First author [ref.] | Study design | Total subjects | Subjects with LAM | Subjects who withdrew | Mean % predicted FEV1 | Treatment | Length of follow-up years | Risk of bias |
Treatment | ||||||||
Bissler [8] | NROL | 25 | 18 | 5 | 57.2 | Sirolimus | 1.0 | Low |
Dabora [9] | NROL | 36 | 21 | 8 | # | Sirolimus | 1.0 | Low |
Davies [10] | NROL | 16 | 9 | 6 | 56.9 | Sirolimus | 2.0 | Low |
Neurohr [13] | NROL/RC | 10 | 10 | 3 | 36.1 | Sirolimus | 1.0¶ | Serious |
McCormack [3] | RPC | 46 | 46 | 5 | 49.3 | Sirolimus | 1.0 | Low |
Ando [15] | RC | 15 | 15 | 0 | # | Sirolimus | 1.4¶ | Critical |
Bissler [5] | RPC | 79 | 22 | 7 | # | Everolimus | 0.7¶ | Low |
Mohammadieh [14] | NROL/RC | 5 | 5 | 0 | NR | Everolimus | 0.5 | Serious |
Goldberg [11] | NROL | 24 | 24 | 7 | 60.4 | Everolimus | 1.2 | Low |
Yao [12] | NROL | 38 | 38 | 0 | 72.1 | Sirolimus | 3.4¶ | Low |
Placebo | ||||||||
McCormack [3] | RPC | 43 | 43 | 7 | 47.7 | Placebo | 1.0 | Low |
Bissler [5] | RPC | 39 | 7 | 13 | # | Placebo | 0.6 | Low |
Chang [4] | RPC | 11 | 11 | 4 | 64.0 | Placebo | 2.0 | Low |
All studies | ||||||||
Total | 387 | 269 | 68 | |||||
Mean±sd | 1.4±0.8 |
LAM: lymphangioleiomyomatosis; FEV1: forced expiratory volume in 1 s; NROL: non-randomised open label; RC: retrospective cohort; RPC: randomised placebo controlled. #: reported as absolute FEV1 or relative change in FEV1 rather than % predicted FEV1; ¶: reported as mean or median follow-up.