Kentaro Watanabe1, Hiroshi Ishii2, Fumiaki Kiyomi3, Yasuhiro Terasaki4, Akira Hebisawa5, Yoshinori Kawabata6, Takeshi Johkoh7, Fumikazu Sakai8, Yasuhiro Kondoh9, Yoshikazu Inoue10, Arata Azuma11, Takafumi Suda12, Takashi Ogura13, Naohiko Inase14, Sakae Homma15. 1. General Medical Research Center, Fukuoka University School of Medicine, 814-0180 Fukuoka, Japan. Electronic address: watanabe@fukuoka-u.ac.jp. 2. Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan. 3. Clinical Research Support Center Kyushu, Fukuoka, Japan. 4. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. 5. Department of Clinical Research, National Hospital Organization Tokyo National Hospital, Japan. 6. Division of Diagnostic Pathology, Saitama Cardiovascular and Respiratory Center, Saitama, Japan. 7. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Hyogo, Japan. 8. Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, Saitama, Japan. 9. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, Japan. 10. Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Osaka, Japan. 11. Department of Pulmonary Medicine, Nippon Medical School, Tokyo, Japan. 12. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. 13. Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Kanagawa, Japan. 14. Department of Respiratory Medicine, Tokyo Medical and Dental University, Japan. 15. Department of Respiratory Medicine, Toho University Omori Medical Center, Japan.
Abstract
BACKGROUND: Physicians have few opportunities to perform surgical lung biopsy (SLB) to diagnose idiopathic pleuroparenchymal fibroelastosis (IPPFE). Therefore, diagnostic criteria for IPPFE that do not require SLB must be established. Herein, we propose diagnostic criteria for IPPFE with and without SLB. METHODS AND RESULTS: The diagnostic criteria for IPPFE with SLB are histological, based on computed tomography (CT) lesions compatible with PPFE, predominantly in the upper lobes. The three diagnostic criteria for IPPFE without SLB are as follows: (1) radiologically possible IPPFE (a radiological criterion confirming CT lesions in both lung apexes, regardless of the lower lobe lesions); (2) radiologically probable IPPFE (also a radiological criterion, but mandatory to confirm chest radiograph findings of bilateral upward shift of the hilar structures and/or CT findings of volume loss of the upper lobes); (3) radiologically and physiologically probable IPPFE. Our data from 41 patients with IPPFE and 97 with idiopathic pulmonary fibrosis (IPF) showed that the percentage of the predicted values of the ratio of residual volume to total lung capacity (RV/TLC %pred.) ≥115% and body mass index (BMI) ≤20 kg/m2 plus RV/TLC %pred. ≥80% performed well for discriminating IPPFE from IPF. These parameters were thus added to criterion (3). CONCLUSIONS: We have proposed diagnostic criteria for IPPFE in patients with and without SLB. Both imaging criteria and physiological criteria using RV/TLC and BMI successfully discriminate IPPFE from chronic IIPs when SLB cannot be performed.
BACKGROUND: Physicians have few opportunities to perform surgical lung biopsy (SLB) to diagnose idiopathic pleuroparenchymal fibroelastosis (IPPFE). Therefore, diagnostic criteria for IPPFE that do not require SLB must be established. Herein, we propose diagnostic criteria for IPPFE with and without SLB. METHODS AND RESULTS: The diagnostic criteria for IPPFE with SLB are histological, based on computed tomography (CT) lesions compatible with PPFE, predominantly in the upper lobes. The three diagnostic criteria for IPPFE without SLB are as follows: (1) radiologically possible IPPFE (a radiological criterion confirming CT lesions in both lung apexes, regardless of the lower lobe lesions); (2) radiologically probable IPPFE (also a radiological criterion, but mandatory to confirm chest radiograph findings of bilateral upward shift of the hilar structures and/or CT findings of volume loss of the upper lobes); (3) radiologically and physiologically probable IPPFE. Our data from 41 patients with IPPFE and 97 with idiopathic pulmonary fibrosis (IPF) showed that the percentage of the predicted values of the ratio of residual volume to total lung capacity (RV/TLC %pred.) ≥115% and body mass index (BMI) ≤20 kg/m2 plus RV/TLC %pred. ≥80% performed well for discriminating IPPFE from IPF. These parameters were thus added to criterion (3). CONCLUSIONS: We have proposed diagnostic criteria for IPPFE in patients with and without SLB. Both imaging criteria and physiological criteria using RV/TLC and BMI successfully discriminate IPPFE from chronic IIPs when SLB cannot be performed.
Authors: Hiroto Hatabu; Gary M Hunninghake; Luca Richeldi; Kevin K Brown; Athol U Wells; Martine Remy-Jardin; Johny Verschakelen; Andrew G Nicholson; Mary B Beasley; David C Christiani; Raúl San José Estépar; Joon Beom Seo; Takeshi Johkoh; Nicola Sverzellati; Christopher J Ryerson; R Graham Barr; Jin Mo Goo; John H M Austin; Charles A Powell; Kyung Soo Lee; Yoshikazu Inoue; David A Lynch Journal: Lancet Respir Med Date: 2020-07 Impact factor: 30.700