%0 Journal Article %A Yuji Yamamoto %A Kazuyuki Tsujino %A Tomoki Kuge %A Fukuko Okabe %A Takahiro Kawasaki %A Takanori Matsuki %A Hiroyuki Kagawa %A Mari Miki %A Keisuke Miki %A Masahide Mori %A Hiroshi Kida %T Pleuroparenchymal fibroelastosis in Mycobacterium avium complex pulmonary disease: clinical characteristics and prognostic impact %D 2021 %R 10.1183/23120541.00765-2020 %J ERJ Open Research %P 00765-2020 %V 7 %N 1 %X The association between Mycobacterium avium complex pulmonary disease (MAC-PD) and pleuroparenchymal fibroelastosis (PPFE) has been reported previously, and interstitial pneumonia as a comorbidity is associated with a worse prognosis. However, no study has thoroughly reported on PPFE associated with MAC-PD. The present study investigated the prevalence, clinical characteristics, and prognostic impact of PPFE in patients with MAC-PD.A total of 224 patients, newly diagnosed with MAC-PD, were retrospectively reviewed. At the time of diagnosis, chest high-resolution computed tomography (HRCT), sputum examination, and clinical characteristics were collected. The extent of PPFE and MAC-PD was evaluated semi-quantitatively using HRCT scores. Risk factor analysis for clinical or radiological deterioration necessitating multidrug antimicrobial treatment within 3 years, and all-cause mortality within 5 years, from the initial diagnosis was performed based on the PPFE score.PPFE was observed in 59 out of 224 patients (26.3%). A higher PPFE score was a risk factor for dyspnoea, fatigue, and lower body mass index (BMI) (p<0.05). Although PPFE score did not correlate with clinical or radiological deterioration within 3 years (p=0.576), a higher PPFE score (adjusted OR 1.66, 95% CI 1.06–2.60, p=0.028) and lower BMI (adjusted OR 0.61, 95% CI 0.39–0.94, p=0.028) increased the risk of 5-year mortality.PPFE is a relatively common complication and an independent poor prognostic factor of MAC-PD. This study highlights the need for further studies investigating whether the presence of PPFE can be a clinical indicator for initiating treatment of MAC-PD.The presence of pleuroparenchymal fibroelastosis in patients with Mycobacterium avium complex pulmonary disease is a risk factor for lower body mass index and worse prognosis https://bit.ly/3g8D6Di %U https://openres.ersjournals.com/content/erjor/7/1/00765-2020.full.pdf