Case | Diagnostic delay3-150 | Treatment | Duration of follow up (months) | Symptomatic change | FEV1 change (%)3-151 | Tlco change (%)3-152 | Final result |
---|---|---|---|---|---|---|---|
2 | 2 years | MP + tamoxifen | 13 | Deterioration | −17.9 | −2 | Progression |
3 | 12 months | MP + tamoxifen | 43 | Deterioration | −59.7 | −47 | Progression |
4 | 3 years | MP + tamoxifen | 29 | Deterioration | −59.3 | −2 | Progression |
5 | 2 years | MP + tamoxifen | 44 | No change | 20.8 | −18 | Stable |
6 | 3 years | MP | 54 | Deterioration | −46.2 | −20 | Death from respiratory insufficiency |
8 | 9 years | MP + tamoxifen | 25 | Deterioration | −29.5 | 9 | Death from infection following transplantation |
15 | 3 years | MP | 15 | Deterioration | −34.9 | −4 | Progression |
17 | 4 years | Tamoxifen | 12 | Deterioration3-153 | NE | NE | Death from respiratory insufficiency |
20 | 2 months | MP | 12 | No change | 3.0 | NE | Stable |
21 | 7 years | MP | 12 | No change | −8.3 | −3 | Progression |
MP = medroxyprogesterone; FEV1 = forced expiratory volume in one second; Tlco = carbon monoxide lung transfer factor; NE = not examined.
↵3-150 The time between symptom onset and diagnosis.
↵3-151 FEV1 change (%) = percentage of (final FEV1— initial FEV1)/initial FEV1.
↵3-152 Tlco change (%) = final Tlco (% predicted) — initial Tlco (% predicted).
↵3-153 She experienced three pneumothoraces, two of which were during pregnancy.