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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.

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]
  • Angioedema is a subcutaneous extension of urticaria, resulting in deep swelling within subcutaneous sites.[emedicine.com]
  • Angioedema, also called Angioneurotic Edema, or Giant Urticaria, allergic disorder in which large, localized, painless swellings similar to hives appear under the skin.[britannica.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]
Pain
  • A 55-year-old Asian female presented to the emergency department complaining of oral pain, swelling, and numbness. Her family reported that she began to experience sharp pain of the tongue and lips immediately after eating "elephant root."[ncbi.nlm.nih.gov]
  • 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]
  • A 19-year-old woman presents to the emergency department with light-headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier.[doi.org]
  • […] from angioedema of the GI tract mucosa can be as intense as pain from an acute abdomen.[emedicine.com]
Collapse
  • This will lead to the following signs : sudden or rapidly escalating breathing problems fainting or dizziness collapsing This is a medical emergency. Call 911 immediately if you notice these signs.[medicalnewstoday.com]
  • 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]
Fatigue
  • […] 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]
  • Nausea, fatigue, headache, and diarrhea are common side effects. People with HAE may also find relief by taking androgens (male hormones) such as methyltestosterone and danazol.[allergies.about.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]
  • In the standard-therapy group, mild exacerbation of chronic obstructive pulmonary disease was reported in one patient, an increase in the blood glucose level in one patient, fatigue in one patient, and an influenza-like illness in one patient.[nejm.org]
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]
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]
  • Edema of the upper airways may cause respiratory distress and stridor; the stridor may be mistaken for asthma. The airways may be completely obstructed. Edema of the intestines may cause nausea, vomiting, colicky abdominal pain, and/or diarrhea.[msdmanuals.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. Mild symptoms may not need treatment. Moderate to severe symptoms may need to be treated.[medlineplus.gov]
  • 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]
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]
  • Edema of the upper airways may cause respiratory distress and stridor; the stridor may be mistaken for asthma. The airways may be completely obstructed. Edema of the intestines may cause nausea, vomiting, colicky abdominal pain, and/or diarrhea.[msdmanuals.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]
Abdominal Pain
  • A 19-year-old woman presents to the emergency department with light-headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier.[doi.org]
  • 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]
  • KEYWORDS: C1-inhibitor; acute abdominal pain; angiotensin-converting enzyme inhibitors; hereditary angioedema; intestinal angioedema[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]
  • Abdominal associated with angioedema may manifest as severe acute-onset abdominal pain or as moderately severe chronic recurrent abdominal pain. Two medications are currently FDA-approved for the treatment of these patients.[ncbi.nlm.nih.gov]
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]
  • In hereditary angioedema, caused by a defect in the immune system, swellings in the intestinal tract may produce pain, vomiting, or diarrhea, and edema of the larynx may cause death by asphyxiation.[britannica.com]
  • 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]
  • A 19-year-old woman presents to the emergency department with light-headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier.[doi.org]
  • 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]
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]
  • A 19-year-old woman presents to the emergency department with light-headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier.[doi.org]
  • 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]
  • Patients often present with non-specific findings of abdominal pain, nausea, and vomiting.[radiopaedia.org]
Diarrhea
  • After an exhaustive laboratory work-up, her diarrhea resolved only after the discontinuation of lisinopril.[ncbi.nlm.nih.gov]
  • In hereditary angioedema, caused by a defect in the immune system, swellings in the intestinal tract may produce pain, vomiting, or diarrhea, and edema of the larynx may cause death by asphyxiation.[britannica.com]
  • 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]
  • The most common adverse drug reactions are injection site reactions, upper respiratory infections, headache, rash, muscle pain, dizziness, and diarrhea. "HAE attacks are painful, debilitating, and potentially life threatening.[medscape.com]
Dysphagia
  • A 65-year-old Saudi Arab man presented to an emergency department with lip and tongue swelling and dysphagia. There were no systemic symptoms and no past history of a similar event.[ncbi.nlm.nih.gov]
  • Upon mucosal contact, injury can cause extreme pain, soft-tissue swelling, salivation, dysphagia, and even aphonia. This presentation can resemble angioedema or anaphylaxis.[ncbi.nlm.nih.gov]
  • 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]
  • […] 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, dysphagia[lifeinthefastlane.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]
  • On further questioning, she reported an episode of tongue and lip swelling occurring shortly after one of her abdominal attacks, which further supported the diagnosis. Subsequent testing described below confirmed the diagnosis.[web.archive.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]
  • […] agonist terbutaline (Brethaire, Brethine) has been shown to be more effective at controlling urticaria than placebo. [6] However, efficacy is low for urticaria and angioedema; therefore, it is seldom used for treatment of angioedema. [6] Patients with hypotension[emedicine.com]
  • Alternatively, the shifting of intravascular fluid into the tissues may result in hypotension and tachycardia regardless of the AE mechanism.[airwayelearning.com]
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
  • 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
  • Björn Gerdle, Ulrich Hilgenfeldt, Britt Larsson, Jesper Kristiansen, Karen Søgaard and Lars Rosendal, Bradykinin and kallidin levels in the trapezius muscle in patients with work-related trapezius myalgia, in patients with whiplash associated pain, and[doi.org]
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]
Angioedema
  • angioedema-urticaria Chemical-aggravated angioedema-urticaria Cholinergic angioedema Drug-aggravated angioedema-urticaria Episodic angioedema with eosinophilia Food-induced angioedema-urticaria Giant urticaria Idiopathic angioedema Idiopathic urticaria[icd9data.com]
  • To evaluate the time to onset of angioedema and the subsequent episodes of angioedema in patients initially experiencing ACE-I- or ARB-induced angioedema.[ncbi.nlm.nih.gov]
  • Crucially, bradykinin-mediated angioedema does not respond to the same treatment as histamine-mediated angioedema.[ncbi.nlm.nih.gov]
  • For other discussions on angioedema, see the overview topics Acquired Angioedema and Hereditary Angioedema.[emedicine.com]
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]
  • […] angioedema-urticaria Cholinergic angioedema Drug-aggravated angioedema-urticaria Episodic angioedema with eosinophilia Food-induced angioedema-urticaria Giant urticaria Idiopathic angioedema Idiopathic urticaria and/or angioedema Latex-induced angioedema-urticaria[icd9data.com]
  • Additionally, clinical manifestations of acute and chronic urticaria, hereditary and acquired angioedema, as well as the physical urticarias will be discussed.[doi.org]
  • […] tissue, mucosa and submucosal tissues Angioneurotic oedema Quincke's edema angioneurotic edema giant urticaria Giant Urticarias Quincke Edema Angioedemas Urticarias, Giant Quinckes Edema Urticaria, Giant Edema, Angioneurotic Edema, Quincke's Angioneurotic[wikidata.org]
Eruptions
  • Itay Tokatly Latzer, Nancy Agmon‐Levin, Raz Somech and Daniel Stein, Eruption of urticaria and angioedema induced by binging and purging in an anorexia nervosa patient, International Journal of Eating Disorders, 49, 8, (822-825), (2016).[doi.org]
  • […] urticaria; giant, 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]
  • 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]
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]
  • 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]
  • 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]
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]
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.[medlineplus.gov]
Headache
  • The most common adverse drug reactions are injection site reactions, upper respiratory infections, headache, rash, muscle pain, dizziness, and diarrhea. "HAE attacks are painful, debilitating, and potentially life threatening.[medscape.com]
  • 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]
  • (severe one-sided headache associated with puffiness around the eye on the same side).[allergy.org.au]
  • Nausea, fatigue, headache, and diarrhea are common side effects. People with HAE may also find relief by taking androgens (male hormones) such as methyltestosterone and danazol.[allergies.about.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]
  • You may also experience skin tingling or tightness.[health.ucsd.edu]
  • The swollen area of skin is usually not itchy (unless accompanied by urticaria) but can often have a burning, tingling, or numb sensation. The swelling can last for several hours or days.[allergies.about.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]
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.

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