Histological characteristics of patients with SFTPC mutations
| All patients (n=13) | Non-BRICHOS domain (n=9) | BRICHOS domain (n=4) | |
|---|---|---|---|
| Median age at biopsy (range), in month | 6 (3–68) | 8 (4–68) | 4 (3–6) |
| Type II cells hyperplasia | 13 (100) | 8 (62) | 5 (38) |
| Septal thickening | 13 (100) | 8 (62) | 5 (38) |
| Mild fibrosis | 7 (54) | 4 (31) | 3 (23) |
| Lymphoplasmocytic infiltrate | 11 (85) | 8 (62) | 3 (23) |
| Intra-alveolar macrophages | 10 (77) | 6 (46) | 4 (31) |
| Intra-alveolar material accumulation | 7 (54) | 3 (23) | 4 (31) |
The percentage of the total is indicated in parentheses.