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Pneumothorax in Patients with Idiopathic Pulmonary Fibrosis

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Pneumothorax in Patients with Idiopathic Pulmonary Fibrosis Yamazaki et al. BMC Pulm Med (2021) 21:5 https://doi.org/10.1186/s12890-020-01370-w RESEARCH ARTICLE Open Access Pneumothorax in patients with idiopathic pulmonary fbrosis: a real-world experience Ryo Yamazaki, Osamu Nishiyama* , Kyuya Gose, Sho Saeki, Hiroyuki Sano, Takashi Iwanaga and Yuji Tohda Abstract Background: Some patients with idiopathic pulmonary fbrosis (IPF) develop pneumothorax. However, the charac- teristics of pneumothorax in patients with IPF have not been elucidated. The purpose of this study was to clarify the clinical course, actual management, and treatment outcomes of pneumothorax in patients with IPF. Methods: Consecutive patients with IPF who were admitted for pneumothorax between January 2008 and Decem- ber 2018 were included. The success rates of treatment for pneumothorax, hospital mortality, and recurrence rate after discharge were examined. Results: During the study period, 36 patients with IPF were admitted with pneumothorax a total of 58 times. During the frst admission, 15 patients (41.7%) did not receive chest tube drainage, but 21 (58.3%) did. Of the 21 patients, 8 (38.1%) received additional therapy after chest drainage. The respective treatment success rates were 86.6% and 66.7% in patients who underwent observation only vs chest tube drainage. The respective hospital mortality rates were 13.3% and 38.0%. The total pneumothorax recurrence rate after hospital discharge was 34.6% (n 9). = Conclusions: Pneumothorax in patients with IPF was difcult to treat successfully, had a relatively poor prognosis, and showed a high recurrence rate. Keywords: Idiopathic pulmonary fbrosis, Hospitalization, Pneumothorax, Recurrence, Treatment Background pneumothorax was signifcantly associated with poor Idiopathic pulmonary fbrosis (IPF) is a specifc form survival in patients with IPF [11]. of chronic, progressive and fbrosing lung disease of In clinical practice, pneumothorax associated with unknown etiology [1]. Te prognosis for patients with IPF shows a wide range of severity, from mild, involving IPF is poor, and the median survival is 3–5 years. How- a small area that does not require any type of treatment, ever, its natural course is highly variable [2, 3]. Many to severe, which is refractory to intervention. Te charac- patients with IPF undergo acute respiratory events such teristics of pneumothorax in patients with IPF have not as acute exacerbation (AE), pulmonary infection, pul- been elucidated. Tis study aimed to clarify the clinical monary edema due to heart failure, pulmonary embo- course, actual management, and treatment outcome of lism, mediastinal emphysema, and pneumothorax [3–6]. pneumothorax in patients with IPF. Te rates of pneumothorax reportedly range from 2 to 20% in patients with IPF, which is second highest to the Methods rates occurring in patients with chronic obstructive pul- Patients monary disease (COPD) [7–11]. It has been shown that We retrospectively reviewed the clinical records of all patients with IPF who were frst admitted for pneumo- thorax to the Kindai University Hospital from January *Correspondence: [email protected] Department of Respiratory Medicine and Allergology, Kindai University 2008 to December 2019. Te diagnosis of IPF was made Faculty of Medicine, Osakasayama, Osaka, Japan according to the classifcation used in the INPULSIS © The Author(s) 2020. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creat iveco mmons .org/licen ses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/publi cdoma in/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Yamazaki et al. BMC Pulm Med (2021) 21:5 Page 2 of 6 trial [12]. Briefy, patients who had histological evidence Results of a usual interstitial pneumonia pattern on a surgical During the survey period, 36 patients with IPF were lung biopsy specimen were included. In the absence of admitted to our university hospital for pneumothorax a biopsy specimen, patients with the following fndings a total of 58 times. Te characteristics of the patients on high-resolution computed tomography (HRCT) were before their frst admission are shown in Table 1. Tis diagnosed with IPF: honeycombing and/or the pres- study included 29 men and 7 women. Te mean age of ence of reticular abnormality and traction bronchiectasis the study patients was 75.5 5.4 years, and the mean ± 2 without features suggestive of alternative causes. Patients body mass index was 18.5 ± 4.1 kg/m . Te mean forced with concomitant pleuroparenchymal fbroelastosis vital capacity (FVC) was 55.8% ± 16.5% predicted and the (PPFE) were excluded. mean difusing capacity for carbon monoxide (DLco) was 62.4% ± 14.9% predicted. Twenty-six patients (72.2%) had Data collection a smoking history. Seven patients (19.4%) were receiving We recorded the clinical characteristics of all the study corticosteroids for IPF. patients, which included the following: fndings on physi- Te clinical data of study patients at the frst admis- cal examination, standard laboratory tests performed on sion are shown in Table 2. Te mean values for Krebs admission, use of long-term oxygen therapy (LTOT), and von der Lungen-6 (KL-6), partial pressure of arterial treatments for IPF. Pulmonary function tests performed oxygen/fraction of inspiratory oxygen (PaO2/FiO2), and within 1 year prior to admission were used for assess- partial pressure of arterial carbon dioxide (PaCO2) were ment of baseline pulmonary function. We also assessed 957 ± 744 U/mL, 295 ± 100, and 46.8 ± 9.8 mm Hg, the specifc treatments for pneumothorax. respectively. Successful treatment for pneumothorax Treatments for pneumothorax were evaluated with Table 1 Baseline characteristics of the study patients and treatments for IPF before the frst admission regard to whether or not they were successful. Successful for pneumothorax management of pneumothorax was defned as hospital Characteristics N 36 discharge without a chest tube. A successful chest tube = treatment was defned as removal of the chest tube. Age, year 75.5 5.4 ± Gender Survival assessment Male/female 29/7 In-hospital deaths and deaths after discharges were ret- Body mass indexa, kg/m2 18.5 4.1 ± rospectively obtained from the study patients’ hospital Preadmission of PFT charts. FVCb, L 1.6 0.6 ± FVCb, % predicted 55.8 16.5 Recurrence of pneumothorax after frst discharge ± FEV b, L 1.5 0.5 from the hospital for successful treatment of frst 1 ± FEV b, % predicted 66.2 18.4 pneumothorax 1 ± FEV /FVCb, % 91.7 10.6 1 ± Data on recurrence of pneumothorax was assessed. Te DLcoc, mL/min/mmHg 8.3 2.2 ± time to recurrence was defned as the number of days DLcoc, % predicted 62.4 14.9 ± from the date of discharge from the frst pneumothorax Smoking status until the date of admission for recurrence of pneumotho- Current/former/never 1/25/10 rax. Contralateral pneumothorax was not included in the Treatment for IPF at baseline recurrence. Pirfenidone 4 Nintedanib 3 Statistical analysis Corticosteroid 7 Continuous variables were expressed as means ± stand- Cyclosporine 4 ard deviation (SD). Categorical variables were expressed None 22 as frequencies. We performed logistic regression analy- Long-term oxygen therapy sis to determine predictors of hospital death. P values Yes/no 9/27 less than 0.05 were considered statistically signifcant. Values are shown as actual numbers or means standard deviation Te median duration of survival after discharge was esti- ± N 36 (aN 35, bN 27, cN 12) mated on a Kaplan–Meier survival curve. Statfex ver.6 = = = = DLco, difusing capacity for carbon monoxide; FEV1, forced expiratory volume in software (Artech, CO., Ltd., Osaka, Japan) was used for 1 s; FVC, forced vital capacity; IPF, idiopathic pulmonary fbrosis; PFT, pulmonary analysis. function tests Yamazaki et al. BMC Pulm Med (2021) 21:5 Page 3 of 6 Table 2 Clinical data at the frst admission administered during all of the admissions was similar for pneumothorax to the success rate of each treatment during the frst Variable N 36 admission only. In patients whose chest tube could be = removed, the mean lengths of time until removal for KL-6a, U/mL 972 744 ± the frst admission and total number of admissions Arterial blood gas were 20.7 14.0 and 23.6 18.5 days, respectively. pHb 7.39 0.06 ± ± ± Some patients remained hospitalized even after the PaO /FiO ratiob 295 100 2 2 ± chest tube was removed, mainly because of subsequent PaCO b, mmHg 46.8 9.8 2 ± type 2 respiratory failure and/or pulmonary infection. Values are shown as actual numbers or means standard deviation ± Te hospital mortality rates for the frst admis- N 36 (aN 28, bN 33) = = = sion and for all the admissions combined were 27.7% KL-6, Krebs von der Lungen-6; PaCO2, partial pressure of arterial carbon dioxide; and 24.1%, respectively (Table 4). Te mean durations PaO /FiO , partial pressure of arterial oxygen/fraction of inspiratory oxygen 2 2 of hospitalization for the frst and total number of admissions were 34.2 ± 27.5 days and 31.0 ± 28.5 days, respectively.
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