Table 1.
Clinical characteristics and laboratory data
| Case 1 | Case 2 | Case 3 | Case 4 | |
|---|---|---|---|---|
| Age at first onset (years) | 69 | 56 | 49 | 32 |
| Gender | Male | Male | Female | Female |
| Age at first surgical lung biopsy (years) | 69 | 56 | 49 | 32 |
| Age at second surgical lung biopsy (years) | – | – | 61 | – |
| Age at autopsy (years) | 73 | 62 | 35 | |
| Smoking status (pack-years) | Never-smoker | Smoker (40) | Never-smoker | Never-smoker |
| History of pneumothorax | No | No | No | Yes |
| Past history | NP | NP | NP | IPAH |
| Family history of interstitial pneumonia | No | No | No | No |
| Occupational history | Medical technologist | Welder for >25 years | Manager at a sushi bar | No occupational history |
| Administration of steroids | Yes | Yes | No | Yes |
| Body mass index (kg/m2) | 15.6 | 18.9 | 15.6 | 17.5 |
| First symptoms | Chest pain | Cough | Chest pain | Exercise dyspnoea |
| Crackles | Audible | Audible | Not audible | Not audible |
| Autoantibodies | No | No | No | No |
| KL-6 (U/ml) | 506 | 793 | 295 | 446 |
IPAH, Post-lung-transplanted state owing to idiopathic pulmonary arterial hypertension; KL-6, Krebs von den Lungen-6; NP, nothing particular.