Hereditary angioedema: what it is, main symptoms and complications

Before analyzing the characteristics of Icatibant Accord, it is necessary to expose the main characteristics of the pathology against which it has been tested: hereditary angioedema.

It is arare inherited diseasethat affects about 1 in 10,000 people, although it is hypothesized that the real incidence may be even lower (1 in 50,000 people). Those who suffer from it are subject to the appearance ofedema (accumulations of liquids in the interstitial spaces of the body) in the skin, mucous membranes and internal organs. The frequency with which these edemas appear differs from patient to patient, but generally there is the appearance of one edema per month.

Skin edemas tend to be very reddened and are a source of pain, even if the patient does not feel itchy. The most dangerous edemas are those that form in the intestinal mucous membranes andupper respiratory tract. The latter can lead to respiratory crises and even the death of the patient. Edemas of the gastrointestinal tract can lead to severe cramps that are most often mistaken for common attacks ofgastroenteritis orappendicitis.

At the origin of everything there is a mutation of theSERPING1 gene, which codes for the protein C1-INH (orC1-esterase-inhibitor). This protein prevents the release of excessive doses ofbradykinin, a neurotransmitter responsible for vessel permeability. Hereditary angioedema is divided into two types based on the malfunction of the SERPING1 gene:

• Type 1: in patients suffering from it, the C1-INH protein is produced in insufficient quantities;
• Type 2: those suffering from hereditary angioedema type 2 produce sufficient amounts of C1-INH, but the latter is not activated and does not perform its functions;

There is also hereditaryangioedema Type 3, which, however, is not caused by a mutation of the SERPING1 gene but ofcoagulation factor XII. In these cases there is no deficiency of C1-INH, but the abnormal increase inbradykinin.

Icatibant Accord: how it works and posology

Like Firazyr, Icatibant Accord acts as anantagonistof bradykinin. In fact, the active ingredient binds to bradykininB2 receptors, blocking their activity and ensuring patients to mitigate symptoms.

The administration of Icatibant Accord should be done under the close supervision of a doctor or healthcare professional. The active substance is available in the form of a solution in aready- madesyringe , with a dosage of 30mgtags. Theabdomenis the area of the body where it is advisable to proceed with the injection, which must take place under the skin. If the doctor deems it appropriate, the patient himself can proceed with the injection, provided that he has previously been sufficiently instructed.

The injection of a single dose is recommended , but if the symptoms persist, the attending physician can authorize a second or third dose at least 6 hours apart from each other.

Icatibant Accord: side effects and contraindications

From the point of view of side effects, the EMA has specified that they are the same as Firazyr, as the new drug has the same active ingredient. Among them are (Source ):

• Pain at the injection site;
• Erythema;
• Itching;
• Swelling;

The new drug cannot be administered to patients younger than 2 years  of age and pregnant women due to still insufficient data regarding any adverse reactions.

In addition, it is not possible to administer more than three doses of the active substance within 24 hours. Healthcare professionals should also evaluate the dosage for children and adolescents according tobody weight.