Hereditary angioedema is a potential life threatening condition.

It requires earliest possible diagnosis and immediate treatment to save the life in certain cases.

It is caused by either deficiency of C1  inhibitor(Type 1) or dysfunction (Type 2).

It is an autosomal dominant disorder.

In a randomised control trial ,published in JAMA,Lanadelumab,a monoclonal human antibody against Kallikrein was proved to be very effective in reducing the frequency of attacks as well as reducing the severity of individual attack.The level of evidence is level 1,means excellent.

The study was conducted at 41 places in Canada,Jordan,Europe and United states. All the subjects included were previously diagnosed cases of Hereditary angioedema and all were more than 12 years of age.

84 patients were given Lanadelumab and 41 were given placebo.There was a run in period of 4 weeks preceded by 2 weeks of wash out period for any prophylactic treatment being used by patients knowingly or unknowingly. Patients were followed up for a period of 26 weeks for occurence of any episode of angioedema its severity, or any adverse effect of interventional medication.Patients were randomised 2:1 to receive Lanadelumab and placebo. The Lanadelumab receiving patients were divided into 3 groups, and the treatment period was of 26 weeks. The first group received Lanadelumab suncutaneously 150 mg every 4 weeks,second group received 300mg every 4 weeks and the third group 300mg every 2 weeks. The number of episode of angioedema and its severity was observed through a study period of 26 weeks. Adverse events were also observed.

In the group receiving 150mg Lanadelumab subcutaneously every 4 weeks,mean number of attack was 0.48(CI95 0.31-0.73) in comparision to mean number of attack 1.97 in the placebo group(CI95 1.64-2.36) ).In the group receiving 300mg of Lanadelumab every 4 weeks .the mean number of attack was 0.5((CI95 0.36-0.77) ) and for those receiving 300mg of Lanadelumab every 2 weeks the mean number of attack was 0.26(CI95 0.14-0.46) )

Patients receiving Lanadelumab had improved quality of life with reduced number of attacks(p<0.001),less use of anti C1 inhibitor medications( an20.2% vs 65.9%; p < 0.001) and less episode of moderate to severe attacks(p<0.001)

Adverse effects were seen in 98.5% cases and they were mild to moderate, including headace(7.1%),pain at the site of injection(41.7%),erythma at the site of injection(9.5%) and bruising at the site of injection(6%)


Pulmonology Advisor > Trial Tracker > Lanadelumab may be an effective prophylactic treatment for hereditary angioedema

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