Acute treatment consists of C1-INH C1-esterase inhibitor concentrate from donor blood, which must be administered intravenously. Dampening or inhibiting bradykinin has been shown quinckd relieve HAE symptoms. Edema develops fully within several hours and disappears in about 3 days. In hereditary angioedemabradykinin formation is caused by continuous activation of the complement system due to a deficiency in one of its prime inhibitors, C1-esterase aka: J Am Dent Assoc. The hereditary form HAE often goes undetected angiiedema a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic.
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Angioedema of half of the tongue Angioedema of the face, most strikingly in the upper lip. The swelling can also occur elsewhere, typically in the hands. The swelling can be itchy or painful. There may also be slightly decreased sensation in the affected areas due to compression of the nerves.
Urticaria hives may develop simultaneously. In severe cases, stridor of the airway occurs, with gasping or wheezy inspiratory breath sounds and decreasing oxygen levels. Tracheal intubation is required in these situations to prevent respiratory arrest and risk of death.
Sometimes, the cause is recent exposure to an allergen e. In hereditary angioedema, often no direct cause is identifiable, although mild trauma , including dental work and other stimuli, can cause attacks.
These stomach attacks can last one to five days on average and can require hospitalization for aggressive pain management and hydration. As the symptoms begin to diminish, the white count slowly begins to decrease, returning to normal when the attack subsides. As the symptoms and diagnostic tests are almost indistinguishable from an acute abdomen e.
HAE may also cause swelling in a variety of other locations, most commonly the limbs, genitals, neck, throat and face. The pain associated with these swellings varies from mildly uncomfortable to agonizing pain, depending on its location and severity.
Predicting where and when the next episode of edema will occur is impossible. Most patients have an average of one episode per month, but there are also patients who have weekly episodes or only one or two episodes per year.
The triggers can vary and include infections, minor injuries, mechanical irritation, operations or stress. In most cases, edema develops over a period of 12—36 hours and then subsides within 2—5 days.
Diagnosis[ edit ] The diagnosis is made on the clinical picture. Routine blood tests complete blood count , electrolytes , kidney function , liver enzymes are typically performed. Mast cell tryptase levels may be elevated if the attack was due to an acute allergic anaphylactic reaction.
When the patient has been stabilized, particular investigations may clarify the exact cause; complement levels, especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor.
The hereditary form HAE often goes undetected for a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic. An important clue is the failure of hereditary angioedema to respond to antihistamines or steroids , a characteristic that distinguishes it from allergic reactions.
It is particularly difficult to diagnose HAE in patients whose episodes are confined to the gastrointestinal tract. Besides a family history of the disease, only a laboratory analysis can provide final confirmation. In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The former is used during the reaction cascade in the complement system of immune defense, which is permanently overactive due to the lack of regulation by C1-INH.
Angioedema is classified as either hereditary or acquired. Acquired angioedema[ edit ] Acquired angioedema AAE can be immunologic, nonimmunologic, or idiopathic. It can also occur as a side effect to certain medications, particularly ACE inhibitors.
It is characterized by repetitive episodes of swelling, frequently of the face, lips, tongue, limbs, and genitals. Edema of the gastrointestinal mucosa typically leads to severe abdominal pain; in the upper respiratory tract, it can be life-threatening.
They are distinguished by the underlying genetic abnormality. All forms of HAE lead to abnormal activation of the complement system , and all forms can cause swelling elsewhere in the body, such as the digestive tract. If HAE involves the larynx , it can cause life-threatening asphyxiation. The end product of this cascade, bradykinin, is produced in large amounts and is believed to be the predominant mediator leading to increased vascular permeability and vasodilation that induces typical angioedema "attacks".
This is most obvious in the face, where the skin has relatively little supporting connective tissue , and edema develops easily.
Bradykinin is released by various cell types in response to numerous different stimuli; it is also a pain mediator. Dampening or inhibiting bradykinin has been shown to relieve HAE symptoms.
Various mechanisms that interfere with bradykinin production or degradation can lead to angioedema. In hereditary angioedema , bradykinin formation is caused by continuous activation of the complement system due to a deficiency in one of its prime inhibitors, C1-esterase aka: C1-inhibitor or C1INH , and continuous production of kallikrein , another process inhibited by C1INH. This serine protease inhibitor serpin normally inhibits the association of C1r and C1s with C1q to prevent the formation of the C1-complex, which - in turn - activates other proteins of the complement system.
Additionally, it inhibits various proteins of the coagulation cascade, although effects of its deficiency on the development of hemorrhage and thrombosis appear to be limited. This acquired angioedema is associated with the development of lymphoma. Consumption of foods that are themselves vasodilators, such as alcoholic beverages or cinnamon , can increase the probability of an angioedema episode in susceptible patients. If the episode occurs at all after the consumption of these foods, its onset may be delayed overnight or by some hours, making the correlation with their consumption somewhat difficult.
In contrast, consumption of bromelain in combination with turmeric may be beneficial in reducing symptoms. The use of acetaminophen typically has a smaller, but still present, increase in the probability of an episode. Allergic[ edit ] In allergic angioedema, avoidance of the allergen and use of antihistamines may prevent future attacks.
Cetirizine is a commonly prescribed antihistamine for angioedema. Some patients have reported success with the combination of a nightly low dose of cetirizine to moderate the frequency and severity of attacks, followed by a much higher dose when an attack does appear. Severe angioedema cases may require desensitization to the putative allergen, as mortality can occur. Chronic cases require steroid therapy, which generally leads to a good response.
In cases where allergic attack is progressing towards airway obstruction, epinephrine may be life-saving. Drug induction[ edit ] ACE inhibitors can induce angioedema. However, this is controversial, as small studies have shown some patients with ACE inhibitor angioedema can develop it with ARBs, as well.
It does not respond to antihistamines, corticosteroids, or epinephrine. Acute treatment consists of C1-INH C1-esterase inhibitor concentrate from donor blood, which must be administered intravenously. In an emergency, fresh frozen blood plasma, which also contains C1-INH, can also be used.
However, in most European countries, C1-INH concentrate is only available to patients who are participating in special programmes. Cinnarizine may also be useful because it blocks the activation of C4 and can be used in patients with liver disease, whereas androgens cannot. These agents increase the level of aminopeptidase P, an enzyme that inactivates kinins ;  kinins especially bradykinin are responsible for the manifestations of angioedema.
In , the U. Food and Drug Administration approved lanadelumab , an injectable monoclonal antibody , to prevent attacks of HAE types I and II in people over age Lanadelumab inhibits the plasma enzyme kallikrein , which liberates the kinins bradykinin and kallidin from their kininogen precursors and is produced in excess in individuals with HAE types I and II.
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