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Angioedema

RAE

Angioedema is the swelling of skin, subcutaneous, or submucosal tissue due to acquired or genetic causes.

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Presentation

In hereditary angioedema, the patients present with swelling that develops 2 to 5 hours after any stimulus such as a minor trauma or infection. The swelling can involve any part of body and is often associated with rashes. The patients frequently experience abdominal symptoms such as nausea, vomiting, abdominal pain and diarrhea. The patients usually have one episode of attack per month. Some patient may have a higher frequency of attacks Attacks usually last from 12 to 48 hours.

In acquired angioedema, the predominant feature of angioedema is swelling of the face, mucous membranes and hands. Swelling may be itchy and associated with skin rash. Sensations of the skin can be decreased due to extravasation of fluid that causes compression of the nerves. If the swelling of the mucus membranes of the upper respiratory tract is severe, respiratory distress along with cough and wheezing may occur [9][10]. The patient is unable to breathe which leads to rapid decrease in the oxygen concentration in the arterial blood. If this is not treated promptly, permanent brain damage may occur.

Stridor
  • Hydromorphone was administered with subsequent rapid development of stridor and edematous changes of the tongue, uvula, and surrounding mucosa.[ncbi.nlm.nih.gov]
  • Potential warning signs are voice changes; a harsh vibrating noise when breathing ( stridor ); tongue or throat swelling; sudden, severe difficulty breathing; and a fainting sensation or collapse.[jamanetwork.com]
  • If your throat is affected, a physical exam might reveal abnormal sounds ( stridor ) when you breathe in. Blood tests or allergy testing may be ordered. Treatment Mild symptoms may not need treatment.[ufhealth.org]
  • If your throat is affected, a physical exam might reveal abnormal sounds ( stridor ) when you breathe in. Blood tests or allergy testing may be ordered. Mild symptoms may not need treatment. Moderate to severe symptoms may need to be treated.[medlineplus.gov]
Hoarseness
  • Red flags Any patient you suspect clinically to have airway compromise IMPENDING AIRWAY DISASTER TRIAD Rapid onset aphagia or severe dysphagia, frequently associated with a severe sore throat Rapid onset laryngeal voice change: hoarse, croaky, husky or[entsho.com]
  • […] asymmetric swelling of the lips and face tongue, the floor of the mouth, neck, and eyelids may affect extremities, genitalia or viscera (e.g. intensitines) Features of significant airway involvement: Dyspnea, dysphagia, dysphonia, odynophagia, stridor, hoarseness[lifeinthefastlane.com]
  • In rare cases, people with angioedema may experience a swollen throat, hoarseness, and difficulty breathing. Angioedema may or may not itch. Call 911 or go to an emergency room right away if you’re having trouble breathing.[healthline.com]
Dyspnea
  • On admission, there was no pharyngeal or laryngeal edema and no dyspnea. Treatment with glucocorticoids and antihistamines had no response.[ncbi.nlm.nih.gov]
  • […] isolated or co-exist with urticaria areas affected asymmetric swelling of the lips and face tongue, the floor of the mouth, neck, and eyelids may affect extremities, genitalia or viscera (e.g. intensitines) Features of significant airway involvement: Dyspnea[lifeinthefastlane.com]
  • Up to 20% of patients experience dyspnea, dysphagia, dysphonia, and stridor, which may progress rapidly to life-threatening airway obstruction unless treated.[americannursetoday.com]
  • One of these patients also required a tracheotomy for dyspnea that was classified as a serious adverse event (see below).[nejm.org]
Respiratory Distress
  • If the swelling of the mucus membranes of the upper respiratory tract is severe, respiratory distress along with cough and wheezing may occur.[symptoma.com]
  • Stridor or any respiratory distress should prompt a consideration of intubation.[epmonthly.com]
  • There was no tongue swelling, stridor or respiratory distress, and no airway obstruction on nasolaryngoscopy. He was transferred to the intensive care unit for monitoring.[nature.com]
  • The patient suddenly developed increasing stridor and respiratory distress, necessitating tracheal intubation. The nasal tracheal route was chosen, and a 7 F tracheal tube was used.[journals.lww.com]
  • Angioedema of the GI tract can cause severe abdominal pain, while angioedema of the larynx or tongue can lead to respiratory distress. Urticaria: Acute vs.[theasthmacenter.org]
Pleural Effusion
  • Abdominal computed tomography – This may show severe edema of the bowel wall [1] Chest radiography – This may show pleural effusion Soft-tissue neck radiography – This may show soft-tissue swelling [ 5 ] See Workup for more detail.[emedicine.medscape.com]
  • Free fluid may be detected in serous cavities ( Figure 3 ) and, if severe, detectable as ascites or pleural effusion. Figure 3.[priory.com]
Swelling
  • In acquired angioedema, the predominant feature of angioedema is swelling of the face, mucous membranes and hands. Swelling may be itchy and associated with skin rash.[symptoma.com]
  • After the ACEI is discontinued, swelling may remain if there is continued pressure from the maxillary and mandibular incisors on the dorsal and lingual surfaces of the tongue.[ncbi.nlm.nih.gov]
  • Most ACE inhibitor-induced angioedema cases describe swelling in the periorbital region, tongue, and pharynx.[ncbi.nlm.nih.gov]
  • Nevertheless, swelling was progressive and required intubation. Even after the second specific treatment with icatibant, her angioedema subsided extremely slowly.[ncbi.nlm.nih.gov]
  • […] 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 .[en.wikipedia.org]
Pain
  • A 38-year-old Chinese woman presented with generalized gastric pain in the following day after renal transplantation. The patient began to crampy abdominal pain accompanied by nausea, vomiting, and diarrhea on postoperative day 6 (POD).[ncbi.nlm.nih.gov]
  • The patient presented with nausea and intermittent left middle and upper quadrant abdominal pain and denied history of angioedema or swelling with any medications or any history of abdominal pain.[ncbi.nlm.nih.gov]
  • Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting.[ncbi.nlm.nih.gov]
  • The most frequent jellyfish in Southern Brazil causes mainly local pain and skin plaques.[ncbi.nlm.nih.gov]
  • 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.[en.wikipedia.org]
Fatigue
  • […] dermatitis , an allergic reaction in which the swelling is red and itchy and typically accompanied by eczema Dermatomyositis , an autoimmune disorder of the skin and skeleton in which the swelling can persist for months and be accompanied by weight loss, fatigue[allergies.about.com]
  • […] possibly associated with an increased incidence of autoimmune disease Prognosis variable after the attack occurs, they may recur throughout the patient's life attack frequency can be reduced with appropriate therapy Presentation Symptoms prodromal symptoms fatigue[step2.medbullets.com]
  • These may include: Extreme fatigue Muscle ache Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
  • Many patients have prodromal symptoms before the onset of angioedema, and these symptoms include fatigue, myalgia, arthralgia, and nonpruritic erythematous rashes such as erythema marginatum ( Figure 1 ) (Agostini et al 2004). Figure 1.[priory.com]
Localized Edema
  • A general term for a vascular reaction of the deep dermis, subcutaneous or submucosal tissues, which corresponds to localized edema 2º to vasodilation and capillary permeability. an·gi·o·e·de·ma ( an'jē-ō-ĕ-dē'mă ) 1.[medical-dictionary.thefreedictionary.com]
  • (NICHD) Definition (MSH) Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.[fpnotebook.com]
Collapse
  • Dial 999 for an ambulance if you, or someone with you, has swelling and: sudden or worsening breathing problems feels faint or dizzy passes out or collapses These are signs of a serious allergic reaction (anaphylaxis) .[nhs.uk]
  • Potential warning signs are voice changes; a harsh vibrating noise when breathing ( stridor ); tongue or throat swelling; sudden, severe difficulty breathing; and a fainting sensation or collapse.[jamanetwork.com]
  • Other symptoms may be dangerous, such as low blood pressure, severe breathing problems, and collapse. Anaphylaxis is rare but is the most serious type of allergic reaction and can be fatal unless promptly treated.[patient.info]
Abdominal Pain
  • The patient presented with nausea and intermittent left middle and upper quadrant abdominal pain and denied history of angioedema or swelling with any medications or any history of abdominal pain.[ncbi.nlm.nih.gov]
  • Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting.[ncbi.nlm.nih.gov]
  • The patient began to crampy abdominal pain accompanied by nausea, vomiting, and diarrhea on postoperative day 6 (POD). We strongly suspected the angioedema was an adverse reaction to SRL.[ncbi.nlm.nih.gov]
  • Symptoms in the larynx and throat, as well as non-specific symptoms, such as dizziness and abdominal pain, were more frequent than previously reported.[medicaljournals.se]
Vomiting
  • Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting.[ncbi.nlm.nih.gov]
  • The patient began to crampy abdominal pain accompanied by nausea, vomiting, and diarrhea on postoperative day 6 (POD). We strongly suspected the angioedema was an adverse reaction to SRL.[ncbi.nlm.nih.gov]
  • In the C1 INH test normal group angioedema was still active at the time of the study in 53% of the patients, and 36% reported symptoms in the throat, 23% in the abdominal area, 17% had diarrhoea, 11% had vomiting and 6% fainted during attacks.[medicaljournals.se]
  • Patients often present with non-specific findings of abdominal pain, nausea, and vomiting.[radiopaedia.org]
  • The patients frequently experience abdominal symptoms such as nausea, vomiting, abdominal pain and diarrhea. The patients usually have one episode of attack per month.[symptoma.com]
Nausea
  • Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting.[ncbi.nlm.nih.gov]
  • The patient began to crampy abdominal pain accompanied by nausea, vomiting, and diarrhea on postoperative day 6 (POD). We strongly suspected the angioedema was an adverse reaction to SRL.[ncbi.nlm.nih.gov]
  • The patient presented with nausea and intermittent left middle and upper quadrant abdominal pain and denied history of angioedema or swelling with any medications or any history of abdominal pain.[ncbi.nlm.nih.gov]
  • […] urticaria or pruritus affecting the skin swelling of the extremities, face, and genitals can affect any area upper airway swelling of the lips, tongue, uvula, soft palate, and larynx gastrointestinal bowel wall edema resulting in gastrointestinal colic, nausea[step2.medbullets.com]
  • Patients often present with non-specific findings of abdominal pain, nausea, and vomiting.[radiopaedia.org]
Diarrhea
  • The patient began to crampy abdominal pain accompanied by nausea, vomiting, and diarrhea on postoperative day 6 (POD). We strongly suspected the angioedema was an adverse reaction to SRL.[ncbi.nlm.nih.gov]
  • Is severe diarrhea sign of angioedema? I have sudden onset of painful diarrhea with stomach pain. A.[medical-dictionary.thefreedictionary.com]
  • , or bloody diarrhea Other: fatigue or feeling tired, sore throat, dizziness, or lightheadedness From Reviewed on 9/11/2017 Sources: Patient Comments & Reviews CONTINUE SCROLLING FOR RELATED ARTICLE[emedicinehealth.com]
  • Swelling in your intestine can cause: Extreme pain Nausea Vomiting Diarrhea You may notice warning signs before swelling begins.[webmd.com]
Abdominal Cramps
  • Rarer cases will also experience abdominal cramping and breathing difficulty due to the amount of swelling. Is Angioedema Dangerous? Angioedema can be deadly if your throat swells up so much that you can no longer breath.[hives.org]
  • Other symptoms may include: Abdominal cramping Breathing difficulty Swollen eyes and mouth Swollen lining of the eyes ( chemosis ) The health care provider will look at your skin and ask if you have been exposed to any irritating substances.[medlineplus.gov]
  • Abdominal cramps, swollen eyes or mouth and swelling near the lining of the eyes are also common. Since this is an allergic reaction, you might have trouble breathing or feel like your throat is closing.[gazettereview.com]
Lip Swelling
  • Six months after the accident he developed acute onset bilateral facial swelling, lip swelling, and difficulty breathing.[hoajonline.com]
  • On post-transplant day 12, he developed upper and lower lip swelling ( Figure 1 ). There was no tongue swelling, stridor or respiratory distress, and no airway obstruction on nasolaryngoscopy.[nature.com]
  • swelling sometimes associated with inflammation of the bowel); subcutaneous emphysema (leakage of air into the soft tissues, often occurring after trauma or surgery); cluster headache (severe one-sided headache associated with puffiness around the eye[allergy.org.au]
  • It has also been associated with URTI and often a lip swelling closely predates a cold sore eruption. Minor trauma has triggered peripheral swellings. These swellings burn rather than itch and are deep to the skin.[stemlynsblog.org]
  • swelling and eventually associated swelling of the tongue and larynx.[scielo.br]
Edema of the Tongue
  • The edema resolved, the tongue returned to normal size, and the patient was extubated the next day. There has been no return of symptoms after discontinuation of the ACE inhibitor.[nejm.org]
Hypotension
  • After he received intravenous alteplase infusion, he developed orolingual angioedema followed by transient bradycardia with subsequent hypotension, resulting in the deterioration of his neurological signs and expansion of the ischemic lesion.[ncbi.nlm.nih.gov]
  • Physical examination revealed a swollen tongue and no hypotension, rash, bronchospasm, urticaria, or flushing.[nejm.org]
  • Alternatively, the shifting of intravascular fluid into the tissues may result in hypotension and tachycardia regardless of the AE mechanism.[airwayelearning.com]
  • The most common adverse reactions to ACE inhibitors are cough, hypotension, and dizziness. Hyperkalemia and functional renal failure arise less often. An even rarer but potentially much more serious adverse reaction is angioedema.[americannursetoday.com]
  • Exercise-induced anaphylaxis was first described in a series of patients in whom combinations of pruritus, urticaria, angioedema, wheezing, and hypotension occurred as a result of exercise.[worldallergy.org]
Tachycardia
  • […] have airway compromise IMPENDING AIRWAY DISASTER TRIAD Rapid onset aphagia or severe dysphagia, frequently associated with a severe sore throat Rapid onset laryngeal voice change: hoarse, croaky, husky or no voice Systemically very unwell: pyrexia, tachycardia[entsho.com]
  • Alternatively, the shifting of intravascular fluid into the tissues may result in hypotension and tachycardia regardless of the AE mechanism.[airwayelearning.com]
Chest Pain
  • It also affects mouth, throat and stomach and cause internal swelling resulting in severe stomach ache, difficulty breathing or chest pain. Common allergens like pollen, animal dander, latex and insect stings.[medindia.net]
  • Internal swelling is relatively common, resulting in presentation to hospital with severe stomach or chest pains without external swelling at the same time.[allergy.org.au]
Myalgia
  • Cold exposure causes a burning rash, fever, chills, arthralgia, myalgia, headache, and leukocytosis. Hereditary vibratory angioedema This reaction consists of pruritus and swelling minutes after a vibratory stimulus.[theasthmacenter.org]
  • Many patients have prodromal symptoms before the onset of angioedema, and these symptoms include fatigue, myalgia, arthralgia, and nonpruritic erythematous rashes such as erythema marginatum ( Figure 1 ) (Agostini et al 2004). Figure 1.[priory.com]
Swelling of Hand
  • Rarer cases will also get swelling on hands, feet and throat. The swelling may also itch and is sometimes accompanied by a burning sensation and pain. There may or may not be red or skin colored welts / hives that appear in the area.[hives.org]
  • Epinephrine is not needed for swelling of hands, feet, genitalia, lips, or eyes, but can be helpful for tongue swelling or pharyngeal swelling, and is critical should laryngeal edema be present.[link.springer.com]
Angioedema
  • In patients with F12 gene mutation, the diagnosis is done by excluding other types of angioedema. The treatment of angioedema depends upon the etiology. Hereditary angioedema: The patient should avoid specific stimuli.[symptoma.com]
  • All data were derived from EHR sources, with angioedema defined by EHR reactions of angioedema, swelling, edema, or lip, eye, face, tongue, throat or mouth swelling.[ncbi.nlm.nih.gov]
  • Angioedema is still best classified by whether it is likely histaminergic or kinin-mediated. New guidelines have been published around the world to help diagnose and treat both forms (urticaria/angioedema and hereditary angioedema).[ncbi.nlm.nih.gov]
  • Classification and Subtypes of Angioedema Angioedema is a highly heterogeneous group of conditions.[emedicine.medscape.com]
  • Common treatments for angioedema, such as antihistamines and glucocorticoids, have not been shown to be effective in ACEI-induced angioedema.[ncbi.nlm.nih.gov]
Urticaria
  • Chronic urticaria comprises both chronic spontaneous urticaria (CSU) and chronic inducible urticaria (CIndU) that includes physical and non-physical urticarias.[ncbi.nlm.nih.gov]
  • The presence or absence of urticaria should always be considered and determined. When angioedema is associated with urticaria, the diagnosis and treatment approach will follow the intervention for urticaria.[emedicine.medscape.com]
  • Many drugs, mainly antibiotics and nonsteroidal anti-inflammatory drugs, can cause angioedema-urticaria. We present the case of angioedema-urticaria due to acitretin, confirmed by oral provocation test, in a 61-year-old man with psoriasis.[ncbi.nlm.nih.gov]
  • Angioedema (whether occurring with or without urticaria) is common in the pediatric population.[ncbi.nlm.nih.gov]
  • Treatment of physical urticaria/angioedema includes high-dose antihistamine prophylaxis, except for delayed pressure urticaria.[accessmedicine.mhmedical.com]
Eruptions
  • Angioedema, NOS , Angioneurotic edema, NOS , angioneurotic edema , angioneurotic oedema , Edema;angioneurotic French URTICAIRE GEANTE , Angio-oedème , Urticaires géantes , Oedème de Qincke , Oedème angionévrotique , Angioedème , Oedème de Quincke , ERUPTION[fpnotebook.com]
  • Patients who develop acute urticarial eruptions can have other accompanying manifestations of a systemic anaphylactic reaction such as wheezing, laryngeal edema, cramps, diarrhea, and hypotension.[worldallergy.org]
  • On DermNet NZ Food allergy Urticaria - an overview Acute urticaria Chronic urticaria Contact urticaria Cholinergic urticaria Cold urticaria Dermographism Drug eruptions Complement deficiencies Allergies explained Exercise-induced anaphylaxis Other websites[dermnetnz.org]
Facial Swelling
  • Female patient, 35 years old, married, doctor, reported crisis of intense facial swelling for nine years, always attacking the lips.[scielo.br]
  • Six months after the accident he developed acute onset bilateral facial swelling, lip swelling, and difficulty breathing.[hoajonline.com]
  • On day 10 post transplantation, she developed a pruritic skin rash on the upper and lower extremities, back and neck, followed by eyelid, lip and facial swelling. There was no tongue swelling or airway obstruction.[nature.com]
  • Take it away Andy…… The Case It’s 8pm and a 35 year old female presents to the ED with unilateral facial swelling.[stemlynsblog.org]
  • Other causes of facial swelling or airway obstruction - eg, epiglottitis (can occur in adults), peritonsillar abscess or retropharyngeal abscess . Other causes of an acute abdomen.[patient.info]
Facial Edema
  • Here we report a case who developed oral-facial edema four years after using lisinopril/hydrochlorothiazide. Laryngeal oedema is a main cause of death in angioedema.[ncbi.nlm.nih.gov]
Urinary Retention
  • However, rarer clinical manifestations, including intense headaches caused by brain edema, urinary retention, and acute pancreatitis, can also occur. 12,35 The incidence and severity of the clinical manifestations of HAE vary among individuals.[scielo.br]
Irritability
  • The best way to prevent angioedema from occurring is to avoid irritating the area where the angioedema has occurred.[gazettereview.com]
  • They turn pale and swell if irritated. The deeper swelling of angioedema may also be painful.[ufhealth.org]
Headache
  • If you find yourself feeling drowsy, you should avoid: driving drinking alcohol operating complex or heavy machinery Other side effects of the newer antihistamines include: headache dry mouth dry nose These side effects are usually short-lasting and should[hse.ie]
  • The adverse reactions reported include headache, rash, and, rarely, hypersensitivity reactions.[priory.com]
  • (severe one-sided headache associated with puffiness around the eye on the same side).[allergy.org.au]
  • These may include: Extreme fatigue Muscle ache Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
Tingling
  • The rash is characterized by red rings that appear on the trunk and appendages as part of a prodrome that may also include a tingling sensation in the area where swelling will occur. 7 Reproduced with permission from the US Hereditary Angioedema Association[jaoa.org]
  • It is neither itchy nor red but can often cause a tingling, numb, or burning sensation. Angioedema may last several hours or days. When the swelling finally stops, the skin will usually appear normal with no flaking, peeling, scarring, or bruising.[allergies.about.com]
  • You may also experience skin tingling or tightness.[health.ucsd.edu]
  • These may include: Extreme fatigue Muscle ache Tingling Headache Belly pain Hoarseness Mood changes Triggers Although you may not be able to tell what your triggers are, common ones include: Stress or anxiety Minor injury or surgery Illnesses such as[webmd.com]
Seizure
  • To help address this question, the Observational Health Data Sciences and Informatics research network conducted a retrospective observational new-user cohort study of seizure patients exposed to levetiracetam (n 276,665) across 10 databases.[ncbi.nlm.nih.gov]

Workup

The diagnosis of angioedema can often be made on clinical grounds. Certain investigations are helpful in confirming the diagnosis and etiology of this disease. Mast cell tryptase levels are raised in the patients suffering from angioedema. However, unlike other allergic conditions, there is no response to therapy with antihistaminic and steroidal drugs.

In hereditary angioedema, there is often a typical family history. In addition, the deficiency of C2 and C4 component of the complement system is present. In patients with F12 gene mutation, the diagnosis is done by excluding other types of angioedema.

Pleural Effusion
  • Abdominal computed tomography – This may show severe edema of the bowel wall [1] Chest radiography – This may show pleural effusion Soft-tissue neck radiography – This may show soft-tissue swelling [ 5 ] See Workup for more detail.[emedicine.medscape.com]
  • Free fluid may be detected in serous cavities ( Figure 3 ) and, if severe, detectable as ascites or pleural effusion. Figure 3.[priory.com]

Treatment

The treatment of angioedema depends upon the etiology.

Hereditary angioedema:

  • The patient should avoid specific stimuli.
  • C1-INH concentrated blood should be transfused.
  • Fresh frozen plasma containing C1-INH may be used.
  • Androgens such as danazol which can increase aminopeptidase-P are given. Aminopeptidases-P inhibits bradykinin.

ACE-inhibitors angioedema:

  • The use of the ACE inhibitor drug should be discontinued.
  • Alternative drugs such as Angiotensin Receptor Blockers (ARBs) are given.

Allergic angioedema:

  • Exposure to the allergen should be avoided. If possible, desensitization should be done.
  • High dose cetirizine in given in acute attacks.
  • Steroids may be used.
  • Adrenaline may be used if respiratory distress occurs.

Acquired angioedema:

  • Antifibrinolytics such as tranexamic acid or ε-aminocaproic acid are used in this case.
  • Cinnarizine can also be used.

Burn angioedema:

  • Endotracheal tube should be passed prophylactically as soon as the patient is received as inhalation burns can lead to swelling obstruction of the airway. If obstruction has developed, endotracheal tube can not be passed.
  • If angioedema develops and endotracheal tube has not yet been passed, emergency cricothyriodectomy or mini-tracheostomy is necessary.

Prognosis

Early recognition of angioedema and aggressive treatment results in a very good prognosis. Early intubation with endotracheal tube lead also improves the prognosis by limiting the respiratory distress. If the treatment is delayed, it may even prove fatal for the patient.

Etiology

Hereditary angioedema: Occurs due to an autosomal dominant gene mutation. The genes include the SERPING1 gene and F12 gene. Both of these genes lead to pathological activation of complement pathway.

Acquired angioedema: Acquired angioedema can result from the following causes:

  • Immunological
  • Allergy to certain allergens such as peanuts and food colors [1]
  • Reaction to medications such as ACE inhibitors
  • Burn
  • Idiopathic

Epidemiology

  • Allergic angioedema is most common in the United States.
  • Drug-induced angioedema is more common in African-American.
  • Annually 80000 to 112000 patients suffering from angioedema visit emergency department.
Sex distribution
Age distribution

Pathophysiology

Angioedema is the swelling of subcutaneous tissues and mucosa due to accumulation of fluid [2] [3]. It results from various stimuli that cause the release of bradykinin [4] [5]. In addition to being a potent vasodilator, bradykinin also facilitates the extravasation of fluid from the blood vessels. Intravascular fluid therefore starts accumulating extravascularly leading to the swelling. The skin around the eyes is the loosest skin of the body; therefore, the fluid accumulate there first and later other area of the face also succumb to swelling.

Angiotensin converting enzyme (ACE) normally causes the breakdown of bradykinin in the body. However, when ACE inhibitors are used, bradykinin breakdown is suppressed and angioedema can develop.

In hereditary angioedema, there is a decreased production of C1-INH which leads to the activation of the complement system [6] [7]. This unopposed activation of complement system results in the continuous production of bradykinin. In autoimmune angioedema, antibodies are formed against C1-INH and bradykinin is similarly increased.

In addition, some agents such as alcohol and some drugs (such as ibuprofen and aspirin) can increase the incidence of angioedema [8].

Prevention

  • Susceptible individuals should avoid drugs such as alcohol, aspirin and ACE Inhibitors.
  • Exposure to known allergens should be avoided.
  • Burns should be prevented.

Summary

Angioedema is swelling of skin, subcutaneous and submucosal tissues and can be acquired or genetic. In genetic angioedema, SERPING1 and F12 genes are involved. Acquired angioedema can be due to drugs, allergy or burns. The main mechanism of swelling in angioedema is the activation of bradykinin which is a potent vasodilator. The patients usually present with swelling of face and hands. In severe cases, respiratory distress can occur.

Patient Information

Angioedema means the swelling of the skin and underlying tissue because of the collection of fluid. It can result from genetic causes or from acquired causes. The patients usually develop swelling of the face, arms and hands. Sometimes, swelling may involve the upper respiratory tract which leads to breathing difficulties. Emergency management may be indicated in such cases. The treatment depends upon the cause but the overall prognosis is good.

References

Article

  1. Frigas E, Nzeako UC. Angioedema. Pathogenesis, differential diagnosis, and treatment. Clinical reviews in allergy & immunology. Oct 2002;23(2):217-231.
  2. Scheirey CD, Scholz FJ, Shortsleeve MJ, Katz DS. Angiotensin-converting enzyme inhibitor-induced small-bowel angioedema: clinical and imaging findings in 20 patients. AJR. American journal of roentgenology. Aug 2011;197(2):393-398.
  3. Wakisaka M, Shuto M, Abe H, et al. Computed tomography of the gastrointestinal manifestation of hereditary angioedema. Radiation medicine. Dec 2008;26(10):618-621.
  4. Kaplan AP. Mediators of urticaria and angioedema. The Journal of allergy and clinical immunology. Nov 1977;60(5):324-332.
  5. Oschatz C, Maas C, Lecher B, et al. Mast cells increase vascular permeability by heparin-initiated bradykinin formation in vivo. Immunity. Feb 25 2011;34(2):258-268.
  6. Kalmar L, Hegedus T, Farkas H, Nagy M, Tordai A. HAEdb: a novel interactive, locus-specific mutation database for the C1 inhibitor gene. Human mutation. Jan 2005;25(1):1-5.
  7. Cugno M, Zanichelli A, Foieni F, Caccia S, Cicardi M. C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress. Trends in molecular medicine. Feb 2009;15(2):69-78.
  8. Asero R, Bavbek S, Blanca M, et al. Clinical management of patients with a history of urticaria/angioedema induced by multiple NSAIDs: an expert panel review. International archives of allergy and immunology. 2013;160(2):126-133.
  9. Rye Rasmussen EH, Bindslev-Jensen C, Bygum A. Angioedema--assessment and treatment. Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke. Nov 12 2012;132(21):2391-2395.
  10. Kaplan AP. Angioedema. The World Allergy Organization journal. Jun 2008;1(6):103-113.

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Last updated: 2017-08-09 17:51