Yasunori Enomoto1, Yutaro Nakamura2, Yasuomi Satake3, Hiromitsu Sumikawa4, Takeshi Johkoh5, Thomas V Colby6, Hideki Yasui3, Hironao Hozumi3, Masato Karayama3, Yuzo Suzuki3, Kazuki Furuhashi3, Tomoyuki Fujisawa3, Noriyuki Enomoto3, Naoki Inui7, Toshihide Iwashita8, Shigeki Kuroishi9, Koshi Yokomura10, Naoki Koshimizu11, Mikio Toyoshima12, Shiro Imokawa13, Takashi Yamada14, Toshihiro Shirai15, Hiroshi Hayakawa16, Takafumi Suda3. 1. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan; Department of Regenerative and Infectious Pathology, Hamamatsu University School of Medicine, Shizuoka, Japan. Electronic address: enomotoy@hama-med.ac.jp. 2. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan. Electronic address: nakayuta@hama-med.ac.jp. 3. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan. 4. Department of Radiology, Osaka International Cancer Institute, Osaka, Japan. 5. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public Teachers, Hyogo, Japan. 6. Department of Laboratory Medicine and Pathology (Emeritus), Mayo Clinic Arizona, Scottsdale, AZ, USA. 7. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan; Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Shizuoka, Japan. 8. Department of Regenerative and Infectious Pathology, Hamamatsu University School of Medicine, Shizuoka, Japan. 9. Department of Internal Medicine, Enshu Hospital, Shizuoka, Japan. 10. Department of Respiratory Medicine, Seirei Mikatahara General Hospital, Shizuoka, Japan. 11. Department of Respiratory Medicine, Fujieda Municipal General Hospital, Shizuoka, Japan. 12. Department of Respiratory Medicine, Hamamatsu Rosai Hospital, Shizuoka, Japan. 13. Department of Respiratory Medicine, Iwata City Hospital, Shizuoka, Japan. 14. Department of Respiratory Medicine, Shizuoka City Shizuoka Hospital, Shizuoka, Japan. 15. Department of Respiratory Medicine, Shizuoka General Hospital, Shizuoka, Japan. 16. Department of Respiratory Medicine, Tenryu Hospital, National Hospital Organization, Shizuoka, Japan.
Abstract
BACKGROUND: Although the accurate diagnosis of pleuroparenchymal fibroelastosis (PPFE) requires pathologic evaluation, this diagnosis is often suggested when the radiologic findings are consistent with typical PPFE and when pulmonary apical cap, which radiologically and pathologically mimics PPFE, can be excluded by confirming disease progression. The aim of this study was to evaluate the validity of the clinical diagnosis of idiopathic PPFE. METHODS: We recruited 44 patients with idiopathic PPFE according to our modified diagnostic criteria: 1) a radiologic PPFE pattern (i.e., bilateral subpleural dense consolidation with or without pleural thickening in the upper lobes and less marked or absent involvement of the lower lobes), 2) radiologic confirmation of disease progression, and 3) exclusion of other lung diseases with identifiable etiologies. The patients' baseline characteristics and clinical course were reviewed. RESULTS: The median age was 70 years, and 28 patients were males. The majority revealed emaciation, hypercapnia, and a high ratio of residual volume to total lung capacity. On chest computed tomography, 39 patients showed abnormal shadows in the lower lobes; more than half were classified as having usual interstitial pneumonia (UIP)/possible UIP pattern. Pneumothorax was the most frequent complication (33/44). The median overall survival time after diagnosis was 35.3 months. The presence of lower lobe UIP/possible UIP pattern did not show a significant prognostic impact. CONCLUSIONS: Using our diagnostic criteria, we could recruit relatively many patients with similar characteristics to those of idiopathic PPFE patients in the literature. The possibility of clinical diagnosis of idiopathic PPFE should be further discussed.
BACKGROUND: Although the accurate diagnosis of pleuroparenchymal fibroelastosis (PPFE) requires pathologic evaluation, this diagnosis is often suggested when the radiologic findings are consistent with typical PPFE and when pulmonary apical cap, which radiologically and pathologically mimics PPFE, can be excluded by confirming disease progression. The aim of this study was to evaluate the validity of the clinical diagnosis of idiopathic PPFE. METHODS: We recruited 44 patients with idiopathic PPFE according to our modified diagnostic criteria: 1) a radiologic PPFE pattern (i.e., bilateral subpleural dense consolidation with or without pleural thickening in the upper lobes and less marked or absent involvement of the lower lobes), 2) radiologic confirmation of disease progression, and 3) exclusion of other lung diseases with identifiable etiologies. The patients' baseline characteristics and clinical course were reviewed. RESULTS: The median age was 70 years, and 28 patients were males. The majority revealed emaciation, hypercapnia, and a high ratio of residual volume to total lung capacity. On chest computed tomography, 39 patients showed abnormal shadows in the lower lobes; more than half were classified as having usual interstitial pneumonia (UIP)/possible UIP pattern. Pneumothorax was the most frequent complication (33/44). The median overall survival time after diagnosis was 35.3 months. The presence of lower lobe UIP/possible UIP pattern did not show a significant prognostic impact. CONCLUSIONS: Using our diagnostic criteria, we could recruit relatively many patients with similar characteristics to those of idiopathic PPFEpatients in the literature. The possibility of clinical diagnosis of idiopathic PPFE should be further discussed.