Cochrane · systematic review · CD013403
Interventions for the long‐term prevention of hereditary angioedema attacks
Beard, N, Frese, M, Smertina, E, et al. · Cochrane Database of Systematic Reviews · 2022 · Cochrane Library
VascularAMSTAR 2: not assessed
SoF tableOnly the outcomes this review put in its Summary of Findings table are listed (the Cochrane Handbook caps them at about seven per comparison). Its other analyses are not shown here.
Questions
49
current version
GRADE rated
31
No studies
18
Included studies
NR
No studies 18 · 36.7%Ungraded 0 · 0%Very low 8 · 16.3%Low 18 · 36.7%Moderate 5 · 10.2%High 0 · 0%
Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.
Questions and results
Avoralstat compared with placebo for preventing hereditary angioedema attacks
Population: children or adults with Types I or II HAE
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Risk of HAE attacks (during Avoralstat vs placebo | RR 0.99 [0.92, 1.06] | 1 study · 110 participants | Moderate Downgraded: imprecision |
Change in number of HAE attacks (per week) Avoralstat vs placebo | no estimate extracted | 2 studies · 134 participants | Low Downgraded: imprecision, inconsistency |
Mortality (during Avoralstat vs placebo | no estimate extracted | NR | No studies |
Serious adverse events (during Avoralstat vs placebo | RR 0.33 [0.01, 7.8] | 1 study · 24 participants | Very low Downgraded: imprecision, indirectness |
Quality of lifeAngioedema Quality of Life scale (lower score is better)(during Avoralstat vs placebo | no estimate extracted | 2 studies · 93 participants | Moderate Downgraded: imprecision |
Disability (any validated scale) (during Avoralstat vs placebo | no estimate extracted | NR | No studies |
Adverse events (during Avoralstat vs placebo | RR 0.85 [0.62, 1.16] | 1 study · 24 participants | Low Downgraded: imprecision, inconsistency |
Berotralstat compared with placebo or active control for preventing hereditary angioedema attacks
Population: children or adults with Types I or II HAE
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Risk of HAE attacks (during Berotralstat vs placebo or active control | RR 0.63 [0.39, 1] | 1 study · 37 participants | Low Downgraded: imprecision |
Change in number of HAE attacks (per week) Berotralstat vs placebo or active control | no estimate extracted | 3 studies · 130 participants | Low Downgraded: imprecision |
Mortality (during Berotralstat vs placebo or active control | no estimate extracted | NR | No studies |
Serious adverse events (during Berotralstat vs placebo or active control | RR 0.77 [0.02, 24.03] | 3 studies · 128 participants | Low Downgraded: imprecision |
Quality of lifeAngioedema Quality of Life scale(lower score is better)(during Berotralstat vs placebo or active control | no estimate extracted | 3 studies · 130 participants | Moderate Downgraded: imprecision |
DisabilityStandardised mean difference (lower is better) (during Berotralstat vs placebo or active control | no estimate extracted | 2 studies · 50 participants | Low Downgraded: imprecision |
Adverse events(during Berotralstat vs placebo or active control | RR 1.03 [0.88, 1.22] | 3 studies · 128 participants | Moderate Downgraded: imprecision |
C1 esterase inhibitor compared with placebo or active control for preventing hereditary angioedema attacks
Population: children or adults with Types I or II HAE
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Risk of HAE attacks (during C1 esterase inhibitor vs placebo or active control | RR 0.29 [0.16, 0.5] | 1 study · 43 participants | Low Downgraded: imprecision |
Change in number of HAE attacks (per week) C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 45 participants | Low Downgraded: imprecision |
Mortality (during C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Serious adverse events (during C1 esterase inhibitor vs placebo or active control | RR 0.34 [0.01, 8.14] | 1 study · 44 participants | Very low Downgraded: imprecision |
Quality of lifestandardised mean difference (lower is better) (during C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 36 participants | Low Downgraded: imprecision |
Disability (any validated scale) (during C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Adverse events (during C1 esterase inhibitor vs placebo or active control | RR 1.03 [0.84, 1.27] | 1 study · 44 participants | Moderate Downgraded: imprecision |
Plasma‐derived C1 esterase inhibitor compared with placebo or active control for preventing hereditary angioedema attacks
Population: children or adults with Types I or II HAE
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Risk of HAE attacks (during Plasma‐derived C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Change in number of HAE attacks (per week) Plasma‐derived C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 71 participants | Low Downgraded: imprecision |
Mortality (during Plasma‐derived C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Serious adverse events (during Plasma‐derived C1 esterase inhibitor vs placebo or active control | RR 0.54 [0.09, 3.1] | 1 study · 71 participants | Very low Downgraded: imprecision |
Quality of lifeAngioedema Quality of Life Score(lower score is better) (during Plasma‐derived C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 31 participants | Low Downgraded: imprecision |
Disability(any validated scale) (during Plasma‐derived C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Adverse events(during Plasma‐derived C1 esterase inhibitor vs placebo or active control | RR 1.05 [0.78, 1.42] | 1 study · 71 participants | Low Downgraded: imprecision |
Nanofiltered C1 esterase inhibitor compared with placebo or active control for preventing hereditary angioedema attacks
Population: children or adults with Types I or II HAE
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Risk of HAE attacks (during Nanofiltered C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Change in number of HAE attacks (per week) Nanofiltered C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision |
Mortality (during Nanofiltered C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Serious adverse events (during Nanofiltered C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Quality of lifestandardised mean difference (lower is better) (during Nanofiltered C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 16 participants | Very low Downgraded: imprecision |
Disability standardised mean difference (lower is better) (during Nanofiltered C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 16 participants | Very low Downgraded: imprecision |
Adverse events (during Nanofiltered C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Recombinant human C1 esterase inhibitor compared with placebo or active control for preventing hereditary angioedema attacks
Population: children or adults with Types I or II HAE
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Risk of HAE attacks (during Recombinant human C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Change in number of HAE attacks (per week) Recombinant human C1 esterase inhibitor vs placebo or active control | no estimate extracted | 1 study · 32 participants | Very low Downgraded: imprecision |
Mortality (during Recombinant human C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Serious adverse events (during Recombinant human C1 esterase inhibitor vs placebo or active control | RR 1.5 [0.06, 34.66] | 1 study · 29 participants | Very low Downgraded: imprecision |
Quality of lifestandardised mean difference(during Recombinant human C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Disability(any validated scale)(during Recombinant human C1 esterase inhibitor vs placebo or active control | no estimate extracted | NR | No studies |
Adverse events(during Recombinant human C1 esterase inhibitor vs placebo or active control | RR 1.39 [0.71, 2.7] | 1 study · 29 participants | Low Downgraded: imprecision |
Lanadelumab compared with placebo or active control for preventing hereditary angioedema attacks
Population: children or adults with Types I or II HAE
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Risk of HAE attacks (during Lanadelumab vs placebo or active control | no estimate extracted | NR | No studies |
Change in number of HAE attacks (per week) Lanadelumab vs placebo or active control | no estimate extracted | 2 studies · 83 participants | Low Downgraded: imprecision |
Mortality (during Lanadelumab vs placebo or active control | no estimate extracted | NR | No studies |
Serious adverse events (during Lanadelumab vs placebo or active control | RR 0.88 [0.08, 10.39] | 2 studies · 162 participants | Low Downgraded: imprecision |
Quality of lifestandardised mean difference(lower is better)(during Lanadelumab vs placebo or active control | no estimate extracted | 1 study · 68 participants | Low Downgraded: imprecision |
DisabilityStandardised mean difference(lower is better)(during Lanadelumab vs placebo or active control | no estimate extracted | 1 study · 64 participants | Low Downgraded: imprecision |
Adverse events(during Lanadelumab vs placebo or active control | RR 1.07 [0.77, 1.47] | 2 studies · 158 participants | Low Downgraded: imprecision |