• Angioedema is a rare problem with episodes of swelling that occurs mostly in the hands and feet, face, genitals and throat
  • The swelling is more raised than hives, less red, and firm
  • Angioedema may come and go over a longer period than hives and last for up to 3 days
  • Angioedema is not itchy but may be painful
  • If angioedema occurs along with an itchy, red or pink rash (“urticaria,” hives, “bommels” or wheals) that has a round or wavy border and comes and goes over minutes to hours leaving no trace on the skin behind, it is often caused by an allergy (see urticaria)
  • However if angioedema occurs on its own, without urticaria, it is either caused by specific medication (ACE inhibitors) or because of a genetic problem called hereditary angioedema
  • Angioedema affects only very few patients taking ACE inhibitors
  • It can occur with the first time taking the drug or only after many years
  • Swelling can be very severe and may be life-threatening
  • It often involves the face, lips and eyes. Tongue and throat swelling can cause severe breathing difficulty
  • If this reaction occurs, the ACE inhibitor must be stopped immediately. Even if the angioedema goes away quickly, it may recur, so all patients should be admitted overnight
  • All types of ACE inhibitors must be avoided, as the reaction is caused by all of the related drugs
  • A “cousin” of ACE inhibitors, the “angiotensin II receptor antagonists” are almost always safe to use
  • Patients should wear a medic alert bracelet
  • Patients should be screened for underlying hereditary angioedema as ACE reactions are more common in this group
  • HAE is a genetic problem that runs in families
  • Patients have attacks of swelling that may affect any part of the body
  • It is not associated with hives but some patients may have a soft pink non-itchy rash before the angioedema starts
  • Swelling may occur in the lining of the gut, causing severe stomach pain and vomiting that is often mistaken for appendicitis or other problems
  • Swelling of the throat can cause difficult breathing and death
  • Within a single family some people can have mild attacks and others very severe attacks
  • HAE is called hereditary because the genetic problem is passed on in families. A child has a 50 percent chance of inheriting this disease if one of his or her parents has it
  • HAE can also occur for the first time in a person. Up to 20% of HAE are “new cases” without it coming from someone else in the family. These people can still pass the abnormal gene to their children
  • HAE patients have a problem in the gene that controls a blood protein called C1 Inhibitor. The genetic problem causes low levels of C1-Inhibitor protein or a C1-Inhibitor protein that doesn’t work properly
  • Normal C1-Inhibitor helps to keep blood within our blood vessels. If the inhibitor is missing, blood leaks into the surrounding tissue causing angioedema
  • Because the disease is very rare, patients often remain undiagnosed for many years
  • There are blood tests for HAE. If you have HAE, all members in the family should have the blood test to see whether they are at risk, even if they have never had any attacks
  • The swelling of HAE can occur quickly and cause death. At the first sign of an attack, the progression of the swelling must be stopped particularly if the swelling is in the airway
  • The only treatment widely available in South Africa is a blood product called “fresh frozen plasma” (FFP)
  • The treatment usually given for hives is NOT effective
  • Other treatment includes pain relief for abdominal attacks, medication to prevent vomiting and extra fluids
  • Other very effective medications are unfortunately not yet available in South Africa.

Short-term prevention

  • Before surgery or dental work, short term treatment is needed to avoid the risk of any attacks
  • Fresh frozen plasma may be used 1-12 hours before the surgery
  • In adults an oral medication (of “high-dose androgens”) may be given for the previous 5–10 days

Long-term prevention

  • Patients in whom episodes occur at least once a month or who are at high risk of developing throat attacks need long-term prevention
  • Monthly injections of fresh frozen plasma may be used
  • Oral “androgen” hormone medications increase production of C1-INH in the liver but have some side effects particularly in women and children and they must not be taken during pregnancy
  • As an alternative, large doses of a medication called “tranexamic acid” or “aminocaproic acid” can be used. The effect is weaker than androgens but side effects are less severe
  • If angioedema occurs on its own, without urticaria, it is usually caused by ACE inhibitors or hereditary angioedema
  • The correct diagnosis must be established
  • Your doctor will complete an application form for a Medic-Alert bracelet and an action plan detailing emergency treatment
  • Patients with attacks must seek emergency help immediately
  • Emergency treatment for HAE is fresh frozen plasma given immediately into the veins

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