From: Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis
Characteristics | Sirolimus | Non-sirolimus | P-value |
---|---|---|---|
Number of patients | 22 | 22 | |
Death or lung transplantation | 0 (0.0) | 3 (13.6) | 0.233 |
Disease progression | 4/21 (19.0) | 6/22 (27.3) | 0.721 |
Lung function decline, % predicted/year | N = 17 | N = 19 | |
FEV1, % predicted/year | 0.5 ± 3.0 | − 0.3 ± 4.7 | 0.452 |
FVC, % predicted/year | 0.7 ± 3.1 | 0.0 ± 4.1 | > 0.999 |
DLCO, % predicted/year | − 1.2 ± 3.9 | 0.6 ± 5.5 | 0.271 |
RV, % predicted/year | 0.1 ± 5.8 | − 3.4 ± 15.3 | 0.485 |
FEF25-75%, % predicted/year | − 1.3 ± 4.1 | − 5.7 ± 13.9 | 0.808 |
Median follow-up period, year | 8.5 (3.8–11.5) | 6.1 (2.4–9.5) | 0.213 |