Materiały źródłowe

• #1 Angioedema: Causes, Symptoms, Types & Treatments

  https://my.clevelandclinic.org/health/diseases/22632-angioedema

  How is angioedema treated? Treating angioedema depends on what kind of angioedema you have. For severe allergic reactions, you’ll often have injectable epinephrine to carry. You should administer this while calling 911. […] For allergic angioedema, your provider may suggest antihistamines or steroids. You may get them either in oral (pill or liquid) or intravenous (in the vein) form. […] If you have a non-allergic drug reaction, your provider will help you find a medication to replace the one that’s causing you to swell. […] Home remedies include things like using ice to reduce swelling or taking cool showers. These may work best on things like swelling in one place or all over your lip or a cool wet cloth over your swollen eyes. […] If you have hereditary, idiopathic or acquired C1 inhibitor deficiency angioedema, you’ll probably be referred to a specialist. Some medications that treat or prevent heredity angioedema include: C1 esterase inhibitor (recombinant) (Ruconest), C1 inhibitor (human) (Berinert, Cinryze, Haegarda), Ecallantide (Kalbitor), Icatibant (Firazyr), Lanadelumab (Takhzyro), Berotralstat (Orladeyo).

• #1 Acute Angioedema: Overview of Angioedema Treatment, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/756261-overview

  The goals of emergency treatment of angioedema are to prevent spontaneous eruption, to maintain a patent airway if eruption does occur, and to stop progression of disease. […] Treatment of angioedema includes histamine blockers (H1 and H2), steroids, and, in those with severe symptoms, epinephrine (intramuscular or subcutaneous). […] Anabolic steroids (eg, danazol), a C1 esterase inhibitor, or a kallikrein inhibitor (ecallantide) may be used for the acute phase of an attack of HAE. […] Most patients with mild acute angioedema may be treated in the same way as those with an allergic reaction. Severe symptoms require steroids, H1 and H2 blockers, and subcutaneous epinephrine in addition to antihistamines. […] For antihistamine treatment, diphenhydramine, cetirizine, loratadine, or fexofenadine are the first-line drugs.

• #1 Angioedema Treatment & Management: Approach Considerations, Histaminergic Angioedema (IH-AAE), Nonhistaminergic Angioedema (InH-AAE)

  https://emedicine.medscape.com/article/135208-treatment

  Patients with moderate to severe angioedema often present to the emergency department (ED). Epinephrine should be used when laryngeal angioedema is suspected. Supportive care (eg, pain control) should also be provided, regardless of the etiology. […] Inpatient care for angioedema is usually not necessary when timely treatment is administered. For patients with a known history of hereditary angioedema (HAE), a treatment option approved by the US Food and Drug Administration (FDA) (eg, C1 esterase inhibitor [C1-INH] concentrate, ecallantide, or icatibant) should be administered as soon as an angioedema attack is recognized. […] Airway protection is the most important consideration with laryngeal angioedema. It is helpful to include anesthesiologists, critical care specialists or pulmonologists, otolaryngologists, and respiratory therapists in the management team. In cases of possible airway compromise, early intubation may be preferred.

• #1 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Angioedema-Treatment.aspx

  Angioedema is essentially an allergic phenomenon. Treatment is common for all types of angioedema when the condition is a medical emergency. […] When angioedema affects the throat and tongue and there is rapidly progressing swelling of the throat, there may be progressive narrowing of the airways leading to difficulty in breathing. The priority of management in these conditions is emergency opening up of the airways and maintenance of respiration. […] If there is compromise in breathing, an endotracheal tube is inserted into the trachea and it is connected to oxygen tubes. This secures airway. Intubation should be performed as soon as possible since excessive swelling of the larynx and back of throat makes intubation difficult. In these cases a hole or opening is made over the neck (on the trachea) to enable air to pass. This is called tracheostomy.

• #1 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Antihistamine, corticosteroids, and epinephrine are treatments of histaminergic angioedema. […] Treatment of Bradykinin-mediated angioedema is often resistant to standard therapies such as epinephrine, glucocorticoids or antihistamines. […] Hereditary angioedema (On-demand treatment) Treat airway via intubation or surgical airway intervention. Treatment should be as early as possible. Hereditary angioedema attacks should be treated with C1 Inhibitor concentrate, ecallantide (Kallikrein inhibitor) or icatibant (bradykinin-receptor antagonist). Icatibant is an effective home-based, on-demand treatment. […] Short-term prophylaxis before high-risk procedures in high-risk individuals. Anesthesiologists must be aware of guideline-based treatment for hereditary and acquired angioedema. […] C1 Inhibitor is the first-line long-term prophylaxis, while the androgens are used as second-line agents.

• #1 Angioedema Treatment & Management: Approach Considerations, Histaminergic Angioedema (IH-AAE), Nonhistaminergic Angioedema (InH-AAE)

  https://emedicine.medscape.com/article/135208-treatment

  For moderate-to-severe cases of angioedema, close monitoring is often necessary. Diphenhydramine 50 mg intramuscularly (IM) or intravenously (IV) is helpful. Hydrocortisone 200 mg IV or methylprednisolone 40-60 mg IV may reduce the possibility of relapse. […] For laryngeal swelling and airway obstruction, close monitoring of the airway is mandatory. Epinephrine (1:1000) should be administered IM at a dose of 0.01 mg/kg or 0.3 mg, repeated every 10-15 minutes if necessary. Occasionally, intubation, or even tracheostomy, may be necessary. These patients should be admitted for at least 24 hours of observation. […] Most cases of angioedema can be managed well with outpatient treatment alone. Antihistamines, usually second-generation agents (eg, cetirizine, desloratadine, fexofenadine, levocetirizine, and loratadine), are often used as first-line treatment. These agents are also given to help reduce the severity or frequency of attacks, in dosages often as high as 4 times the standard dosage.

• #1 Angioedema – RCEMLearning

  https://www.rcemlearning.co.uk/reference/angioedema/

  Angioedema (AE) is pathogenetically similar to urticaria but involves the deeper dermal and subcutaneous tissue. The aetiology of angioedema can be either allergic (IgE and histamine mediated as in urticaria) or non-allergic. 90% of attacks of angioedema are of the allergic type. Non-allergic AE can be further subdivided (1) into drug induced (e.g. ACE-inhibitors), Hereditary (C1-esterase-inhibitor deficiency), Acquired, Idiopathic and Pseudoallergenic (see Diagram). Non-allergic angioedema is generally mediated by bradykinin. […] Bradykinin-mediated angioedema often does not respond to treatment with epinephrine, anti-histamines or corticosteroids, and alternate treatment strategies are required. […] In severe attacks of allergic angioedema and anaphylaxis, intramuscular epinephrine is first line treatment. Its adrenergic actions address both peripheral vasodilatation and oedema formation (mediated via alpha adrenoceptors) and inhibit further mediator release (mediated via beta adrenoceptors).

• #1 Hives and angioedema – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hives-and-angioedema/diagnosis-treatment/drc-20354914

  For severe hives or angioedema, doctors may prescribe a short course of an oral corticosteroid drug such as prednisone to reduce swelling, inflammation and itching. […] For a severe attack of hives or angioedema, you may need a trip to the emergency room and an emergency injection of epinephrine a type of adrenaline. If you have had a serious attack or your attacks recur despite treatment, your doctor may have you carry a penlike device that will allow you to self-inject epinephrine in emergencies.

• #1 Angioedema | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-024-00934-3

  Angioedema can occur in the absence of urticaria and can be broadly divided into three main categories: mast cell-mediated (e.g., histamine), non-mast-cell-mediated (bradykinin-induced) and idiopathic angioedema. […] The mainstays of management for angioedema are: (1) to avoid specific triggers (if possible and where known) and (2) treatment with medication (if indicated). […] For hereditary angioedema (HAE), there are specifically licensed treatments that can be used for the management of attacks, or for prophylaxis in order to prevent attacks. […] ACE inhibitors should be discontinued in any individual who presents with angioedema without urticaria as this condition is associated with life-threatening upper airway angioedema. […] Idiopathic/spontaneous angioedema responds well to prophylactic antihistamines (which may be increased to fourfold the standard dosing); however, more advanced treatment, such as omalizumab, may be needed. In some acute situations, oral corticosteroids may be required.

• #1 Hereditary Angioedema

  https://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/allergy/hereditary-angioedema/

  Berinert is a human, pasteurized, plasmaderived C1 inhibitor product that was first FDA approved in 2009 for the treatment of acute facial, laryngeal, or abdominal attacks in adolescents and adults. […] In 2009, ecallantide (Kalbitor), a kallikreininhibitor synthesized in yeast, was approved to treatment acute HAE attacks in patients 12 years and older. […] Icatibant (Firazyr), a selective bradykinin type 2receptor antagonist, was FDAapproved in 2011 for the treatment of acute attacks in patients 18 years and older. […] The newest treatment option is a recombinant C1 inhibitor (Ruconest) that was FDAapproved in July 2014 to treat acute attacks of HAE in adolescents and adult patients. […] Treatment of HAE poses a special challenge in pregnant women and children. […] With proper therapy, the morbidity and mortality of hereditary angioedema can be significantly reduced.

• #1 Angioedema Treatment & Management: Approach Considerations, Histaminergic Angioedema (IH-AAE), Nonhistaminergic Angioedema (InH-AAE)

  https://emedicine.medscape.com/article/135208-treatment

  Administration of nanofiltered C1-INH concentrate can shorten the duration of acute HAE attacks. […] In 2009, ecallantide, a potent, selective, reversible inhibitor of plasma kallikrein that suppresses bradykinin generation, was approved by the FDA for treatment of acute HAE attacks. […] In 2011, icatibant, a bradykinin B2 receptor antagonist, was approved by the FDA for the treatment of acute HAE attacks. […] The FDA has approved various agents including C1-INHs, the kallikrein inhibitor ecallantide, lanadelumab, a monoclonal antibody that targets kallikrein, and the bradykinin-receptor antagonist icatibant for use in patients with HAE, either as prophylaxis or to treat acute attacks. […] The Hereditary Angioedema International Working Group guidelines consider long-term prophylaxis to be appropriate for C1-INH-HAE patients in whom on-demand acute treatment is inadequate to minimize disease. […] Corticosteroid treatment is reserved for very recalcitrant cases. Antifibrinolytics have been tried in some patients with success (those with IH-AAE, C1-INH-AAE, and HAE).

• #1 Acute Angioedema: Overview of Angioedema Treatment, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/756261-overview

  Ecallantide (Kalbitor) was approved by the US Food and Drug Administration (FDA) for treating acute HAE attacks. […] Icatibant (Firazyr), a selective bradykinin B2 receptor antagonist, was approved by the US Food and Drug Administration for treatment of acute attacks HAE in adults in August 2011. […] Lanadelumab, a monoclonal antibody used for prophylaxis in HAE was approved by the FDA in 2018. […] Patients are given epsilon-aminocaproic acid or tranexamic acids, which are antifibrinolytics for short-term prophylaxis. […] Transfusions of fresh frozen plasma (FFP) have been shown to decrease recurring angioedema.

• #1 Angioedema – RCEMLearning

  https://www.rcemlearning.co.uk/reference/angioedema/

  A number of agents may be used for prophylaxis of HAE attacks (8), including: Tranexamic acid (TXA), which works by inhibiting conversion of plasminogen to plasmin, a key step in the autoamplification cycle that generates bradykinin; C1 estersase inhibitor concentrate (Cinryze); Anabolic steroids such as stanozolol, which have an uncertain mechanism of action; Plasma kallikrein inhibitors (Berotralstat or Lanadelumab) which prevent formation of bradykinin. […] Attacks of HAE can be treated with Icatibant (a bradykinin B2 receptor antagonist). Patients may have administered a dose of Icatibant at home prior to attending ED. Icatibant is administered as a single subcutaneous injection and has been shown to start producing symptomatic improvement in under an hour (9). […] If angioedema is suspected to have been caused by a medication such as an ACEi or NSAID, this medication should be immediately discontinued and avoided in future.

• #1 Evaluation and Management of Angioedema in the Emergency Department

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6625683/

  Bradykinin-mediated forms of angioedema are typically resistant to therapies effective in histamine-mediated reactions. At the time of construction for this review, several medications have been FDA approved for treatment of acute bradykinin-mediated angioedema: three C1-INH concentrates (two plasma-derived and one recombinant), one kallikrein inhibitor, and one bradykinin-2-receptor antagonist. […] Medication management focuses on three aspects: acute episode management, short-term prophylaxis, and long-term prophylaxis, with ED management focusing on the acute episode. If the suspected etiology is drug- or allergic-induced, the trigger must be removed. […] Fresh frozen plasma (FFP) has been recommended for use in angioedema based on case reports demonstrating improvement in HAE and ACEi-mediated angioedema, as FFP contains varying amounts of C1-INH.

• #1 ACE inhibitor angioedema A very late presentation

  https://www.racgp.org.au/afp/2013/december/ace-inhibitor-angioedema

  There are case reports of ACE inhibitor angioedema being successfully treated with fresh frozen plasma. These reports have appeared for over a decade now. There is a recent study of seven patients given fresh frozen plasma for this condition, all of whom had failed to respond to antihistamines, steroid and adrenaline. All seven rapidly improved after fresh frozen plasma. Thus, faced with a patient exhibiting severe ACE inhibitor angioedema, a trial of a couple of units of fresh frozen plasma would seem very reasonable to hopefully shorten the attack or to reduce the need for what could be a very difficult intubation. […] There are also reports of the successful use of the bradykinin receptor antagonist icatibant, a short peptide that has a structure related to bradykinin. It can be administered by subcutaneous injection and is used by patients with hereditary angioedema, often by self-administration. It is not available in rural hospitals and it costs about $3000 per dose. By comparison, fresh frozen plasma is available in most rural hospitals and is relatively cheap at $300 a bag. Fresh frozen plasma, however, has the disadvantage of having to be thawed, and the minor risk of being a blood product. […] Clearly, cases of the severity of this one require urgent airway management and may require emergency cricothyroidotomy.

• #1 Hereditary Angioedema

  https://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/allergy/hereditary-angioedema/

  Treatment of HAE relies on longterm prophylaxis, shortterm prophylaxis, and the treatment of acute attacks. The longterm goal is to minimize the frequency and severity of angioedema episodes. It has been reported that patients with HAE develop 1 to 2 swelling attacks per month on average. […] Long-term prophylaxis should be considered in patients with a history of lifethreatening attacks or serious attacks more than once every 3 months. […] Prior to U.S. Food and Drug Administration (FDA) approval of C1 inhibitor replacement therapy in 2008, prophylactic or longterm treatment of HAE relied on the use of synthetic 17-alkylated androgens: danazol and stanozolol (Winstrol). […] In doubleblind studies, androgens were associated with an increase in the level of C1 inhibitor and a reduction in the number of angioedema attacks.

• #1 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatment_guidelines

  Acute attacks of HAE are unpredictable, disabling, and can be life-threatening if the airway is affected. Quickly and effectively treating these attacks prevents complications and minimizes interference with everyday activities. This is the reason that an on-demand, acute treatment plan is essential for every patient. […] Current HAE management guidelines recommend treating at the first sign of an attack. Studies have shown that treating HAE attacks earlier reduces the total time with swelling symptoms and improves quality of life. […] Self and home administration can reduce the time of treatment and allows medication to be given earlier in an attack. […] Prophylactic treatment is an option for patients who wish to try to reduce the frequency and/or severity of HAE attacks by taking medication on a regular schedule.

• #1 Hereditary Angioedema

  https://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/allergy/hereditary-angioedema/

  Antifibrinolytic agents, such as aminocaproic acid, are another option for longterm treatment. […] More recently, various C1 inhibitor concentrates have been studied and FDAapproved for treatment of HAE in the United States. […] In 2008, a human nanofiltered, pasteurized C1 inhibitor concentrate (Cinryze) was approved by the FDA for prophylactic treatment of HAE. […] The FDA has not approved the use of human purified and nanofiltered C1 inhibitor concentrate for treatment of acute attacks although it has been shown to reduce the median time to onset of relief compared with placebo. […] Angioedema attacks are most often triggered by trauma or stress. Therefore, patients with HAE require additional protection perioperatively and, in particular, prior to undergoing dental procedures in efforts to minimize the risk of oropharyngeal swelling.

• #1 Angioedema: An Overview

  https://www.uspharmacist.com/article/angioedema-an-overview

  Short-term prophylaxis is recommended prior to an event that may induce an acute exacerbation. Consensus guidelines recommend the use of C1-INH concentrate prior to a dental or other surgical procedure. Alternatively, attenuated androgens may be used at a doubled dosage for 5 days before and 2 days after the procedure or event. Fresh frozen plasma (2-3 U) also may be useful for short-term prophylaxis. […] Treatment for an acute onset of swelling in AAE patients is similar to treatment for HAE. C1-INH concentrate, ecallantide, and icatibant may be utilized for acute attacks. Higher doses of C1-INH concentrate may be required for AAE patients than for HAE patients, and C1-INH concentrate may become ineffective over time in AAE. Treatment of the underlying lymphoproliferative disorder should also be addressed.

• #1 C1 Esterase inhibitor deficiency

  https://www.rch.org.au/clinicalguide/guideline_index/c1_esterase_inhibitor_deficiency/

  Intravenous C1 esterase inhibitor concentrate should be administered (see below for dosing). […] The use of a prophylactic agent prior to such procedures reduces the risk of precipitating angioedema. […] For planned procedures, danazol is the first choice of prophylactic agent, (10 mg/kg/day for 5-10 days before and 2-5 days after the procedure). […] Antihistamines and corticosteroids have no role in the management of HAE related angioedema. […] C1 esterase inhibitor is the treatment of choice for airway angioedema caused by HAE.

• #1 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatments

  CINRYZE is a C1 Esterase Inhibitor indicated for routine prevention against HAE attacks in adults, adolescents and pediatric patients (6 years of age and older). Cinryze is delivered intravenously and is approved for self-administration. […] FIRAZYR is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of HAE in adults 18 years of age and older. Firazyr is delivered by subcutaneous injection and is approved for self-administration. […] HAEGARDA is a plasma-derived concentrate of C1 Esterase Inhibitor (Human) indicated for routine prevention of HAE attacks in patients 6 years of age and older. Heagarda is delivered by subcutaneous injection and is approved for self-administration. […] ICATIBANT (GENERIC) is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of Hereditary Angioedema (HAE) in adults 18 years of age and older. Generic Icatibant is delivered by subcutaneous injection and is approved for self-administration.

• #1 Approved Treatments for Angioedema | Angioedema NewsEnvelope icon

  https://angioedemanews.com/approved-treatments-for-angioedema/

  Danazol is an approved oral medication that’s used to prevent swelling attacks of all types, including those involving the skin, abdomen, and larynx, or voice box, in people with hereditary angioedema (HAE). Only generic versions of the therapy are currently available in the U.S. […] Firazyr (icatibant) is an approved on-demand therapy for the management of swelling attacks in people with hereditary angioedema (HAE). It is given via a subcutaneous injection and can be self-administered. Several generic versions of the therapy are also available. in the U.S. […] Haegarda (human C1 esterase inhibitor) is a prophylactic treatment approved to prevent acute swelling attacks in children and adults with hereditary angioedema (HAE). It is given via a subcutaneous injection and can be self-administered. It works by restoring C1-INH levels.

• #1 Treatment of Hereditary Angioedema

  https://www.aaaai.org/tools-for-the-public/drug-guide/immunomodulator-medications

  Treatment of Hereditary Angioedema: Replacement therapy or immune modulating medicines pertaining to hereditary angioedema. […] Acute attacks of hereditary angioedema Intravenous medication to treat acute attacks of hereditary angioedema. […] To prevent angioedema due to C1 esterase inhibitor deficiency. Intravenous medication to prevent angioedema due to C1 esterase inhibitor deficiency. […] Subcutaneous medication to prevent angioedema due to C1 esterase inhibitor deficiency. […] Acute treatment of angioedema. […] Acute attacks of hereditary angioedema- can be self-administered 1 dose with onset of attack, can repeat in 6 hours, no more than 3 doses in 24 hrs. […] 12 years and above Type I and II hereditary angioedema 300 mg subcutaneous every 2 weeks; consider every 4 weeks after 6 months.

• #1 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatments

  KALBITOR is a plasma kallikrein inhibitor indicated for treatment of acute attacks of HAE in patients 12 years of age and older. Kalbitor is delivered by subcutaneous injection and must be administered by a healthcare professional. […] ORLADEYO is a plasma kallikrein inhibitor indicated for routine prevention of HAE attacks in adults and pediatric patients 12 years and older. ORLADEYO is taken orally once daily. […] RUCONEST is a plasma free recombinant C1-Inhibitor concentrate for treating HAE attacks in adults and adolescents. Ruconest is delivered intravenously and is approved for self-administration. […] TAKHZYRO is a plasma kallikrein inhibitor (monoclonal antibody) indicated for routine prevention of HAE attacks in patients 12 years and older. TAKHZYRO is administered by subcutaneous injection and is approved for self-administration.

• #1 Angioedema: Causes, Appearance and Treatment — DermNet

  https://dermnetnz.org/topics/angioedema

  Treatment for HAE aims to decrease morbidity and mortality, and reduce the need for hospitalisation. Acute presentations include: C1-INH replacement – human plasma-derived treatments administered via Intravenous injection eg, complement C1 esterase inhibitors (Berinert, Cinryze). Bradykinin antagonists eg, Icatibant. […] Prophylaxis includes: Androgen therapy eg, Danazol. Side effects should be considered including weight gain, menstrual irregularities, and hirsutism. Tranexamic acid.

• #1 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Angioedema-Treatment.aspx

  Typically fresh-frozen plasma, which contains C1 INH is found to be as effective as C1 INH concentrate and symptoms improve within 30 to 60 minutes of the infusion. […] Attenuated androgens including danazol are also used to increase the production of C1 INH from the liver and this prevents attacks in hereditary angioedema.

• #1 Angioedema – assessment and treatment | Tidsskrift for Den norske legeforening

  https://tidsskriftet.no/en/2012/11/angioedema-assessment-and-treatment

  Long-term treatment with tranexamic acid or weak androgens (e.g. danazol) may be used for chronic bradykinergic angioedema and idiopathic angioedema. […] Follow-up and treatment of patients with chronic angioedema are often conducted by the GP, an allergologist, pulmonary specialist or dermatologist.

• #1 Angioedema in the emergency department: a practical guide to differential diagnosis and management | International Journal of Emergency Medicine | Full Text

  https://intjem.biomedcentral.com/articles/10.1186/s12245-017-0141-z

  For patients with nonhistamine-mediated angioedema, ACEis should be discontinued immediately with prescription of an alternative class of medication with a different mechanism of action. […] To ensure optimal treatment of patients presenting with angioedema, each ED would benefit from having an established protocol, algorithm, or management plan in place that is displayed or easily accessible. […] Hospitals should ensure that adequate procedures and treatments are in place for the management of angioedema.

• #1 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Treatment is with antihistamines, epinephrine, and glucocorticoids. Care should be taken to stop the offending ACE inhibitor, and the patient should be not rechallenged with any of the ACE inhibitors in the future. […] Majority cases are asymptomatic and respond to immunochemotherapy. Treatment of acute attacks with icatibant and plasma-derived C1 inhibitor concentrate, and prophylaxis is with rituximab with or without chemotherapy and splenectomy. […] In pregnancy, the recommended therapy is plasma-derived nano-filtered C1-inhibitor, however, in acute episodes, bradykinin receptor antagonist Icatibant can be used as it is safe with no maternal and fetal adverse effects. […] Icatibant is a well-tolerated medication in the pediatric group and might have a role in treating angiotensin II receptor blocker induced angioedema.

• #1 ACE inhibitor-induced angioedema

  https://dermnetnz.org/topics/ace-inhibitor-induced-angioedema

  ACE inhibitor-induced angioedema is a rare but potentially fatal adverse effect of the class of medications that inhibit angiotensin converting enzyme (ACEi). […] General measures to treat ACE inhibitor-induced angioedema can include: supportive care and monitoring, airway management, including tracheostomy or intubation, and adrenaline, antihistamines, and steroids are unhelpful. […] Specific measures to treat ACE inhibitor-induced angioedema can include the following: cessation of the ACE inhibitor no ACE inhibitor drug should ever be taken again. Angiotensin receptor-neprilysin inhibitor (ARNi) drugs are also contraindicated. Renin inhibitors and dipeptidyl peptidase-4 (DDP-4) inhibitors should be used with caution. The safest replacement for the ACEi would be an angiotensin receptor blocker (ARB).

• #1 Angioedema – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/immunology-allergic-disorders/allergic-autoimmune-and-other-hypersensitivity-disorders/angioedema

  For mast cell-mediated angioedema, medications that may relieve symptoms include H1 blockers. […] If symptoms are severe, a corticosteroid and antihistamine can be given IV. […] For bradykinin-mediated angioedema, epinephrine, corticosteroids, and antihistamines have not been shown to be effective. […] Angioedema due to ACE inhibitor use usually resolves about 24 to 48 hours after stopping the medication. […] For idiopathic angioedema, a high dose of a nonsedating oral antihistamine can be tried. […] For symptomatic and adjunctive treatment, an antihistamine (eg, H1 blocker) and a systemic corticosteroid can relieve symptoms of mast cell-mediated angioedema; frozen plasma, C1 inhibitor concentrate, and/or ecallantide or icatibant may be tried if bradykinin-mediated angioedema is severe or refractory.

• #1 ACE inhibitor-induced angioedema

  https://dermnetnz.org/topics/ace-inhibitor-induced-angioedema

  Mild episodes of ACE inhibitor-induced angioedema are self-limiting and resolve without treatment. However, episodes tend to become more frequent and more serious with continued use of the ACEi, and can be fatal if the cause is unrecognised. Discontinuing the ACEi is the only long-term management option, and episodes may continue in the weeks or months after cessation.

• #1 What is Angioedema – Asthma & Allergy Clinic | Boise, Nampa, Eagle, Meridian, Caldwell

  https://theallergygroup.com/what-is-angioedema/

  In some cases, the cause of angioedema is not identified. This is referred to as idiopathic angioedema. In these instances, an oral antihistamine at higher doses might be prescribed. Omalizumab is once a month subcutaneous injection for idiopathic angioedema and very helpful in patients with recurrent symptoms. […] It is important that you avoid your triggers. When itching occurs, talk to your doctor about using a non-prescription oral antihistamine to help alleviate this discomfort. Wearing clothing that is cotton and loose can help to decrease your risk of skin irritation. Cool compresses or a cool bath might also be beneficial for calming irritation and itching.

• #1 Angioedema Treatment in NJ & PA | Becker ENT & Allergy

  https://www.beckerentandallergy.com/allergy/angioedema-treatment

  Xolair (omalizumab) is an anti-IgE medication used to treat chronic idiopathic angioedema and allergic angioedema that is not responsive to other treatments. […] Omalizumab is a monoclonal antibody medication that can be used to treat chronic idiopathic angioedema by blocking the action of immunoglobulin E (IgE), a protein involved in allergic reactions. […] Danazol is an androgenic steroid medication used to treat hereditary angioedema by increasing the production of C1-inhibitor protein. […] Biologic medications or immunotherapy target specific proteins or cells involved in the inflammatory response and can help manage angioedema symptoms. […] When choosing a doctor to treat angioedema, it is important to find someone who has experience in dealing with the condition. […] It is also important to have a plan of action in place for acute treatment of angioedema when needed. […] For those suffering from severe angioedema, a healthcare provider may be able to diagnose the condition based on a physical exam and a review of symptoms. […] Preventing future angioedema attacks involves identifying and avoiding triggers, as well as making certain lifestyle changes.

• #1 Hereditary angioedema Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/hereditary-angioedema

  Antihistamines, corticosteroids and other treatments used for idiopathic angioedema and hives (urticaria) do not work well for HAE. Epinephrine could be used in life-threatening reactions, although it is unlikely to stop the swelling. There are a number of newer FDA-approved treatments for HAE. […] Some are given through a vein (IV) and can be used at home. Others are given as an injection under the skin by the person. Another is given by mouth. Some of these medicines are used regularly to prevent attacks and others are used emergently when an attack occurs to prevent it from getting worse. The choice of which agent may be based on the age of the person, personal preference, and where the symptoms occur. […] Before these newer medicines became available, androgen medicines such as danazol, were used to reduce the frequency and severity of attacks. These medicines help the body make more C1 inhibitor. However, many women have serious side effects from these medicines. They can also not be used in children.

• #1 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatments

  HAE Treatments The information, including opinions and recommendations, on this page is for educational purposes only. It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. No one should act upon any information provided on this page without first seeking medical advice from a qualified, licensed medical doctor. For assistance finding a physician, please click here. […] With eight products currently approved by the FDA for preventing and treating Hereditary Angioedema (HAE) attacks, and several therapies approved for generic use, people with HAE and their physicians have options for developing an HAE treatment plan tailored to meet each person’s unique needs. […] BERINERT is a plasma-derived C1 Esterase Inhibitor (Human) indicated for the treatment of HAE attacks in adult and pediatric patients. Berinert is delivered intravenously and is approved for self-administration.

• #1 Urticaria and Angioedema: A Practical Approach | AAFP

  https://www.aafp.org/pubs/afp/issues/2004/0301/p1123.html

  Laryngeal angioedema is a medical emergency requiring prompt assessment. […] Patients with a history of urticaria and angioedema or isolated angioedema episodes should be prescribed epinephrine in an auto-injectable instrument (EpiPen) in a dosage of 0.3 mg given intra-muscularly in the thigh and be instructed in its proper use for laryngeal edema, bronchospasm, and hypotension. […] Investigative treatment using immunomodulatory therapies such as cyclosporine, plasmapheresis, and intravenous immunoglobulin have been shown to be beneficial in autoimmune chronic urticaria.

• #1 Hereditary Angioedema (HAE) Treatment and Diagnosis

  https://www.firazyr.com/understanding-hae/diagnosis-and-treatment

  HAE is usually passed down through families, so your doctor will likely ask if anyone else in your family has been diagnosed. […] Current 2020 guidelines from the US Hereditary Angioedema Association (HAEA) recommend that people with HAE have at least 2 doses of an acute therapy, such as FIRAZYR, on hand for immediate use in the case of an acute attack. […] The guidelines recommend that acute therapy of an HAE attack be self-administered (or administered by a caregiver) as soon as symptoms begin, no matter where the swelling occurs or how severe it is. […] FIRAZYR is prescribed to treat acute HAE attacks in adults 18 years of age and older. […] Experts recommend you treat all HAE attacks, regardless of where they are located on your body.

• #1 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatment_guidelines

  HAE is a highly variable condition, which means that attack severity, frequency, and affected body regions can change over time. It may be necessary to adjust the HAE treatment plan along with these changes, so it’s essential for the HAE physician to regularly monitor the patient’s condition. […] One of the most important resources for any HAE patient is your expert physician.

• #1 An overview of angioedema: Clinical features, diagnosis, and management – UpToDate

  https://www.uptodate.com/contents/an-overview-of-angioedema-clinical-features-diagnosis-and-management

  Angioedema in or near the airway […] Angioedema with anaphylaxis […] Acute allergic angioedema (less severe than anaphylaxis) […] ACE inhibitor-induced angioedema […] C1 inhibitor deficiency (hereditary angioedema) […] Recurrent, idiopathic angioedema […] Treatment of acute episodes.

• #1 Hereditary Angioedema – Allergy & Asthma Network

  https://allergyasthmanetwork.org/health-a-z/hae-hereditary-angioedema/

  Hereditary angioedema (HAE) is a rare and potentially life-threatening genetic disease causing episodes of severe and painful swelling (called edema) in the hands, feet, arms, legs or face, as well as the gastrointestinal tract. […] Allergists often treat HAE because the swelling appears to be an allergic reaction. However, HAE cannot be treated by standard allergy medications, including antihistamines, corticosteroids and epinephrine. […] Because HAE is so rare and requires a personalized treatment plan, seeing an HAE specialist is important. If you’re diagnosed with HAE, ask your doctor for a referral to an immunologist. […] The U.S. Food and Drug Administration (FDA) has approved seven medications to help treat or prevent HAE swelling. Patients should talk with an HAE specialist about which therapy is best for them. Some treatments can be self-administered at home. […] More therapies are undergoing clinical trials and could come to market in the near future. […] Today, HAE specialists can help create an individualized treatment plan that makes it possible to lead a full life.

• #1 Hereditary Angioedema Types, Treatment, Symptoms, Diagnosis

  https://www.medicinenet.com/hereditary_angioedema_hae/article.htm

  What is the best treatment guidelines hereditary angioedema? […] The World Allergy Organization (WAO) issued these guidelines for HAE Types I and II in 2017 (updated 2021): […] Consider on-demand treatment for all hereditary angioedema attacks. Attacks affecting the upper airways must be treated. […] Treat all hereditary angioedema attacks as early as possible. C1-INH, Kalbitor (ecallantide), or Firazyr (icatibant) are recommended treatments. […] All HAE-I/II patients should have on-demand treatment for two attacks and carry their on-demand treatment at all times. […] A CC1-inhibitor is recommended as a first-line treatment long-term therapy for patients. […] Androgens are recommended as a second-line treatment long-term therapy for patients. […] Long-term therapy for patients with adjusted doses and/or treatment intervals is recommended.

• #1 Hereditary Angioedema (HAE) Treatment & Management

  https://www.discoverhae.com/hcp/treatment

  HAE management plans should be individualized with treatment tailored to each patients1: Medical needs, Life circumstances, Preferences, Tolerance of and response to specific medications. […] Patients reporting difficulty with frequent, severe, or disruptive HAE attacks should be assessed for a dosage adjustment or alternative therapy. […] On-demand or acute treatments can minimize morbidity and prevent mortality from an ongoing HAE attack. […] All patients with HAE should have access to at least 2 doses of an on-demand medication with them at all times for the treatment of acute attacks. […] Preventive or long-term prophylactic (LTP) treatments help prevent HAE attacks before they occur. […] According to hereditary angioedema treatment guidelines, decisions regarding which patients should be considered for LTP should take into account the patients quality of life and treatment preference in the context of attack frequency, attack severity, comorbid conditions, and access to emergent treatment. […] HAE guidelines recommend reviewing management plans for patients with HAE, including the need to start or continue a preventive treatment. […] Work with your patient’s larger care team to advocate for an effective management plan that prevents and reduces HAE attacks.

• #1 Hereditary Angioedema Types, Treatment, Symptoms, Diagnosis

  https://www.medicinenet.com/hereditary_angioedema_hae/article.htm

  Plasma-derived C1-INH is the preferred on-demand therapy for HAE-I/II attacks in children under 12. and for pregnant females or women who are breastfeeding. […] All individuals with HAE should have an action plan, a product available to treat HAE attacks, and an HAE identification card. […] Self-administration of treatment should be taught to all individuals who are licensed for self-administration to give or be given the on-demand treatment of HAE. […] All individuals with HAE should have at least one annual assessment by an HAE specialist to have comprehensive and integrated care. […] Patient education about HAE triggers. […] Guidelines are subject to change as newer drugs and modifications of treatment protocols are made available. People with HAE are urged to discuss their treatments and treatment changes, if any, with their healthcare professional(s).

• #1 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Recombinant human C1 esterase inhibitor for acute hereditary angioedema treatment has a persistent response for three days. […] C1 inhibitor is used in the acute management of hereditary angioedema associated pancreatitis. […] Patients with life-threatening orolingual angioedema who are treated with recombinant tissue plasminogen activator infusion, have a rapid response after using icatibant treatment. […] Use of omalizumab (Anti-immunoglobulin-E antibody) in Idiopathic non-histaminergic acquired angioedema, which is a rare disease resistant to antihistamines.

• #1

  https://link.springer.com/article/10.1007/s40521-019-0203-y

  Angioedema is a well-known side effect of ACE inhibitors. Current knowledge shows that the underlying pathophysiological mechanism is an excess of bradykinin, most likely due to ineffective breakdown pathways. […] The objective of this review was to evaluate the evidence for treatment of ACE inhibitor-induced angioedema by means of drugs intervening in the bradykinin system. […] Double-blind placebo-controlled trials were only found for ecallantide and icatibant. Both treatments did not show superiority over conventional treatment with histamine receptor antagonists and steroids. […] To date, there is insufficient evidence for the use of bradykinin receptor antagonists and kallikrein production inhibitors. Although case reports regarding the use of fresh frozen plasma and C1 inhibitor concentrate are positive, well-designed trials are lacking.

• #1

  https://link.springer.com/article/10.1007/s40521-019-0203-y

  In recent years, several new drugs have been developed for the treatment of HAE which have also been evaluated in patients with ACEia motivated by the assumed similar pathophysiological principle, being the excessive presence of BK. […] Theoretically, both inhibition of production (ecallantide) and prevention of the binding of BK to the receptor (icatibant) could be of use in ACEia. […] Although the earliest reports of icatibant showed improvement of time to relief or resolution of ACEia when compared to an historical cohort or a group of patients that received standard therapy with antihistamines and corticosteroids, this observation could not be confirmed in larger randomized-controlled trials. […] Ecallantide is a KK inhibitor with a proven efficacy in hereditary angioedema. […] To date, no phase 3 clinical studies have been performed with C1 inhibitor concentrate in ACEia. However, several case reports and case series have reported successful outcomes with C1 inhibitor concentrate.

• #1 Evaluation and Management of Angioedema in the Emergency Department

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6625683/

  Angioedema is defined by non-dependent, non-pitting edema that affects several different sites and is potentially life-threatening due to laryngeal edema. This narrative review provides emergency physicians with a focused overview of the evaluation and management of angioedema. […] Histamine-mediated angioedema should be treated with epinephrine intramuscularly, antihistaminergic medications, and steroids. These medications are not effective for bradykinin-mediated forms. Other medications include C1-INH protein replacement, kallikrein inhibitor, and bradykinin receptor antagonists. Evidence is controversial concerning the efficacy of these medications in an acute episode, and airway management is the most important intervention when indicated. […] Histamine-mediated angioedema is treated like anaphylaxis with epinephrine, antihistamines, and steroids. These medications are not effective for the bradykinin-mediated forms, although they can be attempted in the absence of effective therapy. Other medications include C1-INH protein replacement, kallikrein inhibitor, and bradykinin receptor antagonists. Several studies have evaluated these for angioedema, but the evidence is lacking for efficacy. The focus should be on airway management rather than medications in bradykinin-mediated angioedema.

• #1 Angioedema – assessment and treatment | Tidsskrift for Den norske legeforening

  https://tidsskriftet.no/en/2012/11/angioedema-assessment-and-treatment

  Angioedema has numerous hereditary, acquired and iatrogenic causes. A number of studies show that angioedema is inadequately assessed and treated during its acute phase as well as in the follow-up period. We present an algorithm for the assessment and treatment of patients with angioedema. […] The acute and prophylactic treatment will depend on the subtype of angioedema and is best provided through cross-disciplinary collaboration. […] Angioedema is a potentially life-threatening condition and should be assessed and treated systematically. It is important to remember that angioedema is either histaminergic or non-histaminergic, as the treatment of the two types is different. […] The treatment of angioedema will depend on its subtype. In acute severe instances treatment is provided by AE, Intensive Care or Ear, Nose Throat departments. In acute instances of angioedema of the airways, keeping the airways clear is paramount.

• #1 Angioedema – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/angioedema/

  Angioedema is a self-limited, localized swelling of the dermis, subcutaneous tissues, and/or submucosal tissues caused by fluid leakage into the interstitial tissue. […] Treatment generally consists of aggressive supportive care and avoidance of triggers, if applicable. In acute cases, histamine-mediated angioedema is treated with systemic glucocorticoids, antihistamines, and, if necessary, epinephrine for anaphylaxis. Bradykinin-mediated angioedema is treated with C1 inhibitor (C1-INH) concentrate, bradykinin-B2-receptor antagonists, or kallikrein inhibitors. […] Once the patient has been stabilized, identify and treat the cause of angioedema. […] Standard therapy includes IV antihistamines and IV glucocorticoids. […] Administer one of the following targeted therapies: Purified C1-INH concentrate, kallikrein inhibitor, or bradykinin-B2 receptor antagonist. […] Epinephrine, glucocorticoids, and antihistamines are usually not effective in acute hereditary angioedema attacks!

• #1 Hives and angioedema – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hives-and-angioedema/symptoms-causes/syc-20354908

  Angioedema can arise with hives or alone. It causes swelling in the deeper layers of skin, often around the face and lips. Short-lived (acute) hives and angioedema are common. Most times, they are harmless, clear up within in a day and don’t leave any lasting marks, even without treatment. Hives and angioedema are usually treated with antihistamine medication. Angioedema can be life-threatening if swelling of the tongue or in the throat blocks the airway. […] Severe angioedema can be life-threatening if swelling of the tongue or in the throat blocks the airway.

• #2 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Antihistamine, corticosteroids, and epinephrine are treatments of histaminergic angioedema. […] Treatment of Bradykinin-mediated angioedema is often resistant to standard therapies such as epinephrine, glucocorticoids or antihistamines. […] Hereditary angioedema (On-demand treatment) Treat airway via intubation or surgical airway intervention. Treatment should be as early as possible. Hereditary angioedema attacks should be treated with C1 Inhibitor concentrate, ecallantide (Kallikrein inhibitor) or icatibant (bradykinin-receptor antagonist). Icatibant is an effective home-based, on-demand treatment. […] Short-term prophylaxis before high-risk procedures in high-risk individuals. Anesthesiologists must be aware of guideline-based treatment for hereditary and acquired angioedema. […] C1 Inhibitor is the first-line long-term prophylaxis, while the androgens are used as second-line agents.

• #2 Angioedema – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/immunology-allergic-disorders/allergic-autoimmune-and-other-hypersensitivity-disorders/angioedema

  Angioedema can also be a hereditary or an acquired disorder characterized by an abnormal complement response. […] Treatment is with airway management as needed, elimination or avoidance of the allergen, and drugs to minimize swelling (eg, H1 blockers). […] For mast cell-mediated angioedema, an antihistamine and sometimes a systemic corticosteroid and epinephrine. […] For ACE inhibitor-related angioedema, occasionally fresh frozen plasma and C1 inhibitor concentrate. […] Securing an airway is the highest priority. […] In mast cell-mediated angioedema, treatment usually rapidly reduces airway edema; however, in bradykinin-mediated angioedema, edema usually takes 30 minutes to decrease after treatment begins. […] Treatment of angioedema also includes removing or avoiding the allergen and using medications that relieve symptoms.

• #2 Angioedema Treatment & Management: Approach Considerations, Histaminergic Angioedema (IH-AAE), Nonhistaminergic Angioedema (InH-AAE)

  https://emedicine.medscape.com/article/135208-treatment

  Patients with moderate to severe angioedema often present to the emergency department (ED). Epinephrine should be used when laryngeal angioedema is suspected. Supportive care (eg, pain control) should also be provided, regardless of the etiology. […] Inpatient care for angioedema is usually not necessary when timely treatment is administered. For patients with a known history of hereditary angioedema (HAE), a treatment option approved by the US Food and Drug Administration (FDA) (eg, C1 esterase inhibitor [C1-INH] concentrate, ecallantide, or icatibant) should be administered as soon as an angioedema attack is recognized. […] Airway protection is the most important consideration with laryngeal angioedema. It is helpful to include anesthesiologists, critical care specialists or pulmonologists, otolaryngologists, and respiratory therapists in the management team. In cases of possible airway compromise, early intubation may be preferred.

• #2 Acute Angioedema: Overview of Angioedema Treatment, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/756261-overview

  The goals of emergency treatment of angioedema are to prevent spontaneous eruption, to maintain a patent airway if eruption does occur, and to stop progression of disease. […] Treatment of angioedema includes histamine blockers (H1 and H2), steroids, and, in those with severe symptoms, epinephrine (intramuscular or subcutaneous). […] Anabolic steroids (eg, danazol), a C1 esterase inhibitor, or a kallikrein inhibitor (ecallantide) may be used for the acute phase of an attack of HAE. […] Most patients with mild acute angioedema may be treated in the same way as those with an allergic reaction. Severe symptoms require steroids, H1 and H2 blockers, and subcutaneous epinephrine in addition to antihistamines. […] For antihistamine treatment, diphenhydramine, cetirizine, loratadine, or fexofenadine are the first-line drugs.

• #2 Angioedema: Causes, Appearance and Treatment — DermNet

  https://dermnetnz.org/topics/angioedema

  Treatment is dependent on the subtype of angioedema. Angioedema is usually treated in the same way as acute urticaria when mild. […] General measures include: Aim to identify potential triggers. Avoidance or removal of causative agents eg, allergens, drugs. Adequate observation time in a hospital environment if angioedema has continued to progress despite treatments. […] Specific measures for Allergic and Idiopathic Histaminergic Angioedema include: For very mild symptoms, treatment may not be required. Antihistamines in the acute phase. Non-sedating second-generation antihistamines tend to be used first-line e.g. cetirizine, loratadine. Higher than usual (four times the standard) doses may be necessary. Prophylactic antihistamines may be used. Depending on the severity or with impending airway jeopardy, a short course of oral steroids eg, prednisolone as well as adrenaline may be required. Tranexamic acid can be offered for refractory recurrent angioedema. Omalizumab may be reserved as prophylaxis for refractory recurrent angioedema. Adrenaline autoinjectors are seldom required for angioedema with weals.

• #2 Hives and angioedema – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hives-and-angioedema/diagnosis-treatment/drc-20354914

  For severe hives or angioedema, doctors may prescribe a short course of an oral corticosteroid drug such as prednisone to reduce swelling, inflammation and itching. […] For a severe attack of hives or angioedema, you may need a trip to the emergency room and an emergency injection of epinephrine a type of adrenaline. If you have had a serious attack or your attacks recur despite treatment, your doctor may have you carry a penlike device that will allow you to self-inject epinephrine in emergencies.

• #2 Angioedema Treatment & Management: Approach Considerations, Histaminergic Angioedema (IH-AAE), Nonhistaminergic Angioedema (InH-AAE)

  https://emedicine.medscape.com/article/135208-treatment

  For moderate-to-severe cases of angioedema, close monitoring is often necessary. Diphenhydramine 50 mg intramuscularly (IM) or intravenously (IV) is helpful. Hydrocortisone 200 mg IV or methylprednisolone 40-60 mg IV may reduce the possibility of relapse. […] For laryngeal swelling and airway obstruction, close monitoring of the airway is mandatory. Epinephrine (1:1000) should be administered IM at a dose of 0.01 mg/kg or 0.3 mg, repeated every 10-15 minutes if necessary. Occasionally, intubation, or even tracheostomy, may be necessary. These patients should be admitted for at least 24 hours of observation. […] Most cases of angioedema can be managed well with outpatient treatment alone. Antihistamines, usually second-generation agents (eg, cetirizine, desloratadine, fexofenadine, levocetirizine, and loratadine), are often used as first-line treatment. These agents are also given to help reduce the severity or frequency of attacks, in dosages often as high as 4 times the standard dosage.

• #2 Angioedema Treatment & Management: Approach Considerations, Histaminergic Angioedema (IH-AAE), Nonhistaminergic Angioedema (InH-AAE)

  https://emedicine.medscape.com/article/135208-treatment

  Administration of nanofiltered C1-INH concentrate can shorten the duration of acute HAE attacks. […] In 2009, ecallantide, a potent, selective, reversible inhibitor of plasma kallikrein that suppresses bradykinin generation, was approved by the FDA for treatment of acute HAE attacks. […] In 2011, icatibant, a bradykinin B2 receptor antagonist, was approved by the FDA for the treatment of acute HAE attacks. […] The FDA has approved various agents including C1-INHs, the kallikrein inhibitor ecallantide, lanadelumab, a monoclonal antibody that targets kallikrein, and the bradykinin-receptor antagonist icatibant for use in patients with HAE, either as prophylaxis or to treat acute attacks. […] The Hereditary Angioedema International Working Group guidelines consider long-term prophylaxis to be appropriate for C1-INH-HAE patients in whom on-demand acute treatment is inadequate to minimize disease. […] Corticosteroid treatment is reserved for very recalcitrant cases. Antifibrinolytics have been tried in some patients with success (those with IH-AAE, C1-INH-AAE, and HAE).

• #2 Acute Angioedema: Overview of Angioedema Treatment, Prehospital Care, Emergency Department Care

  https://emedicine.medscape.com/article/756261-overview

  Ecallantide (Kalbitor) was approved by the US Food and Drug Administration (FDA) for treating acute HAE attacks. […] Icatibant (Firazyr), a selective bradykinin B2 receptor antagonist, was approved by the US Food and Drug Administration for treatment of acute attacks HAE in adults in August 2011. […] Lanadelumab, a monoclonal antibody used for prophylaxis in HAE was approved by the FDA in 2018. […] Patients are given epsilon-aminocaproic acid or tranexamic acids, which are antifibrinolytics for short-term prophylaxis. […] Transfusions of fresh frozen plasma (FFP) have been shown to decrease recurring angioedema.

• #2 Angioedema – Wikipedia

  https://en.wikipedia.org/wiki/Angioedema

  Angioedema can be treated with antihistamines, corticosteroids, and epinephrine. […] In those with bradykinin-related disease a C1 esterase inhibitor, ecallantide, or icatibant may be used. Fresh frozen plasma may be used instead. […] In allergic angioedema, avoidance of the allergen and use of antihistamines may prevent future attacks. […] Severe angioedema cases may require desensitization to the putative allergen, as mortality can occur. […] 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. […] The medications ecallantide and icatibant may be used to treat attacks. […] Future attacks of HAE can be prevented by the use of androgens such as danazol, oxandrolone or methyltestosterone. […] 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.

• #2 Angioedema: Causes, Symptoms, Types & Treatments

  https://my.clevelandclinic.org/health/diseases/22632-angioedema

  How is angioedema treated? Treating angioedema depends on what kind of angioedema you have. For severe allergic reactions, you’ll often have injectable epinephrine to carry. You should administer this while calling 911. […] For allergic angioedema, your provider may suggest antihistamines or steroids. You may get them either in oral (pill or liquid) or intravenous (in the vein) form. […] If you have a non-allergic drug reaction, your provider will help you find a medication to replace the one that’s causing you to swell. […] Home remedies include things like using ice to reduce swelling or taking cool showers. These may work best on things like swelling in one place or all over your lip or a cool wet cloth over your swollen eyes. […] If you have hereditary, idiopathic or acquired C1 inhibitor deficiency angioedema, you’ll probably be referred to a specialist. Some medications that treat or prevent heredity angioedema include: C1 esterase inhibitor (recombinant) (Ruconest), C1 inhibitor (human) (Berinert, Cinryze, Haegarda), Ecallantide (Kalbitor), Icatibant (Firazyr), Lanadelumab (Takhzyro), Berotralstat (Orladeyo).

• #2 ACE inhibitor-induced angioedema

  https://dermnetnz.org/topics/ace-inhibitor-induced-angioedema

  ACE inhibitor-induced angioedema is a rare but potentially fatal adverse effect of the class of medications that inhibit angiotensin converting enzyme (ACEi). […] General measures to treat ACE inhibitor-induced angioedema can include: supportive care and monitoring, airway management, including tracheostomy or intubation, and adrenaline, antihistamines, and steroids are unhelpful. […] Specific measures to treat ACE inhibitor-induced angioedema can include the following: cessation of the ACE inhibitor no ACE inhibitor drug should ever be taken again. Angiotensin receptor-neprilysin inhibitor (ARNi) drugs are also contraindicated. Renin inhibitors and dipeptidyl peptidase-4 (DDP-4) inhibitors should be used with caution. The safest replacement for the ACEi would be an angiotensin receptor blocker (ARB).

• #2 Angioedema – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/immunology-allergic-disorders/allergic-autoimmune-and-other-hypersensitivity-disorders/angioedema

  For mast cell-mediated angioedema, medications that may relieve symptoms include H1 blockers. […] If symptoms are severe, a corticosteroid and antihistamine can be given IV. […] For bradykinin-mediated angioedema, epinephrine, corticosteroids, and antihistamines have not been shown to be effective. […] Angioedema due to ACE inhibitor use usually resolves about 24 to 48 hours after stopping the medication. […] For idiopathic angioedema, a high dose of a nonsedating oral antihistamine can be tried. […] For symptomatic and adjunctive treatment, an antihistamine (eg, H1 blocker) and a systemic corticosteroid can relieve symptoms of mast cell-mediated angioedema; frozen plasma, C1 inhibitor concentrate, and/or ecallantide or icatibant may be tried if bradykinin-mediated angioedema is severe or refractory.

• #2 Hives (Urticaria) and Angioedema Overview

  https://www.aaaai.org/tools-for-the-public/conditions-library/allergies/hives-(urticaria)-and-angioedema-overview

  Angioedema usually occurs in the face, throat, hands, and feet. […] Understanding the underlying mechanism of swelling or the specific disease is critical in determining the best treatment. […] Antihistamines are frequently an effective form of treatment. […] Antihistamines are the best initial medication to treat your hives. […] Sometimes, a combination of several antihistamines or an increased dose of one antihistamine may be recommended. […] Non-sedating antihistamines are often the first medication doctors will prescribe for your hives. […] About 50% of chronic spontaneous urticaria (hives over 6 weeks with no identifiable cause) will respond to antihistamine as discussed above. […] For those who do not improve on antihistamines, 65% respond to omalizumab. […] Corticosteroids, such as prednisone or prednisolone may help hives.

• #2 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Treatment is with antihistamines, epinephrine, and glucocorticoids. Care should be taken to stop the offending ACE inhibitor, and the patient should be not rechallenged with any of the ACE inhibitors in the future. […] Majority cases are asymptomatic and respond to immunochemotherapy. Treatment of acute attacks with icatibant and plasma-derived C1 inhibitor concentrate, and prophylaxis is with rituximab with or without chemotherapy and splenectomy. […] In pregnancy, the recommended therapy is plasma-derived nano-filtered C1-inhibitor, however, in acute episodes, bradykinin receptor antagonist Icatibant can be used as it is safe with no maternal and fetal adverse effects. […] Icatibant is a well-tolerated medication in the pediatric group and might have a role in treating angiotensin II receptor blocker induced angioedema.

• #2 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatment_guidelines

  Acute attacks of HAE are unpredictable, disabling, and can be life-threatening if the airway is affected. Quickly and effectively treating these attacks prevents complications and minimizes interference with everyday activities. This is the reason that an on-demand, acute treatment plan is essential for every patient. […] Current HAE management guidelines recommend treating at the first sign of an attack. Studies have shown that treating HAE attacks earlier reduces the total time with swelling symptoms and improves quality of life. […] Self and home administration can reduce the time of treatment and allows medication to be given earlier in an attack. […] Prophylactic treatment is an option for patients who wish to try to reduce the frequency and/or severity of HAE attacks by taking medication on a regular schedule.

• #2 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatments

  CINRYZE is a C1 Esterase Inhibitor indicated for routine prevention against HAE attacks in adults, adolescents and pediatric patients (6 years of age and older). Cinryze is delivered intravenously and is approved for self-administration. […] FIRAZYR is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of HAE in adults 18 years of age and older. Firazyr is delivered by subcutaneous injection and is approved for self-administration. […] HAEGARDA is a plasma-derived concentrate of C1 Esterase Inhibitor (Human) indicated for routine prevention of HAE attacks in patients 6 years of age and older. Heagarda is delivered by subcutaneous injection and is approved for self-administration. […] ICATIBANT (GENERIC) is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of Hereditary Angioedema (HAE) in adults 18 years of age and older. Generic Icatibant is delivered by subcutaneous injection and is approved for self-administration.

• #2 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatments

  KALBITOR is a plasma kallikrein inhibitor indicated for treatment of acute attacks of HAE in patients 12 years of age and older. Kalbitor is delivered by subcutaneous injection and must be administered by a healthcare professional. […] ORLADEYO is a plasma kallikrein inhibitor indicated for routine prevention of HAE attacks in adults and pediatric patients 12 years and older. ORLADEYO is taken orally once daily. […] RUCONEST is a plasma free recombinant C1-Inhibitor concentrate for treating HAE attacks in adults and adolescents. Ruconest is delivered intravenously and is approved for self-administration. […] TAKHZYRO is a plasma kallikrein inhibitor (monoclonal antibody) indicated for routine prevention of HAE attacks in patients 12 years and older. TAKHZYRO is administered by subcutaneous injection and is approved for self-administration.

• #2 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatment_guidelines

  HAE is a highly variable condition, which means that attack severity, frequency, and affected body regions can change over time. It may be necessary to adjust the HAE treatment plan along with these changes, so it’s essential for the HAE physician to regularly monitor the patient’s condition. […] One of the most important resources for any HAE patient is your expert physician.

• #2 Hereditary Angioedema (HAE) Treatment & Management

  https://www.discoverhae.com/hcp/treatment

  HAE management plans should be individualized with treatment tailored to each patients1: Medical needs, Life circumstances, Preferences, Tolerance of and response to specific medications. […] Patients reporting difficulty with frequent, severe, or disruptive HAE attacks should be assessed for a dosage adjustment or alternative therapy. […] On-demand or acute treatments can minimize morbidity and prevent mortality from an ongoing HAE attack. […] All patients with HAE should have access to at least 2 doses of an on-demand medication with them at all times for the treatment of acute attacks. […] Preventive or long-term prophylactic (LTP) treatments help prevent HAE attacks before they occur. […] According to hereditary angioedema treatment guidelines, decisions regarding which patients should be considered for LTP should take into account the patients quality of life and treatment preference in the context of attack frequency, attack severity, comorbid conditions, and access to emergent treatment. […] HAE guidelines recommend reviewing management plans for patients with HAE, including the need to start or continue a preventive treatment. […] Work with your patient’s larger care team to advocate for an effective management plan that prevents and reduces HAE attacks.

• #2 Hereditary Angioedema Types, Treatment, Symptoms, Diagnosis

  https://www.medicinenet.com/hereditary_angioedema_hae/article.htm

  What is the best treatment guidelines hereditary angioedema? […] The World Allergy Organization (WAO) issued these guidelines for HAE Types I and II in 2017 (updated 2021): […] Consider on-demand treatment for all hereditary angioedema attacks. Attacks affecting the upper airways must be treated. […] Treat all hereditary angioedema attacks as early as possible. C1-INH, Kalbitor (ecallantide), or Firazyr (icatibant) are recommended treatments. […] All HAE-I/II patients should have on-demand treatment for two attacks and carry their on-demand treatment at all times. […] A CC1-inhibitor is recommended as a first-line treatment long-term therapy for patients. […] Androgens are recommended as a second-line treatment long-term therapy for patients. […] Long-term therapy for patients with adjusted doses and/or treatment intervals is recommended.

• #2 Angioedema in the emergency department: a practical guide to differential diagnosis and management | International Journal of Emergency Medicine | Full Text

  https://intjem.biomedcentral.com/articles/10.1186/s12245-017-0141-z

  For patients with nonhistamine-mediated angioedema, ACEis should be discontinued immediately with prescription of an alternative class of medication with a different mechanism of action. […] To ensure optimal treatment of patients presenting with angioedema, each ED would benefit from having an established protocol, algorithm, or management plan in place that is displayed or easily accessible. […] Hospitals should ensure that adequate procedures and treatments are in place for the management of angioedema.

• #2 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Recombinant human C1 esterase inhibitor for acute hereditary angioedema treatment has a persistent response for three days. […] C1 inhibitor is used in the acute management of hereditary angioedema associated pancreatitis. […] Patients with life-threatening orolingual angioedema who are treated with recombinant tissue plasminogen activator infusion, have a rapid response after using icatibant treatment. […] Use of omalizumab (Anti-immunoglobulin-E antibody) in Idiopathic non-histaminergic acquired angioedema, which is a rare disease resistant to antihistamines.

• #2 Urticaria and Angioedema: A Practical Approach | AAFP

  https://www.aafp.org/pubs/afp/issues/2004/0301/p1123.html

  Laryngeal angioedema is a medical emergency requiring prompt assessment. […] Patients with a history of urticaria and angioedema or isolated angioedema episodes should be prescribed epinephrine in an auto-injectable instrument (EpiPen) in a dosage of 0.3 mg given intra-muscularly in the thigh and be instructed in its proper use for laryngeal edema, bronchospasm, and hypotension. […] Investigative treatment using immunomodulatory therapies such as cyclosporine, plasmapheresis, and intravenous immunoglobulin have been shown to be beneficial in autoimmune chronic urticaria.

• #2 Evaluation and Management of Angioedema in the Emergency Department

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6625683/

  Angioedema is defined by non-dependent, non-pitting edema that affects several different sites and is potentially life-threatening due to laryngeal edema. This narrative review provides emergency physicians with a focused overview of the evaluation and management of angioedema. […] Histamine-mediated angioedema should be treated with epinephrine intramuscularly, antihistaminergic medications, and steroids. These medications are not effective for bradykinin-mediated forms. Other medications include C1-INH protein replacement, kallikrein inhibitor, and bradykinin receptor antagonists. Evidence is controversial concerning the efficacy of these medications in an acute episode, and airway management is the most important intervention when indicated. […] Histamine-mediated angioedema is treated like anaphylaxis with epinephrine, antihistamines, and steroids. These medications are not effective for the bradykinin-mediated forms, although they can be attempted in the absence of effective therapy. Other medications include C1-INH protein replacement, kallikrein inhibitor, and bradykinin receptor antagonists. Several studies have evaluated these for angioedema, but the evidence is lacking for efficacy. The focus should be on airway management rather than medications in bradykinin-mediated angioedema.

• #2 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatments

  Prior to 2008, limited HAE treatment options were available in the United States. Now, people with HAE can choose from eight FDA-approved medications (and several generic icatibants) for managing their condition. […] The availability of multiple on-demand and prophylaxis treatment options that can be administered at home has significantly increased the HAEA community’s quality of life while decreasing emergency room visits and hospitalizations. […] Prophylactic treatment is an option for people who wish to try to reduce the frequency and/or severity of HAE attacks by taking medication on a regular schedule. […] Acute attacks of HAE are unpredictable, disabling and can be life-threatening if the airway is affected. Quickly and effectively treating these attacks prevents complications and minimizes interference with everyday activities.

• #3 Angioedema Treatment & Management: Approach Considerations, Histaminergic Angioedema (IH-AAE), Nonhistaminergic Angioedema (InH-AAE)

  https://emedicine.medscape.com/article/135208-treatment

  Patients with moderate to severe angioedema often present to the emergency department (ED). Epinephrine should be used when laryngeal angioedema is suspected. Supportive care (eg, pain control) should also be provided, regardless of the etiology. […] Inpatient care for angioedema is usually not necessary when timely treatment is administered. For patients with a known history of hereditary angioedema (HAE), a treatment option approved by the US Food and Drug Administration (FDA) (eg, C1 esterase inhibitor [C1-INH] concentrate, ecallantide, or icatibant) should be administered as soon as an angioedema attack is recognized. […] Airway protection is the most important consideration with laryngeal angioedema. It is helpful to include anesthesiologists, critical care specialists or pulmonologists, otolaryngologists, and respiratory therapists in the management team. In cases of possible airway compromise, early intubation may be preferred.

• #3 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Antihistamine, corticosteroids, and epinephrine are treatments of histaminergic angioedema. […] Treatment of Bradykinin-mediated angioedema is often resistant to standard therapies such as epinephrine, glucocorticoids or antihistamines. […] Hereditary angioedema (On-demand treatment) Treat airway via intubation or surgical airway intervention. Treatment should be as early as possible. Hereditary angioedema attacks should be treated with C1 Inhibitor concentrate, ecallantide (Kallikrein inhibitor) or icatibant (bradykinin-receptor antagonist). Icatibant is an effective home-based, on-demand treatment. […] Short-term prophylaxis before high-risk procedures in high-risk individuals. Anesthesiologists must be aware of guideline-based treatment for hereditary and acquired angioedema. […] C1 Inhibitor is the first-line long-term prophylaxis, while the androgens are used as second-line agents.

• #3 ACE inhibitor-induced angioedema

  https://dermnetnz.org/topics/ace-inhibitor-induced-angioedema

  ACE inhibitor-induced angioedema is a rare but potentially fatal adverse effect of the class of medications that inhibit angiotensin converting enzyme (ACEi). […] General measures to treat ACE inhibitor-induced angioedema can include: supportive care and monitoring, airway management, including tracheostomy or intubation, and adrenaline, antihistamines, and steroids are unhelpful. […] Specific measures to treat ACE inhibitor-induced angioedema can include the following: cessation of the ACE inhibitor no ACE inhibitor drug should ever be taken again. Angiotensin receptor-neprilysin inhibitor (ARNi) drugs are also contraindicated. Renin inhibitors and dipeptidyl peptidase-4 (DDP-4) inhibitors should be used with caution. The safest replacement for the ACEi would be an angiotensin receptor blocker (ARB).

• #3 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Treatment is with antihistamines, epinephrine, and glucocorticoids. Care should be taken to stop the offending ACE inhibitor, and the patient should be not rechallenged with any of the ACE inhibitors in the future. […] Majority cases are asymptomatic and respond to immunochemotherapy. Treatment of acute attacks with icatibant and plasma-derived C1 inhibitor concentrate, and prophylaxis is with rituximab with or without chemotherapy and splenectomy. […] In pregnancy, the recommended therapy is plasma-derived nano-filtered C1-inhibitor, however, in acute episodes, bradykinin receptor antagonist Icatibant can be used as it is safe with no maternal and fetal adverse effects. […] Icatibant is a well-tolerated medication in the pediatric group and might have a role in treating angiotensin II receptor blocker induced angioedema.

• #3 US Hereditary Angioedema Association

  https://www.haea.org/pages/p/treatment_guidelines

  Acute attacks of HAE are unpredictable, disabling, and can be life-threatening if the airway is affected. Quickly and effectively treating these attacks prevents complications and minimizes interference with everyday activities. This is the reason that an on-demand, acute treatment plan is essential for every patient. […] Current HAE management guidelines recommend treating at the first sign of an attack. Studies have shown that treating HAE attacks earlier reduces the total time with swelling symptoms and improves quality of life. […] Self and home administration can reduce the time of treatment and allows medication to be given earlier in an attack. […] Prophylactic treatment is an option for patients who wish to try to reduce the frequency and/or severity of HAE attacks by taking medication on a regular schedule.

• #3 Hereditary Angioedema Types, Treatment, Symptoms, Diagnosis

  https://www.medicinenet.com/hereditary_angioedema_hae/article.htm

  What is the best treatment guidelines hereditary angioedema? […] The World Allergy Organization (WAO) issued these guidelines for HAE Types I and II in 2017 (updated 2021): […] Consider on-demand treatment for all hereditary angioedema attacks. Attacks affecting the upper airways must be treated. […] Treat all hereditary angioedema attacks as early as possible. C1-INH, Kalbitor (ecallantide), or Firazyr (icatibant) are recommended treatments. […] All HAE-I/II patients should have on-demand treatment for two attacks and carry their on-demand treatment at all times. […] A CC1-inhibitor is recommended as a first-line treatment long-term therapy for patients. […] Androgens are recommended as a second-line treatment long-term therapy for patients. […] Long-term therapy for patients with adjusted doses and/or treatment intervals is recommended.

• #3 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Recombinant human C1 esterase inhibitor for acute hereditary angioedema treatment has a persistent response for three days. […] C1 inhibitor is used in the acute management of hereditary angioedema associated pancreatitis. […] Patients with life-threatening orolingual angioedema who are treated with recombinant tissue plasminogen activator infusion, have a rapid response after using icatibant treatment. […] Use of omalizumab (Anti-immunoglobulin-E antibody) in Idiopathic non-histaminergic acquired angioedema, which is a rare disease resistant to antihistamines.

• #4 Angioedema – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK538489/

  Treatment is with antihistamines, epinephrine, and glucocorticoids. Care should be taken to stop the offending ACE inhibitor, and the patient should be not rechallenged with any of the ACE inhibitors in the future. […] Majority cases are asymptomatic and respond to immunochemotherapy. Treatment of acute attacks with icatibant and plasma-derived C1 inhibitor concentrate, and prophylaxis is with rituximab with or without chemotherapy and splenectomy. […] In pregnancy, the recommended therapy is plasma-derived nano-filtered C1-inhibitor, however, in acute episodes, bradykinin receptor antagonist Icatibant can be used as it is safe with no maternal and fetal adverse effects. […] Icatibant is a well-tolerated medication in the pediatric group and might have a role in treating angiotensin II receptor blocker induced angioedema.

• #4 Hereditary Angioedema Types, Treatment, Symptoms, Diagnosis

  https://www.medicinenet.com/hereditary_angioedema_hae/article.htm

  What is the best treatment guidelines hereditary angioedema? […] The World Allergy Organization (WAO) issued these guidelines for HAE Types I and II in 2017 (updated 2021): […] Consider on-demand treatment for all hereditary angioedema attacks. Attacks affecting the upper airways must be treated. […] Treat all hereditary angioedema attacks as early as possible. C1-INH, Kalbitor (ecallantide), or Firazyr (icatibant) are recommended treatments. […] All HAE-I/II patients should have on-demand treatment for two attacks and carry their on-demand treatment at all times. […] A CC1-inhibitor is recommended as a first-line treatment long-term therapy for patients. […] Androgens are recommended as a second-line treatment long-term therapy for patients. […] Long-term therapy for patients with adjusted doses and/or treatment intervals is recommended.

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