Source	Search strategy	Hits retrieved
VASCULAR REGISTER IN CRSW (date of most recent search: 3 August 2021)	Angioedema* OR HAE AND INREGISTER	August 2019: 1 May 2020: 0 August 2021: 0
CENTRAL via CRSO (date of most recent search: 3 August 2021)	#1 MESH DESCRIPTOR Angioedemas, Hereditary EXPLODE ALL TREES 77 #2 (angioedema ADJ3 hereditary):TI,AB,KY 29 #3 HAE:TI,AB,KY 259 #4 #1 OR #2 OR #3 283 #5 MESH DESCRIPTOR Antibodies, Monoclonal EXPLODE ALL TREES 10335 #6 MESH DESCRIPTOR Bradykinin B2 Receptor Antagonists EXPLODE ALL TREES 13 #7 MESH DESCRIPTOR Complement C1 Inactivator Proteins EXPLODE ALL TREES 83 #8 MESH DESCRIPTOR Complement C1 Inhibitor Protein EXPLODE ALL TREES 59 #9 MESH DESCRIPTOR DANAZOL EXPLODE ALL TREES 197 #10 MESH DESCRIPTOR KALLIKREINS EXPLODE ALL TREES 1385 #11 MESH DESCRIPTOR Recombinant Proteins EXPLODE ALL TREES 8176 #12 MESH DESCRIPTOR Tranexamic Acid EXPLODE ALL TREES 830 #13 (attenuated androgens):TI,AB,KY 4 #14 BCX7353:TI,AB,KY 21 #15 Berinert:TI,AB,KY 25 #16 (bradykinin B2 receptor antagonists):TI,AB,KY 13 #17 (C1 esterase inhibitors):TI,AB,KY 0 #18 C1INH:TI,AB,KY 38 #19 C1‐INH:TI,AB,KY 115 #20 Cinryze*:TI,AB,KY 21 #21 (Complement C1*):TI,AB,KY 111 #22 (concentrated C1 esterase inhibitors):TI,AB,KY 0 #23 Danazol:TI,AB,KY 405 #24 Ecallantide:TI,AB,KY 39 #25 FFP:TI,AB,KY 400 #26 Firazyr:TI,AB,KY 7 #27 (fresh frozen plasma):TI,AB,KY 696 #28 Icatibant:TI,AB,KY 84 #29 Kalbitor:TI,AB,KY 0 #30 (kallikrein inhibit*):TI,AB,KY 113 #31 Lanadelumab:TI,AB,KY 28 #32 (monoclonal antibody):TI,AB,KY 6661 #33 (monoclonal anti‐f XII antibody):TI,AB,KY 0 #34 (nanofiltered C1 inhibitor):TI,AB,KY 10 #35 rhC1INH:TI,AB,KY 31 #36 rhC1‐INH:TI,AB,KY 5 #37 Ruconest:TI,AB,KY 5 #38 SDP:TI,AB,KY 73 #39 (tranexamic acid):TI,AB,KY 2225 #40 #5 OR #6 OR #7 OR #8 OR #9 OR #10 OR #11 OR #12 OR #13 OR #14 OR #15 OR #16 OR #17 OR #18 OR #19 OR #20 OR #21 OR #22 OR #23 OR #24 OR #25 OR #26 OR #27 OR #28 OR #29 OR #30 OR #31 OR #32 OR #33 OR #34 OR #35 OR #36 OR #37 OR #38 OR #39 28198 #41 #4 AND #40 238	August 2019: 238 May 2020: 115 August 2021: 75
ClinicalTrials.gov (date of most recent search: 3 August 2021)	Angioedemas, Hereditary OR HAE | Antibodies, Monoclonal OR Bradykinin B2 Receptor Antagonists OR Complement C1 Inactivator Proteins OR Complement C1 Inhibitor Protein OR DANAZOL OR KALLIKREINS OR Recombinant Proteins OR Tranexamic Acid OR attenuated androgens	August 2019: 61 May 2020: 2 August 2021: 11
ICTRP Search Portal (ICTRP portal not available May 2020) (date of most recent search: 3 August 2021)	Angioedemas, Hereditary OR HAE | Antibodies, Monoclonal OR Bradykinin B2 Receptor Antagonists OR Complement C1 Inactivator Proteins OR Complement C1 Inhibitor Protein OR DANAZOL OR KALLIKREINS OR Recombinant Proteins OR Tranexamic Acid OR attenuated androgens	August 2019: 207 May 2020: n/a August 2021: 15
MEDLINE (Ovid MEDLINE Epub Ahead of Print, In‐Process & Other Non‐Indexed Citations, Ovid MEDLINE Daily and Ovid MEDLINE) 1946 to present (date of most recent search: 3 August 2021)	1 exp Angioedemas, Hereditary/ 2 (angioedema adj3 hereditary).ti,ab. 3 HAE.ti,ab. 4 or/1‐3 5 exp Antibodies, Monoclonal/ 6 exp Bradykinin B2 Receptor Antagonists/ 7 exp Complement C1 Inactivator Proteins/ 8 exp Complement C1 Inhibitor Protein/ 9 exp DANAZOL/ 10 exp KALLIKREINS/ 11 exp Recombinant Proteins/tu [Therapeutic Use] 12 exp Tranexamic Acid/ 13 attenuated androgens.ti,ab. 14 BCX7353.ti,ab. 15 Berinert.ti,ab. 16 bradykinin B2 receptor antagonists.ti,ab. 17 C1 esterase inhibitors.ti,ab. 18 C1INH.ti,ab. 19 C1‐INH.ti,ab. 20 Cinryze*.ti,ab. 21 Complement C1*.ti,ab. 22 concentrated C1 esterase inhibitors.ti,ab. 23 Danazol.ti,ab. 24 Ecallantide.ti,ab. 25 FFP.ti,ab. 26 Firazyr.ti,ab. 27 fresh frozen plasma.ti,ab. 28 Icatibant.ti,ab. 29 Kalbitor.ti,ab. 30 kallikrein inhibit*.ti,ab. 31 Lanadelumab.ti,ab. 32 monoclonal antibody.ti,ab. 33 monoclonal anti‐f XII antibody.ti,ab. 34 nanofiltered C1 inhibitor.ti,ab. 35 rhC1INH.ti,ab. 36 rhC1‐INH.ti,ab. 37 Ruconest.ti,ab. 38 SDP.ti,ab. 39 tranexamic acid.ti,ab. 40 or/5‐39 41 4 and 40 42 randomized controlled trial.pt. 43 controlled clinical trial.pt. 44 randomized.ab. 45 placebo.ab. 46 drug therapy.fs. 47 randomly.ab. 48 trial.ab. 49 groups.ab. 50 or/42‐49 51 exp animals/ not humans.sh. 52 50 not 51 53 41 and 52	August 2019: 713 May 2020: 63 August 2021: 79
Embase via Ovid (date of most recent search: 3 August 2021)	1 exp angioneurotic edema/ 2 (angioedema adj3 hereditary).ti,ab. 3 HAE.ti,ab. 4 or/1‐3 5 exp monoclonal antibody/ 6 exp bradykinin B2 receptor antagonist/ 7 exp complement component C1s inhibitor/ 8 exp danazol/ 9 exp kallikrein/ 10 recombinant protein/ 11 exp tranexamic acid/ 12 attenuated androgens.ti,ab. 13 BCX7353.ti,ab. 14 Berinert.ti,ab. 15 bradykinin B2 receptor antagonists.ti,ab. 16 C1 esterase inhibitors.ti,ab. 17 C1INH.ti,ab. 18 C1‐INH.ti,ab. 19 Cinryze*.ti,ab. 20 Complement C1*.ti,ab. 21 concentrated C1 esterase inhibitors.ti,ab. 22 Danazol.ti,ab. 23 Ecallantide.ti,ab. 24 FFP.ti,ab. 25 Firazyr.ti,ab. 26 fresh frozen plasma.ti,ab. 27 Icatibant.ti,ab. 28 Kalbitor.ti,ab. 29 kallikrein inhibit*.ti,ab. 30 Lanadelumab.ti,ab. 31 monoclonal antibody.ti,ab. 32 monoclonal anti‐f XII antibody.ti,ab. 33 nanofiltered C1 inhibitor.ti,ab. 34 rhC1INH.ti,ab. 35 rhC1‐INH.ti,ab. 36 Ruconest.ti,ab. 37 SDP.ti,ab. 38 tranexamic acid.ti,ab. 39 or/5‐38 40 4 and 39 41 randomized controlled trial/ 42 controlled clinical trial/ 43 random$.ti,ab. 44 randomization/ 45 intermethod comparison/ 46 placebo.ti,ab. 47 (compare or compared or comparison).ti. 48 ((evaluated or evaluate or evaluating or assessed or assess) and (compare or compared or comparing or comparison)).ab. 49 (open adj label).ti,ab. 50 ((double or single or doubly or singly) adj (blind or blinded or blindly)).ti,ab. 51 double blind procedure/ 52 parallel group$1.ti,ab. 53 (crossover or cross over).ti,ab. 54 ((assign$ or match or matched or allocation) adj5 (alternate or group$1 or intervention$1 or patient$1 or subject$1 or participant$1)).ti,ab. 55 (assigned or allocated).ti,ab. 56 (controlled adj7 (study or design or trial)).ti,ab. 57 (volunteer or volunteers).ti,ab. 58 trial.ti. 59 or/41‐58 60 40 and 59	August 2019: 1018 May 2020: 173 August 2021: 146
CINAHL (date of most recent search: 3 August 2021)	S53 S37 AND S52 S52 S38 OR S39 OR S40 OR S41 OR S42 OR S43 OR S44 OR S45 OR S46 OR S47 OR S48 OR S49 OR S50 OR S51 S51 MH "Random Assignment" S50 MH "Triple‐Blind Studies" S49 MH "Double‐Blind Studies" S48 MH "Single‐Blind Studies" S47 MH "Crossover Design" S46 MH "Factorial Design" S45 MH "Placebos" S44 MH "Clinical Trials" S43 TX "multi‐centre study" OR "multi‐center study" OR "multicentre study" OR "multicenter study" OR "multi‐site study" S42 TX crossover OR "cross‐over" S41 AB placebo* S40 TX random* S39 TX trial* S38 TX "latin square" S37 S4 AND S36 S36 S5 OR S6 OR S7 OR S8 OR S9 OR S10 OR S11 OR S12 OR S13 OR S14 OR S15 OR S16 OR S17 OR S18 OR S19 OR S20 OR S21 OR S22 OR S23 OR S24 OR S25 OR S26 OR S27 OR S28 OR S29 OR S30 OR S31 OR S32 OR S33 OR S34 OR S35 S35 TX tranexamic acid S34 TX SDP S33 TX Ruconest S32 TX rhC1‐INH S31 TX rhC1INH S30 TX nanofiltered C1 inhibitor S29 TX monoclonal anti‐f XII antibody S28 TX monoclonal antibody S27 TX Lanadelumab S26 TX kallikrein inhibit* S25 TX Kalbitor S24 TX Icatibant S23 TX fresh frozen plasma S22 TX Firazyr S21 TX FFP S20 TX Ecallantide S19 TX Danazol S18 TX concentrated C1 esterase inhibitors S17 TX Complement C1* S16 TX Cinryze* S15 TX C1‐INH S14 TX C1INH S13 TX C1 esterase inhibitors S12 TX bradykinin B2 receptor antagonists S11 TX Berinert S10 TX BCX7353 S9 TX attenuated androgens S8 (MH "Tranexamic Acid") S7 (MH "Recombinant Proteins+") S6 (MH "Danazol") S5 (MH "Antibodies, Monoclonal+") S4 S1 OR S2 OR S3 S3 TX HAE S2 TX angioedema N3 hereditary S1 (MH "Angioedema")	August 2019: 76 May 2020: 8 August 2021: 10
TOTAL before deduplication		August 2019: 2314 May 2020: 361 August 2021: 336
TOTAL after deduplication		August 2019: 1863 May 2020: 299 August 2021: 267