Summary of the 12 cases of pleuroparenchymal fibroelastosis (PPFE)

Subjects n12
Idiopathic/secondary PPFE n/n#8/4
Age years62 (27–70)
 Males7 (58)
 Females5 (42)
BMI kg·m−220.0 (13.5–24.2)
Smoking habits
 Never-smoker7 (58)
 Current or ex-smoker5 (42)
Clinical symptoms
 Asymptomatic3 (25)
 Symptomatic9 (75)
  Dyspnoea on exertion7 (58)
  Dry cough5 (42)
  Chest pain1 (8)
Pneumothorax8 (67)
HRCT findings+
 Pleuroparenchymal thickening12 (100)
 Elevated hilar shadows10 (83)
 Coexistent ILD in lower lobes11 (92)
  UIP pattern5 (42)
  Possible UIP pattern4 (33)
  NSIP pattern1 (8)
  Others1 (8)
Pathological findings
 Subpleural elastosis12 (100)
 Intra-alveolar collagen deposition12 (100)
 Pleural thickening with fibrosis8 (67)
 Preserved alveolar structure12 (100)
 Coexistent ILD in lower lobes9 (75)
  UIP pattern8 (67)
  Non-classifiable interstitial pneumonia pattern1 (8)
Pulmonary function test (n=8)
 FVC % predicted70.6 (53.8–108.6)
 FVC decline mL·year−1−187 (−878–4)
 TLC % predicted71.7 (50.3–133.9)
 TLC decline mL·year−1−310 (−1335– −54)
 Steroid6 (50)
 Immunosuppressant7 (58)
 Pirfenidone4 (33)
Median survival time days
 From the first visit2459 (55–2996)
 From SLB838 (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.