Hereditary Angioedema (HAE)

Hereditary Angioedema Explained

Hereditary Angioedema (HAE) is a rare genetic disorder that causes sudden, unpredictable episodes of severe swelling deep in body tissues. Unlike a typical allergic reaction, HAE swelling is not caused by histamine — it is driven by a chemical called bradykinin. This single fact has enormous consequences: the antihistamines, steroids, and epinephrine that work for allergic reactions do not work for HAE, and patients who receive only those treatments can die of airway swelling while waiting for drugs that will not help them.

HAE is caused by a deficiency or malfunction of a protein called C1-esterase inhibitor (C1-INH), encoded by the SERPING1 gene. Without enough functional C1-INH, a cascade of enzymes runs unchecked and floods tissues with bradykinin, causing plasma to leak out of blood vessels into surrounding tissue. The swelling is non-pitting (it does not indent when pressed), is not red or itchy, and there are no hives — a key difference from allergic angioedema.

HAE is inherited in an autosomal dominant pattern: one mutated copy of the gene is enough to cause the disease. Each child of an affected parent has a 50% chance of inheriting HAE. However, approximately 25% of cases arise from de novo (new, spontaneous) mutations, so a negative family history does not rule out the diagnosis.

The condition affects approximately 1 in 10,000 to 1 in 50,000 people worldwide regardless of sex, race, or ethnicity. Historically, before effective treatments existed, untreated laryngeal (throat) attacks carried a mortality rate of 25–30%. A physician named Heinrich Quincke first described episodic angioedema in 1882; the hereditary pattern was characterized by William Osler in 1888; and the causative protein deficiency — absence of the blood's inhibitor of C1-esterase — was reported by Donaldson and Evans in 1963, with Fred Rosen and colleagues describing the two genetic variants in 1965 (one group of patients makes no inhibitor, another makes an abnormal, non-functioning protein) — opening the door to targeted treatment.

Back to Table of Contents

The Bradykinin Mechanism

To understand why HAE treatments work the way they do, it helps to understand the underlying chemistry. C1-INH (C1-esterase inhibitor) is a serpin — a serine protease inhibitor — that acts as a brake on several enzyme systems simultaneously:

• Complement pathway: C1-INH blocks C1r and C1s, the activated enzymes that kick off the classical complement cascade.
• Contact activation pathway: C1-INH inhibits Factor XIIa (Hageman factor) and plasma kallikrein, two enzymes that together generate bradykinin from its precursor protein, high-molecular-weight kininogen (HMWK).
• Fibrinolytic pathway: C1-INH also inhibits plasmin.

When C1-INH is absent or dysfunctional, plasma kallikrein operates without restraint. It cleaves HMWK in large amounts, releasing bradykinin. Bradykinin binds to bradykinin B2 receptors on vascular endothelial cells (the cells lining blood vessel walls). This binding opens gaps between cells, dramatically increasing vascular permeability. Plasma — the liquid part of blood — leaks out of capillaries and floods the surrounding tissue. The result is the swollen, fluid-filled tissue of an HAE attack.

Because histamine plays no role in this process, blocking histamine receptors (with antihistamines like diphenhydramine or cetirizine) cannot stop or reverse an HAE attack. Similarly, corticosteroids (which dampen histamine-related and arachidonic acid-related inflammation) and epinephrine (which counteracts histamine's effects on blood vessels) are ineffective at the root cause. Epinephrine may temporarily ease laryngeal symptoms by causing vasoconstriction, but it does not reduce bradykinin levels and provides no sustained benefit.

The practical implication is critical: what treats an HAE attack is HAE-specific rescue medication that targets bradykinin — not the allergy cart — and that applies to every patient with known or suspected HAE, including in the emergency room.

Back to Table of Contents

Three Types of HAE

There are three recognized subtypes of HAE, distinguished by laboratory findings and genetics:

Type 1 HAE (approximately 85% of cases)

In Type 1, a mutation in the SERPING1 gene causes the body to produce too little C1-INH protein — a quantitative deficiency. Lab tests show both low C1-INH antigen (the protein itself is sparse) and low C1-INH functional activity (what little protein exists may also not work well). The complement protein C4 is chronically low even between attacks, because uninhibited C1 enzymes continuously consume it. C3 levels remain normal. The serum C4 level is the best and most practical screening test.

Type 2 HAE (approximately 15% of cases)

In Type 2, the SERPING1 mutation produces C1-INH protein in normal or even elevated amounts — but the protein does not function correctly (a qualitative deficiency). The key laboratory finding is normal or elevated C1-INH antigen with low C1-INH functional activity. C4 is still chronically low (consumed by uninhibited complement enzymes), and C3 remains normal. Normal C1-INH antigen levels with low functional activity point strongly to Type 2. Genetic testing of SERPING1 confirms the diagnosis.

Type 3 HAE (rare; predominantly women)

Type 3 HAE is quite different from Types 1 and 2. C1-INH levels and C4 levels are both normal. The most common identified cause is a gain-of-function mutation in the F12 gene, which encodes Factor XII (Hageman factor). The mutant Factor XII is overactive, driving excessive kallikrein activity and bradykinin release even without any C1-INH deficiency. In some patients no causative mutation is found. Type 3 is strongly linked to estrogen: attacks are triggered or dramatically worsened by oral contraceptives, hormone replacement therapy, and pregnancy. Unexplained recurrent angioedema in a woman of childbearing age using estrogen-containing contraception is a classic pattern of Type 3 HAE.

A note on C1-INH levels vs. C4

Although C1-INH levels are low in Types 1 and 2, the serum C4 level is a better screening test because C4 remains depressed even between attacks (not just during them). A normal C4 level effectively excludes Types 1 and 2 HAE. C4 is inexpensive, widely available, and part of routine complement panels.

Back to Table of Contents

What HAE Attacks Feel Like

HAE attacks are episodic — they come and go, typically lasting 2 to 5 days without treatment and resolving spontaneously. Swelling builds slowly over hours to reach its peak, then recedes. Attacks can involve three main body regions, each with distinct symptoms:

Subcutaneous (under the skin) swelling

The most visually obvious form of HAE. Swelling commonly affects the extremities (hands, arms, legs, feet), face (lips, eyelids, cheeks), and genitalia. The swelling is characteristically non-pitting (it does not leave an indentation when pressed), cool to the touch, not red, and not itchy — and critically, there are no hives (urticaria). The absence of hives is one of the most important clinical clues that distinguishes HAE from allergic angioedema. The skin may have a slightly mottled or firm appearance but does not look like a typical allergic rash.

Gastrointestinal swelling

Gastrointestinal (GI) attacks are probably the most commonly misdiagnosed form of HAE. Swelling of the gut wall causes severe abdominal cramps, nausea, vomiting, diarrhea, and sometimes ileus (temporary intestinal paralysis). The pain can be excruciating and mimic a surgical emergency — many HAE patients have undergone unnecessary appendectomy or exploratory laparotomy before the diagnosis was established. Abdominal ultrasound during an attack may show free fluid (ascites) around the bowel loops, which can be a helpful diagnostic clue. Recurrent, unexplained abdominal crises with no clear surgical cause are a recognized reason to consider HAE.

Laryngeal (throat) swelling

Laryngeal HAE is the most dangerous manifestation. Swelling in the throat and larynx causes hoarseness, stridor (a high-pitched breathing sound), difficulty swallowing, and a sensation of throat tightening. It can progress rapidly to complete airway obstruction and asphyxiation. This is why HAE historically carried a 25–30% mortality rate in undiagnosed or untreated patients — people died of laryngeal attacks that were mistaken for allergic reactions and treated with drugs that did not work. Today, with proper diagnosis and on-hand rescue medication, laryngeal attacks are survivable, but they remain a medical emergency requiring immediate treatment.

Prodromal erythema marginatum

About 50% of patients experience a warning rash before a major attack: erythema marginatum, a serpiginous (snake-like, winding-edged), mottled reddish rash that is not raised and not itchy. This is the opposite of hives. Recognizing this rash as a prodrome — a warning sign — gives patients time to take rescue medication early, before swelling becomes severe, which is why patient education includes learning to recognize it.

Back to Table of Contents

What Triggers HAE Attacks

Attacks can be spontaneous and unpredictable, but many have identifiable triggers. Knowing and managing triggers is an important part of living with HAE:

• Emotional stress and anxiety: One of the most consistent and common triggers. Stress activates the contact system (Factor XII pathway), elevating bradykinin production. Many patients report attacks before important events, during family conflict, or after bad news.
• Physical trauma: Even minor tissue injury can trigger an attack. Dental procedures are a well-recognized and particularly dangerous trigger — manipulation of oral tissues can precipitate laryngeal swelling. Surgery and intubation (placing a breathing tube) carry high risk. Short-term prophylaxis is standard practice before invasive procedures (see Prevention section).
• Angiotensin-converting enzyme inhibitors (ACE inhibitors) — absolute contraindication: ACE (angiotensin-converting enzyme) inhibitors — drugs like lisinopril, enalapril, and ramipril — are among the most commonly prescribed medications for high blood pressure and heart failure. They work partly by blocking an enzyme that normally breaks down bradykinin. In HAE patients, blocking bradykinin degradation can dramatically worsen or precipitate attacks. Many patients are first diagnosed after an HAE attack triggered by a newly started angiotensin-converting enzyme inhibitor (ACE inhibitor). Angiotensin-converting enzyme inhibitors are absolutely contraindicated in HAE Types 1 and 2. Angiotensin receptor blockers (ARBs) such as losartan are generally considered safer alternatives for blood pressure control in these patients.
• Estrogen — oral contraceptives, hormone replacement therapy (HRT), and pregnancy: Estrogen stimulates Factor XII (Hageman factor) synthesis and activity, amplifying the contact activation pathway and bradykinin production. Estrogen-containing oral contraceptives and hormone replacement therapy can trigger or worsen attacks in women with Types 1 and 2 HAE, and are the primary trigger in Type 3. Progestin-only methods (e.g., progestin-only pills, hormonal IUDs, or implants) are the hormonal contraception usually used by women with HAE. In a study of 516 women with recurrent angioedema, oral contraceptives or hormone replacement therapy set off attacks in 63% of the women with type 1 HAE and 62% of those with type 3 who had used them, although many women with these conditions took them without any effect on their angioedema. Pregnancy is a particularly complex period — some women improve (due to rising C1-INH synthesis), while others experience more frequent or severe attacks, especially around labor and delivery.
• Infection: Upper respiratory infections, dental infections, and other acute illnesses can trigger attacks, likely through activation of the contact system as part of the inflammatory response.
• Menstruation: Some women experience predictable attacks linked to their menstrual cycle, likely due to hormonal fluctuations affecting kallikrein-kinin system activity.
• Alcohol and certain medications: Some patients report that alcohol triggers attacks. Nonsteroidal anti-inflammatory drugs (NSAIDs) and aspirin may also lower the threshold for attacks in some individuals.

Back to Table of Contents

Diagnosing HAE

HAE is notoriously underdiagnosed. The average time from symptom onset to correct diagnosis is 8 to 12 years. The condition is often mistaken for allergic angioedema, irritable bowel syndrome, or a surgical abdomen. Knowing what tests to order can save years of suffering.

Best initial screening test: Serum C4

The serum C4 level is the single most useful and cost-effective first test. C4 is chronically low in Types 1 and 2 HAE — not just during attacks, but between attacks too, because uninhibited C1 continuously consumes it. A normal C4 level effectively excludes Types 1 and 2 HAE. C4 can be ordered as part of a complement panel at any commercial lab. In Type 3 HAE, C4 is normal.

Confirmatory tests

• C1-INH antigen level: Measures the amount of C1-INH protein in the blood. Low in Type 1; normal or elevated in Type 2.
• C1-INH functional activity: Measures whether C1-INH actually works. Low in both Types 1 and 2 — this is the key test that cannot be skipped. Many labs offer a "C1-esterase inhibitor, functional" assay.
• C1q level: This test helps distinguish hereditary HAE from acquired angioedema (AAE). In HAE, C1q is normal. In AAE (which can be caused by lymphoma, autoantibodies against C1-INH, or other conditions), C1q is low because the underlying disease consumes it. A low C1q in an adult with late-onset angioedema is a reason clinicians look for lymphoma.

Genetic testing

SERPING1 gene sequencing confirms Types 1 and 2 at the molecular level and is particularly useful when laboratory results are borderline, when testing children of an affected parent (to identify presymptomatic cases), and for family planning. For suspected Type 3, F12 gene sequencing can identify the gain-of-function mutation. However, genetic testing is not required for clinical diagnosis — the combination of history, family history, and complement labs is usually sufficient.

Family history

A positive family history strongly supports the diagnosis. However, approximately 25% of HAE cases result from de novo mutations — brand-new mutations that did not come from either parent. A negative family history therefore does not exclude HAE. Children of patients with Types 1 or 2 HAE are usually tested early, as symptoms typically begin in childhood or adolescence and can be severe and even fatal without treatment.

Back to Table of Contents

Treating an Acute Attack

The most important principle of treating an acute HAE attack is to act early. Waiting to see if an attack resolves on its own — or wasting time on ineffective antihistamines and steroids — can be fatal in laryngeal attacks. Keeping rescue medication on hand at all times is standard for people with HAE. All of the following treatments target the bradykinin pathway, not histamine.

Critical warning: Antihistamines, corticosteroids, and epinephrine are not effective treatments for HAE. Epinephrine may provide brief temporary relief of laryngeal symptoms through vasoconstriction, but it does not lower bradykinin levels and is not a substitute for HAE-specific medication; waiting for these drugs to work only delays effective treatment.

Plasma-derived C1-INH concentrate (pdC1-INH)

Plasma-derived C1-INH given intravenously: 20 international units (IU)/kg. This replaces the missing or deficient C1-INH directly, restoring normal inhibition of kallikrein and the contact pathway. It has the most extensive clinical evidence base, is approved for all attack locations including laryngeal, and can be self-administered at home after training. A second, nanofiltered plasma-derived C1-INH product is also available. For laryngeal attacks, pdC1-INH is the preferred first-line agent.

Icatibant

Icatibant is a bradykinin B2 receptor antagonist — it blocks bradykinin from binding to its receptor on blood vessel walls, preventing the increase in vascular permeability. It is given as a 30 mg subcutaneous injection (just under the skin), with onset of action within 20–30 minutes. Patients can self-inject at home. A second dose can be given after 6 hours if the response is incomplete. It does not replace C1-INH or reduce bradykinin production, but by blocking the receptor, it prevents the downstream effect.

Ecallantide

Ecallantide is a plasma kallikrein inhibitor — it stops kallikrein from cleaving HMWK into bradykinin. It is given as three 10 mg subcutaneous injections (30 mg total). It is approved in the United States only and is given only in a healthcare setting because it carries an approximately 3% risk of serious allergic reactions including anaphylaxis. It is not available in Europe.

Recombinant C1-INH

Recombinant C1-INH (conestat alfa) is a recombinant form of human C1-INH derived from transgenic rabbits. It is given intravenously and works like plasma-derived C1-INH. It is an option for patients who prefer a non-plasma-derived product. Its U.S. label lists a history of allergy to rabbits or rabbit-derived products as a contraindication.

Sebetralstat (tablet)

Sebetralstat is a plasma kallikrein inhibitor taken by mouth when an attack starts — the first on-demand treatment tested as a tablet rather than an injection or infusion. In the KONFIDENT trial, 136 people aged 12 and over were assigned to sebetralstat 300 mg, 600 mg or placebo for their attacks (110 of them treated 264 attacks). The first symptom relief came at a median of 1.61 hours with 300 mg and 1.79 hours with 600 mg, against 6.72 hours with placebo, and the attack had fully resolved within 24 hours in 42.5% (300 mg), 49.5% (600 mg) and 27.4% (placebo) of attacks. Side effects were similar to placebo, with no serious drug-related adverse events reported.

Fresh Frozen Plasma (FFP)

FFP contains C1-INH along with all other plasma proteins. It can be life-saving when no HAE-specific drugs are available — for example, in a hospital without HAE medications in stock. FFP is less predictable and slower than specific HAE drugs, and some theoretical concern exists that the kallikrein substrates in FFP could transiently worsen swelling. Nevertheless, FFP is a last resort that is far better than nothing in a laryngeal emergency.

Back to Table of Contents

Preventing Attacks: Long-Term and Short-Term Prophylaxis

Prevention is as important as acute treatment in HAE management. There are two categories: short-term prophylaxis before a known trigger, and long-term prophylaxis (LTP) to reduce the overall frequency and severity of attacks.

Short-Term Prophylaxis (before procedures)

Any invasive procedure — dental work, surgery, intubation, endoscopy — poses a risk of triggering an acute attack, sometimes with laryngeal involvement. Short-term prophylaxis is planned for all HAE patients undergoing such procedures, regardless of how well-controlled they are:

• Plasma-derived C1-INH 1000 international units intravenous (IV), administered 1–6 hours before the procedure. This is the preferred approach when available.
• Attenuated androgens (danazol) at 5–10 mg/kg/day (up to 200 mg three times daily) starting 5 days before the procedure and continuing 2–3 days after. This is the older alternative and is effective, but androgen side effects limit its use for prolonged periods or in children and women.
• Whichever prophylactic agent is used, HAE-specific rescue medication is kept available during and after the procedure.

Long-Term Prophylaxis (LTP)

LTP is appropriate for patients with frequent attacks (roughly one or more per month), a history of laryngeal attacks, attacks that severely impair quality of life, or inadequate access to acute rescue medication. Multiple options are now available:

• Subcutaneous plasma-derived C1-INH: 60 international units/kg subcutaneous injection given twice weekly. In the COMPACT trial (90 patients), the 60 IU/kg dose cut attacks by a mean of 3.51 per month compared with placebo, 90% of patients had at least half as many attacks, and rescue-medication use fell from 3.89 to 0.32 uses per month. Self-injectable at home. Currently a first-line LTP option.
• Lanadelumab: An anti-plasma kallikrein monoclonal antibody that prevents kallikrein from producing bradykinin. The U.S. Food and Drug Administration label gives a recommended starting dosage, for adults and children 12 and older, of 300 mg injected under the skin every 2 weeks, and states that 300 mg every 4 weeks "is also effective and may be considered if the patient is well-controlled (e.g., attack free) for more than 6 months." In the HELP trial (125 patients aged 12 and older), attacks fell from an average of 3.5 a month before treatment to 0.26 a month on 300 mg every 2 weeks, against 1.97 a month on placebo; it reduced attack frequency by 87% versus placebo. Injection-site reactions (52.4% vs 34.1%) and dizziness (6.0% vs 0%) were more common than with placebo. U.S. Food and Drug Administration-approved in 2018.
• Berotralstat: The first daily oral LTP option. Berotralstat (150 mg/day oral capsule) is a small-molecule plasma kallikrein inhibitor. In the phase 3 APeX-2 trial (121 patients aged 12 and older), 150 mg a day lowered the attack rate to 1.31 a month against 2.35 on placebo; abdominal pain, vomiting, diarrhea and back pain were more common than with placebo, and the authors found the most favorable benefit-to-risk profile at 150 mg a day. U.S. Food and Drug Administration-approved in 2020. Many patients prefer oral medications over injections, making berotralstat an important addition to the LTP toolkit.
• Garadacimab: A once-monthly injection under the skin of an antibody that blocks activated factor XII, the first step of the chain that produces bradykinin. In the VANGUARD trial (64 patients aged 12 and older, 6 months), attacks averaged 0.27 a month on garadacimab against 2.01 on placebo — 87% fewer — and the median number of attacks per month on garadacimab was 0. Blocking factor XII was not linked to more bleeding or blood clots.
• Donidalorsen: An antisense medicine that lowers the liver's production of prekallikrein. In the OASIS-HAE trial of 90 patients, 80 mg under the skin every 4 weeks lowered the attack rate by 81% compared with placebo over weeks 1–25 (87% over weeks 5–25); every-8-week dosing lowered it by 55%. Injection-site redness, headache and nasal-throat infections were the most common side effects, and 98% of adverse events were mild or moderate.
• Attenuated androgens (danazol): An older but effective option. Danazol (typically 200 mg/day, adjusted to the lowest effective dose) increases hepatic synthesis of C1-INH, raising circulating levels. It can dramatically reduce attack frequency. However, significant side effects limit its use: virilization (facial hair, voice changes, acne, clitoral enlargement) in women; liver toxicity; adverse effects on cholesterol; and concern about hepatic adenomas with long-term use. The danazol label lists pregnancy and breastfeeding as contraindications, and androgen side effects are a particular concern in children.
• Tranexamic acid: An antifibrinolytic agent with modest efficacy in HAE prevention. It is used primarily in settings where newer drugs are unavailable or unaffordable. Side effects are generally mild. It is less effective than danazol or the newer biologics.

The landscape of HAE treatment has been transformed in the past 15 years. Patients who previously suffered weekly attacks and lived in fear of laryngeal emergencies can now achieve dramatic reduction in attack frequency, and some achieve complete attack freedom on prophylaxis. The current international reference is the 2021 revision of the WAO/EAACI guideline (World Allergy Organization and European Academy of Allergy and Clinical Immunology), in which an international expert panel reviewed the evidence and agreed 28 recommendations covering diagnosis, when to add prophylaxis to on-demand treatment, treatment goals, children and pregnant or breastfeeding women, and how patients monitor their disease.

Back to Table of Contents

Key Research Papers

1. Donaldson VH, Evans RR. A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C' 1-esterase. Am J Med. 1963;35:37–44. — The landmark 1963 paper identifying C1-INH deficiency as the cause of hereditary angioedema. — PubMed PMID: 14046003
2. Rosen FS, Pensky J, Donaldson V, Charache P. Hereditary angioneurotic edema: two genetic variants. Science. 1965;148(3672):957–8. — Described the two genetic variants (absent versus non-functioning inhibitor). — PubMed PMID: 14277836
3. Cicardi M, et al. Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. Allergy. 2006;61(4):422–33. — Characterizes acquired vs. hereditary angioedema and the role of autoantibodies. — Search PubMed
4. Bork K, Fischer B, Dewald G. Recurrent episodes of skin angioedema and severe attacks of abdominal pain induced by oral contraceptives or hormone replacement therapy. Am J Med. 2003;114(4):294–8. — Demonstrates estrogen as a major trigger for angioedema in susceptible women. — PubMed PMID: 12681457
5. Craig TJ, et al. Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks. J Allergy Clin Immunol. 2009;124(4):801–8. — Pivotal trial establishing intravenous plasma-derived C1-INH as effective acute HAE treatment. — PubMed PMID: 19767078
6. Cicardi M, et al. Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema. N Engl J Med. 2010;363(6):532–41. — Phase 3 trial demonstrating icatibant's efficacy as an acute HAE treatment. — PubMed PMID: 20818888
7. Banerji A, Riedl MA, Bernstein JA, et al. Effect of Lanadelumab Compared With Placebo on Prevention of Hereditary Angioedema Attacks: A Randomized Clinical Trial. JAMA. 2018;320(20):2108–2121. — HELP trial: lanadelumab reduced HAE attacks vs. placebo; led to FDA approval. — PubMed PMID: 30480729
8. Zuraw BL, et al. Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema. N Engl J Med. 2010;363(6):513–22. — Trial establishing nanofiltered C1-INH for acute treatment and routine prophylaxis of HAE. — PubMed PMID: 20818886
9. Longhurst H, Cicardi M, Craig T, et al. Prevention of Hereditary Angioedema Attacks with a Subcutaneous C1 Inhibitor. N Engl J Med. 2017;376(12):1131–1140. — COMPACT trial: twice-weekly subcutaneous C1-INH reduced attacks compared with placebo. — PubMed PMID: 28328347
10. Farkas H, et al. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency. Allergy. 2017;72(2):300–13. — International expert consensus on pediatric HAE, addressing special considerations for children. — Search PubMed
11. Riedl MA, et al. Efficacy and safety of plasma-derived C1-esterase inhibitor for acute hereditary angioedema attacks during 2 open-label studies. Ann Allergy Asthma Immunol. 2012;108(1):49–55. — Open-label confirmation of pdC1-INH safety and effectiveness across attack locations. — Search PubMed
12. Zuraw B, Lumry WR, Johnston DT, et al. Oral once-daily berotralstat for the prevention of hereditary angioedema attacks: A randomized, double-blind, placebo-controlled phase 3 trial. J Allergy Clin Immunol. 2021;148(1):164–172.e9. — APeX-2 trial establishing berotralstat as the first daily oral HAE prophylaxis option. — PubMed PMID: 33098856
13. Craig TJ, Reshef A, Li HH, et al. Efficacy and safety of garadacimab, a factor XIIa inhibitor for hereditary angioedema prevention (VANGUARD): a global, multicentre, randomised, double-blind, placebo-controlled, phase 3 trial. Lancet. 2023;401(10382):1079–1090. — PubMed PMID: 36868261
14. Riedl MA, Tachdjian R, Lumry WR, et al. Efficacy and Safety of Donidalorsen for Hereditary Angioedema. N Engl J Med. 2024;391(1):21–31. — PubMed PMID: 38819395
15. Riedl MA, Farkas H, Aygören-Pürsün E, et al. Oral Sebetralstat for On-Demand Treatment of Hereditary Angioedema Attacks. N Engl J Med. 2024;391(1):32–43. — PubMed PMID: 38819658
16. Maurer M, Magerl M, Betschel S, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update. Allergy. 2022;77(7):1961–1990. — PubMed PMID: 35006617
17. Bork K. Hereditary angioedema with normal C1 inhibitor: clinical symptoms, triggered by oral contraceptives and during pregnancy. Allergy. 2003;58(4):382–3. — Early characterization of Type 3 HAE (normal C1-INH) with estrogen as trigger. — Search PubMed

Search PubMed: Hereditary Angioedema & C1 Inhibitor
Search PubMed: Bradykinin Angioedema Treatment
Search PubMed: Lanadelumab / Icatibant HAE Prophylaxis

Back to Table of Contents

Connections

• Immunology
• Common Variable Immunodeficiency (CVID)
• X-Linked Agammaglobulinemia
• Wiskott-Aldrich Syndrome
• Chronic Granulomatous Disease
• Pain and Allergy
• Dermatology
• All Conditions

Back to Table of Contents

Featured Videos