Angioedema
Angioedema | |
---|---|
Other names | Angiooedema, Quincke's edema, angioneurotic edema |
Frequency | ~100,000 per year (US)[1] |
Angioedema is an area of swelling (edema) of the lower layer of skin and tissue just under the skin or mucous membranes.[1][3] The swelling may occur in the face, tongue, larynx, abdomen, or arms and legs.[1] Often it is associated with hives, which are swelling within the upper skin.[1][3] Onset is typically over minutes to hours.[1]
The underlying mechanism typically involves
Treatment to protect the
Signs and symptoms
The skin of the face, normally around the mouth, and the mucosa of the mouth and/or throat, as well as the
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
In hereditary angioedema (HAE), often no direct cause is identifiable, although mild
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.
Pathophysiology
Various mechanisms that interfere with bradykinin production or degradation can lead to angioedema. ACE inhibitors block
The three types of hereditary angioedema are:
- Type I - decreased levels of C1INH (85%);
- Type II - normal levels, but decreased function of C1INH (15%);
- Type III - no detectable abnormality in C1INH, occurs in an X-linked dominant fashion and therefore mainly affects women; it can be exacerbated by pregnancy and use of hormonal contraception (exact frequency uncertain).[6] It has been linked with mutations in the factor XII gene.[7]
Angioedema can be due to
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.[8]
The use of
Diagnosis
The diagnosis is made on the clinical picture. Routine blood tests (
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
Angioedema is classified as either hereditary or acquired.
Acquired angioedema
Acquired angioedema (AAE) can be immunologic, nonimmunologic, or idiopathic.
Hereditary angioedema
Management
Allergic
In allergic angioedema, avoidance of the allergen and use of antihistamines may prevent future attacks.
Drug induction
Hereditary
In hereditary angioedema (HAE), specific stimuli that have previously led to attacks may need to be avoided in the future. 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.[citation needed] The medications ecallantide and icatibant may be used to treat attacks.[1] In 2017 these medications cost between 5,700 and 14,000 US$ per dose in the United States, prices that tripled in two years.[20][medical citation needed] In those given icatibant, specialists monitor is recommended.[21]
Acquired
In acquired angioedema, HAE types I and II, and nonhistaminergic angioedema, antifibrinolytics such as tranexamic acid or ε-aminocaproic acid may be effective. Cinnarizine may also be useful because it blocks the activation of C4 and can be used in patients with liver disease, whereas androgens cannot.[22]
Prophylaxis
Future attacks of HAE can be prevented by the use of androgens such as
In 2018, the U.S. Food and Drug Administration approved lanadelumab, an injectable monoclonal antibody, to prevent attacks of HAE types I and II in people over age 12. 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.[24][25]
Epidemiology
In the U.S., there are as many as 80,000 to 112,000
History
Heinrich Quincke first described the clinical picture of angioedema in 1882,[27] though there had been some earlier descriptions of the condition.[28][29][30]
William Osler remarked in 1888 that some cases may have a hereditary basis; he coined the term "hereditary angio-neurotic edema".[31]
The link with C1 esterase inhibitor deficiency was proved in 1963.[32]
See also
- Drug-induced angioedema
- Gleich's syndrome (unexplained angioedema with high eosinophil counts)
- Ruconest(C1-inhibitor)
References
- ^ PMID 28405953.
- ^ ISBN 9781405103572. Archivedfrom the original on 2017-09-10.
- ^ ISBN 978-0323080378. Archivedfrom the original on 2017-09-10.
- PMID 12956349.
- S2CID 22772933.
- S2CID 30105665.
- PMID 17186468.
- ^ University of Maryland Medical Center. Angioedema. "Page is No Longer Available". Archived from the original on 2007-10-12. Retrieved 2008-01-08.
- PMID 21913206.
- ^ Moon, Amanda T.; Heymann, Warren R. "Acquired Angioedema". MedScape. Archived from the original on 5 September 2015. Retrieved 1 October 2015.
- PMID 18768946.
- ^ Loew, Burr. "A 68-Year-Old Woman With Recurrent Abdominal Pain, Nausea, and Vomiting". MedScape. Archived from the original on 22 October 2012. Retrieved 19 October 2012.
- ^ PMID 9068723.
- ^ S2CID 41952350.
- PMID 16043683.
- S2CID 12030323.
- S2CID 13313000.
- PMID 17225721.
- PMID 15111379.
- ^ "Study: Drug Costs for Rare Hereditary Angioedema Disorder Tripled in Two Years". PR Newswire (Press release). Archived from the original on 2015-10-25.
- ^ "Angioedema". Australasian Society of Clinical Immunology and Allergy (ASCIA). Retrieved 2020-03-24.
- ^ "Archived copy" (PDF). Archived from the original (PDF) on 2007-09-28. Retrieved 2007-01-26.
{{cite web}}
: CS1 maint: archived copy as title (link) - PMID 18158172.
- ^ "FDA Approves Takhzyro (lanadelumab-flyo) for Hereditary Angioedema". Drugs.com.
- ^ "FDA OKs New Prophylactic Drug for Rare Hereditary Angioedema". Medscape.
- ^ "Emergency Medicine, Allergy Physicians Partner to Create New Standards" (July 3, 2014). UC Academic Health Center. University of Cincinnati. http://healthnews.uc.edu/news/?/24791/ Archived 2014-07-14 at the Wayback Machine
- ^ Quincke H (1882). "Über akutes umschriebenes Hautödem". Monatsh Prakt Derm. 1: 129–131.
- Who Named It?
- ^ Marcello Donati. De medica historia mirabili. Mantuae, per Fr. Osanam, 1586
- ^ J. L. Milton. On giant urticaria. Edinburgh Medical Journal, 1876, 22: 513-526.
- PMID 20145434
- PMID 14046003.
External links
- Angioedema at Curlie