Table 3
Treatment and clinical course
| 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 |
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MP = medroxyprogesterone; FEV1 = forced expiratory volume in one second; Tlco = carbon monoxide lung transfer factor; NE = not examined.
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↵3-150 The time between symptom onset and diagnosis.
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↵3-151 FEV1 change (%) = percentage of (final FEV1— initial FEV1)/initial FEV1.
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↵3-152 Tlco change (%) = final Tlco (% predicted) — initial Tlco (% predicted).
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↵3-153 She experienced three pneumothoraces, two of which were during pregnancy.
