ACE Inhibitor
Class of medicines used to treat high blood pressure and other diseases.
Activating factor
A functioning plasma protein that works in the coagulation pathway to help form a clot.
Anabolic steroid
Also referred to as attenuated androgen, a class of steroid hormones used to prevent HAE attacks. Although their mechanism of action in HAE patients is unknown, these agents are believed to partially reverse the biochemical defect by increasing levels of C1-esterase inhibitor (C1-INH), helping to prevent angioedema attacks in patients with inherited C1-INH deficiency.
Antifibrolytic agent
Medicines used to prevent HAE attacks. These agents inhibit the activation of plasmin, a chemical in the blood that can make the C1-INH less effective.
Attenuated androgen
Also referred to as anabolic steroid, a class of steroid hormones used to prevent HAE attacks. Although their mechanism of action in HAE patients is unknown, these agents are believed to partially reverse the biochemical defect by increasing levels of C1-esterase inhibitor (C1-INH), helping to prevent angioedema attacks in patients with inherited C1-INH deficiency.
Bradykinin
A protein, which when released, causes blood vessels to expand (dilate) and leak fluid, such as serum, into surrounding tissue.
C1 inhibitor
Also referred to as C1-INH, a protein found in the fluid part of your blood that controls C1, a component of your body's complement system. Abnormal swelling can result when C1-INH is insufficient or defective.
C1-INH
Also referred to as C1 inhibitor, a protein found in the fluid part of your blood that controls C1, a component of your body's complement system. Abnormal swelling can result when C1-INH is insufficient or defective.
Cleave
To cut; to split
Coagulation pathway
A process in which blood cells work together to form a clot.
Complement system
The complement system is made up of proteins like C1 that float in your bloodstream, which work with the immune system to fight organisms like bacteria, viruses and parasites.
Drug formulary
A list of prescription medications selected for coverage under a health insurance plan.
Edema
Swelling caused by an excessive amount of fluid in the body tissues.
Endothelial cells
A thin layer of cells that line the surface of blood vessels.
Enzyme
A protein that changes the rate of chemical reactions in the body.
HAE
HAE, or Hereditary Angioedema, is a rare, hereditary disease of the immune system that affects an estimated 1 in 10,000 to 1 in 50,000 adults and children globally. HAE causes attacks of spontaneous swelling that are often painful and severe, and can be life-threatening.
Hereditary Angioedema
A rare, hereditary disease of the immune system that affects an estimated 1 in 10,000 to 1 in 50,000 adults and children globally. Hereditary Angioedema (HAE) causes attacks of spontaneous swelling that are often painful and severe, and can be life-threatening.
High-molecular-weight kininogen
HMWK, a protein that acts on the inflammation and coagulation processes; inactive until it is cleaved by kallikrein and in turn, releases bradykinin.
Hypotension
Low blood pressure.
Inflammatory pathway
A process in which the body responds to an injury either inside or outside of the body.
Inhibitor
That which reduces, prevents or stops; i.e. a substance that stops enzyme activity.
Kallikrein–kinin system
A system of proteins in the blood that has a role in the inflammatory pathway; through this system, bradykinin is released.
Kinin
A protein that is a component of the kallikrein-kinin system.
Laryngeal attack
An HAE attack that affects the larynx (throat); this can block or close the airway, leading to difficulty breathing and, potentially, death.
Liberate
To release
Plasma kallikrein
An enzyme present in blood plasma, urine and tissue; responsible for cleaving high-molecular-weight kininogen (HMWK), which then releases bradykinin.
Prekallikrein
Precursor to kallikrein; inactive until it cleaved by activated factor (specifically, activated factor XII) to generate active kallikrein.
Preventive therapy
In HAE, medicine taken to prevent attacks.
Prodrome
One or more physical warnings of an HAE attack; can appear as a rash or a feeling of skin tightness.
Protease
An enzyme that breaks proteins down into a simpler form.
Proteolytic cascade
A step-by-step process in which proteins are broken down into simpler forms.
Receptor
A component in a cell that can combine with a drug or a chemical in the body (i.e. hormone) to change the function of the cell.
Serum
The clear, watery, fluid-component of blood that resembles fluid in a blister.
Subcutaneous
Beneath the skin.
Substrate
A molecule or substance acted upon by an enzyme.
Trigger
In HAE, something that causes an attack. Stress and trauma are two common HAE attack triggers.
Vascular permeability
The capacity of blood vessels to allow fluids or molecules to pass in and out of the vessel.
Vasodilator
A substance that opens, or dilates, blood vessels; e.g. bradykinin and nitric oxide.
Header: About HAE
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Types of HAE

There are several types of HAE. Two main types of HAE involve insufficient or defective C1-inhibitor (C1-INH) protein, which helps to regulate the flow of body fluids in and out of cells.  This regulation plays a key role in HAE attacks.

Two Main Types of HAE

  • Type I HAE: Most patients (approximately 85%) have Type I HAE, which results from insufficient production of the C1-inhibitor (C1-INH) protein.
  • Type II HAE: A minority of patients have this type of HAE, which is caused by production of defective or dysfunctional C1-inhibitor (C1-INH) protein.

Type III HAE

A third type of HAE, sometimes referred to as Type III, is extremely rare and occurs only in women. Clinically, symptoms of Type III HAE are the same as those of Type I and Type II; however, C1-inhibitor (C1-INH) activity and levels are normal in these patients. A large proportion of Type III HAE occurs in women on oral contraceptives or hormone replacement therapy, and may be linked to the estrogen component of hormonal therapies.

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