Summary of the 12 cases of pleuroparenchymal fibroelastosis (PPFE)
| Subjects n | 12 |
| Idiopathic/secondary PPFE n/n# | 8/4 |
| Age years¶ | 62 (27–70) |
| Sex | |
| Males | 7 (58) |
| Females | 5 (42) |
| BMI kg·m−2¶ | 20.0 (13.5–24.2) |
| Smoking habits¶ | |
| Never-smoker | 7 (58) |
| Current or ex-smoker | 5 (42) |
| Clinical symptoms¶ | |
| Asymptomatic | 3 (25) |
| Symptomatic | 9 (75) |
| Dyspnoea on exertion | 7 (58) |
| Dry cough | 5 (42) |
| Chest pain | 1 (8) |
| Pneumothorax | 8 (67) |
| HRCT findings+ | |
| Pleuroparenchymal thickening | 12 (100) |
| Elevated hilar shadows | 10 (83) |
| Coexistent ILD in lower lobes | 11 (92) |
| UIP pattern | 5 (42) |
| Possible UIP pattern | 4 (33) |
| NSIP pattern | 1 (8) |
| Others | 1 (8) |
| Pathological findings | |
| Subpleural elastosis | 12 (100) |
| Intra-alveolar collagen deposition | 12 (100) |
| Pleural thickening with fibrosis | 8 (67) |
| Preserved alveolar structure | 12 (100) |
| Coexistent ILD in lower lobes | 9 (75) |
| UIP pattern | 8 (67) |
| Non-classifiable interstitial pneumonia pattern | 1 (8) |
| Pulmonary function test (n=8) | |
| FVC % predicted¶ | 70.6 (53.8–108.6) |
| FVC decline mL·year−1 | −187 (−878–4) |
| TLC % predicted¶ | 71.7 (50.3–133.9) |
| TLC decline mL·year−1 | −310 (−1335– −54) |
| Medication | |
| Steroid | 6 (50) |
| Immunosuppressant | 7 (58) |
| Pirfenidone | 4 (33) |
| Median survival time days | |
| From the first visit | 2459 (55–2996) |
| From SLB | 838 (29–2014) |
Data are presented as median (range) or n (%) unless otherwise stated. BMI: body mass index; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; UIP: usual interstitial pneumonia; NSIP: nonspecific interstitial pneumonia; FVC: forced vital capacity; TLC: total lung capacity; SLB: surgical lung biopsy. #: of the four secondary PPFE cases one was a cyclophosphamide-induced case and the remaining three cases coexisted with chronic hypersensitivity pneumonia, rheumatoid arthritis or Sjögren's syndrome; ¶: the data were obtained at the first visit; +: the data were obtained before the SLB.