-700

    Conclusion In patients with IPF who develop lung cancer, the rate of death from AE is higher than that in patients with IPF alone. They tend to have unusual CT patterns associated with AE, such as a peribronchial or asymmetric distribution.

    Index terms Idiopathic Pulmonary Fibrosis; Lung Neoplasm; Usual Interstitial Pneumonia; Mortality


    Idiopathic pulmonary fibrosis (IPF) is the most common and fatal type of idiopathic in- terstitial pneumonia. It is defined as a specific form of chronic fibrosing interstitial pneu- monia of unknown cause and is associated with the histopathologic pattern of usual in- terstitial pneumonia (UIP) (1, 2). In most cases, IPF progresses relatively slowly, and acute lesions of varying degrees can be seen without specific causes. If a cause of this de- terioration cannot be identified, this deterioration is termed an acute exacerbation (AE) of IPF. The 1- to 3-year incidence of AE was reported at 14–20% (3). High-resolution CT reveals new ground-glass abnormalities or consolidation superimposed on the reticular or honeycomb abnormalities typical of UIP. Lung pathology in AE is characterized by a combination of underlying UIP patterns associated with diffuse alveolar damage or orga- nizing pneumonia features (4). AE is associated with poor outcomes and acute deteriora- tion in IPF, and preceded death in 47% of deaths in a previous study (5). The risk factors of AE are unknown infection, smoking, and pulmonary hypertension (6-8). It has also been recently observed that AE develops in lung cancer patients after thoracic surgery and chemotherapy (9).

    The previous literature reported there is significant difference in the survival time between IPF and IPF developing lung cancer patients. A shorter survival time is associated with the progression of lung cancer, as well as complications related to the diagnosis or treatment of lung cancer (9-11). To our knowledge, there has not yet been a study comparing the occur- rence of AE and its mortality between the IPF patient with or without lung cancer.

    The aim of our study was to compare the incidence and mortality of AE between IPF pa- tients with and without lung cancer and to investigate whether there are differences in CT findings.


    PATIENTS AND DATA COLLECTION Institutional Review Board approval was obtained for this retrospective study, and in-

    formed consent was waived (IRB No. 2018-08-025). Patients who were given a diagnosis of IPF on the basis of CT and clinical characteristics or histopathological confirmation were includ- ed between June 2004 and July 2018. The patient database in this institution revealed 150 pa- tients who satisfied the diagnostic criteria for UIP. Among them, 41 cases were excluded be- cause they did not have a follow-up CT scan. Also, 8 patients with connective tissue disease- related interstitial lung disease, 2 patients with asbestosis-related pulmonary fibrosis, and 10

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    Acute Exacerbation in IPF Patients with Lung Cancer

    patients who were not confirmed for lung cancer were excluded. Patients were divided into IPF with lung cancer (IPF-LCA) and IPF without lung cancer (IPF) groups. The medical re- cords including age, sex, smoking history, results of pulmonary function test (PFT), survival period, onset/period of AE, cause of death, and CT imaging findings were reviewed.

    IMAGE ACQUISITION The chest CT images were obtained at the time of the first diagnosis for IPF and onset of AE

    and the CT images were also obtained at the time of first diagnosis for lung cancer in the IPF- LCA group. CT scans were acquired using a multi-detector CT system (Somatom Sensation 64 or dual-source Flash 128 multi-detector CT system, Siemens Medical Solutions, Erlangen, Ger- many). Scanning was performed from the lower part of the neck to the level of the middle portion of the kidneys. Intravenous contrast medium injection was used in a part of patients including IPF-LCA. An amount of 1.5 mL/kg (body weight) of Iomeron 300 (Somatom Sensa- tion 64 or dual-source Flash 128 multi-detector CT system) was injected at an infusion rate of 3 mL/s using a power injector (Mallinckrodt, Tyco and Vistron CT, Medrad, Warrendale, PA, USA). The CT images were obtained with the following parameters: detector collimation 1.25 or 0.625 mm; field of view, 36 cm; 100 or 120 kVp; 90 to 200 mA; tube rotation time, 0.5 s; pitch, 1.2; and the reconstruction interval, 1 to 2.5 mm. CT data were reconstructed using a high- spatial-frequency algorithm for lung window images and by using a soft-tissue algorithm for mediastinal window images. On the monitors, both the mediastinal [window width, 400 Hounsfield unit (H); window level, 20 H] and lung (window width, 1500 H; window level, -700 H) window images were available for analysis.

    DIAGNOSIS OF IPF IPF was diagnosed according to the 2018 consensus of the American Thoracic Society, the

    European Respiratory Society, the Japanese Respiratory Society, and the Latin American Tho- racic Association (1, 12). UIP defined on chest CT by the presence of subpleural, base pre- dominance, reticular abnormality, honeycombing with or without peripheral traction bron- chiectasis or bronchiolectasis. We declared IPF when there was a pattern of UIP on CT. In cases of probable UIP or indeterminate with UIP on CT, IPF was diagnosed through multidis- ciplinary considerations including clinical and pathological results.

    The pathologic diagnosis of UIP included dense fibrosis, which causes remodeling of lung architecture with frequent honeycomb fibrosis; fibroblast foci, which are typically scattered at the edges of dense scars; patchy lung involvement; and frequent subpleural, paraseptal, and peripheral acinar distribution (13).

    The other forms of interstitial lung disease such as domestic and occupational environ- mental exposures, connective tissue disease, and drug toxicity were excluded.

    EVALUATION OF LUNG CANCER In the IPF-LCA group, all lung cancers were pathologically determined through surgery,

    bronchoscopy, or percutaneous biopsy. The histological type of cancer, stage at the time of diagnosis, types of treatment, treatment response and causes of deaths were reviewed. Chest CT