In patients with acute hereditary angioedema is treatment with C1 esterase inhibitor better than fresh frozen plasma?

Report By: Katherine Palmer - Medical Student
Search checked by Katherine Palmer - Medical Student
Institution: Manchester Royal Infirmary
Date Submitted: 12th July 2010
Last Modified: 13th July 2010
Status: Blue (submitted but not checked)

Three Part Question

In [patients with acute hereditary angioedema] is [C1 esterase inhibitor] better than [fresh frozen plasma] at [reducing mortality]?

Clinical Scenario

A 50 year old female teacher with known hereditary angioedema presents to the Emergency Department 2 hours after a routine dental extraction with a rash and periorbital oedema.

Search Strategy

MEDLINE 1950 to 2010 June Week 5 and Embase 1980 to 2010 Week 27 using the OVID interface on the world wide web on 12/7/2010
CINAHL using the EBSCOhost on the world wide web on 12/7/2010

MEDLINE and Embase - (exp Hereditary Angioedema Type III/ OR exp Angioedema/ OR exp "Hereditary Angioedema Types I and II"/ OR hereditary angioedema.mp. OR angioedema.mp.) AND (exp Plasma/ OR fresh frozen plasma.mp. OR ffp.mp.) AND (exp Complement C1 Inactivator Proteins/ OR exp Complement C1 Inhibitor Protein/ OR exp Complement C1/ OR c1 esterase inhibitor.mp. OR c1 inhibitor.mp.)
LIMIT to English Language and Humans
CINAHL - (Angioedema) AND (Fresh Frozen Plasma) and (C1 inhibitor)

Search Outcome

MEDLINE - 69 papers, 0 relevant
Embase - 129 papers, 0 relevant
CINAHL - 5 papers, 0 relevant

Comment(s)

In the UK C1 inhibitor is licensed for use in acute hereditary angioedema attacks and despite a lack of clinical trials it is recommended for use over fresh frozen plasma. Fresh frozen plasma carries a small risk of blood bourn disease transmission but C1 inhibitor can cause anaphylaxis in some people.

Clinical Bottom Line

Despite little evidence C1 inhibitor seems to be the treatment of choice for acute hereditary angioedema attacks rather than fresh frozen plasma but more trials need to be carried out.