Table 1.
Clinical data
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | Patient 8 | |
|---|---|---|---|---|---|---|---|---|
| Age (y) | 65 | 57 | 62 | 55 | 65 | 43 | 40 | 55 |
| Final diagnosis | iPPFE | iPPFE | iPPFE | iPPFE | iPPFE | iPPFE | iPPFE | ACFE |
| Smoke (Y/N/ex) | N | N | N | N | N | N | N | Ex |
| Sex (M/F) | F | F | F | F | F | M | F | M |
| Symptoms | chest pain, cough | fever, cough | fever, cough | weight loss, dyspnea | fever, chest pain and dyspnea | weight loss, dyspnea | dyspnea | Chest pain, dyspnea |
| FVC % | 80 | 75 | 61 | 62 | 79 | 58 | 94 | 62 |
| DLCO % | 74 | 64 | 55 | 51 | 72 | 69 | 74 | 64 |
Abbreviations: iPPFE = idiopathic pleuro-parenchymal fibroelastosis; ACFE = airway centered fibroelastosis