Hereditary Angioedema in France: Symptoms, Causes & Treatment | aihealz
Allergy & Immunologysevere
Hereditary Angioedema.Care & specialists in France
In France, hereditary Angioedema is managed by allergy & immunologists. Hereditary angioedema (HAE) is a rare autosomal-dominant disease that produces unpredictable attacks of non-itchy, non-urticarial swelling of the skin, abdominal wall, larynx, and oropharynx, driven by uncontrolled bradykinin generation rather than histamine. Prevalence is approximately 1 in 50,000 worldwide (Bygum 2009), and over 90% of cases are caused by deficient (type 1) or dysfunctional (type 2) C1-esterase inhibitor (C1-INH).
Hereditary angioedema (ICD-10: D84.1, hereditary deficiency of complement component C1 inhibitor) is a bradykinin-mediated swelling disorder caused by inherited dysfunction or deficiency of C1-esterase inhibitor (C1-INH) — a serine protease inhibitor (serpin) encoded by SERPING1 that regulates the classical complement, contact, fibrinolytic, and coagulation pathways. Three classic phenotypes are recognized. HAE type 1 (85% of cases) features low antigenic and functional C1-INH from missense, nonsense, or splicing variants in SERPING1. HAE type 2 (15%) features normal antigenic but reduced functional C1-INH from mutations that produce a dysfunctional protein, typically at the reactive-center loop.
key facts
Prevalence
Approximately 1 in 50,000 in most populations (Bygum 2009); estimated 6,000-10,000 diagnosed cases in the United States and 25,000-50,000 worldwide
Demographics
Autosomal dominant; women and men equally affected genetically, but women experience more frequent and severe attacks because estrogens upregulate kininogenesis
Avg. age
First attack typically age 5-15; over 90% have first attack before age 30; median diagnostic delay 8-13 years from first attack
Global cases
Estimated 1 in 50,000 globally; under-recognition substantial — many countries have a fraction of expected prevalence diagnosed (HAEi 2022)
Specialist
Allergy & Immunology
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How you might notice it
The key symptoms of Hereditary Angioedema are: Recurrent attacks of non-pitting, non-itchy subcutaneous swelling of the hands, feet, face, genitals, or limbs that build over 12-36 hours and resolve over 2-5 days without treatment., Severe colicky abdominal pain with nausea, vomiting, and watery diarrhea from bowel-wall edema, often mimicking surgical abdomen — a frequent cause of unnecessary appendectomy or laparotomy before diagnosis., Laryngeal edema with throat tightness, hoarseness, dysphagia, stridor, and progressive airway obstruction — accounts for most pre-treatment deaths., A flat, serpiginous, non-pruritic skin rash (erythema marginatum) preceding 25-50% of attacks by hours to a day., Tingling, tightness, or fullness in the affected area as a prodrome 4-24 hours before visible swelling., Triggers identifiable in 60-70% of attacks — minor trauma (dental work, intubation, surgery), psychological stress, infections, menstruation, hormonal contraceptives or hormone replacement therapy, ACE inhibitors, and some antibiotics., Mood symptoms (irritability, fatigue, low mood) commonly preceding an attack, particularly in adolescents..
01Recurrent attacks of non-pitting, non-itchy subcutaneous swelling of the hands, feet, face, genitals, or limbs that build over 12-36 hours and resolve over 2-5 days without treatment.
02Severe colicky abdominal pain with nausea, vomiting, and watery diarrhea from bowel-wall edema, often mimicking surgical abdomen — a frequent cause of unnecessary appendectomy or laparotomy before diagnosis.
03Laryngeal edema with throat tightness, hoarseness, dysphagia, stridor, and progressive airway obstruction — accounts for most pre-treatment deaths.
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How it’s diagnosed
diagnosis
Diagnosis is biochemical and depends on three blood tests in the right clinical setting: serum C4, antigenic C1-INH, and functional C1-INH. In HAE type 1, both antigenic and functional C1-INH are below the lower limit of normal and C4 is low. In HAE type 2, antigenic C1-INH is normal or elevated but functional C1-INH is low and C4 is low. C4 alone is approximately 88-92% sensitive and 96% specific when measured outside an attack; sensitivity exceeds 98% during an attack. Acquired angioedema (AAE) shares low C4, low C1-INH function, and low antigenic C1-INH but classically also low C1q (versus normal C1q in hereditary disease). HAE with normal C1-INH (HAE-nC1INH) shows normal C4 and C1-INH testing; the diagnosis combines a strong clinical picture (recurrent bradykinin-pattern angioedema, family history, lack of response to antihistamines and steroids) with genetic testing for F12, PLG, KNG1, and other recognized genes. WAO/EAACI 2021 guidelines recommend confirmatory testing twice at least 4 weeks apart. Genetic testing is offered when the diagnosis remains uncertain, before family screening, or for HAE-nC1INH. Differential diagnosis includes mast-cell-mediated allergic angioedema (acute onset, often with urticaria, responsive to adrenaline), ACE inhibitor angioedema (clinically similar but acquired and reversible with drug withdrawal), and idiopathic histaminergic angioedema. Imaging is rarely required; abdominal CT during an attack shows characteristic bowel-wall edema with ascites that resolves within days.
Key tests
01
Serum C4Highly cost-effective screening test; low between attacks supports HAE diagnosis
02
Antigenic C1-esterase inhibitorDistinguishes HAE type 1 (low antigen) from HAE type 2 (normal/elevated antigen)
03
Functional C1-esterase inhibitorRequired to confirm HAE type 2 and to exclude HAE in patients with low antigen from non-genetic causes
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Treatment & cost
medical treatments
✓Plasma-derived C1-esterase inhibitor IV concentrate (Berinert, Cinryze) 20 IU/kg on-demand
✓Recombinant human C1-esterase inhibitor (Ruconest) 50 IU/kg IV on-demand
✓Icatibant 30 mg subcutaneously on-demand
✓Ecallantide 30 mg subcutaneously on-demand
surgical options
Emergency surgical airway (cricothyroidotomy or tracheostomy)Life-saving when performed in time; not a long-term solution. Modern on-demand therapy has dramatically reduced need for emergency surgical airway.
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Causes & risk factors
known causes
SERPING1 mutations causing C1-INH deficiency (HAE type 1)
More than 700 distinct pathogenic variants in SERPING1 (chromosome 11q12-q13.1) cause haploinsufficiency. C1-INH plasma levels and function fall to 5-30% of normal, freeing kallikrein and factor XIIa to cleave high-molecular-weight kininogen and release bradykinin.
SERPING1 mutations causing C1-INH dysfunction (HAE type 2)
Missense variants near the reactive-center loop produce a normally secreted but functionally impaired C1-INH protein. Antigenic levels are normal or elevated, but functional activity is below the lower limit of normal.
F12 mutations and other non-C1-INH genes (HAE-nC1INH)
Gain-of-function variants in F12 (factor XII), and pathogenic variants in PLG, KNG1, ANGPT1, MYOF, and HS3ST6 cause an HAE-like phenotype with normal C1-INH testing. Estrogen exposure and the early reproductive years predominate.
Estrogen exposure
Combined oral contraceptives, hormone replacement therapy, and pregnancy increase factor XII expression and worsen attack frequency, particularly in HAE-nC1INH. Stopping estrogen reduces attacks substantially in many women.
ACE inhibitor exposure
Angiotensin-converting enzyme inhibitors block bradykinin breakdown and convert subclinical HAE into severe recurrent attacks. ACE inhibitors are contraindicated in all forms of HAE.
Physical and emotional triggers
Dental procedures, surgery, intubation, sports trauma, infections (especially Helicobacter pylori, EBV, herpes simplex), psychological stress, menstrual cycle phase, and certain antibiotics (rarely) precipitate attacks in known patients.
risk factors
Family history of HAE or unexplained recurrent angioedema
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Living with it
01Carry two doses of on-demand therapy (C1-INH, icatibant, or ecallantide) at all times and ensure family or partner can recognize an attack and call for help.
02Avoid ACE inhibitors and combined oral contraceptives; consider progestin-only contraception if hormonal control is required.
03Schedule short-term prophylaxis with C1-INH concentrate before dental work, surgery, intubation, and endoscopy.
04Treat Helicobacter pylori infection if confirmed and attacks are predominantly abdominal.
05Wear medical identification stating diagnosis and treatment plan; carry an HAE emergency card.
06Enroll in an HAE patient registry and update emergency department care plans annually.
recommended foods
•Balanced Mediterranean-style diet with adequate calorie intake
•Adequate hydration to support fibrinolysis and reduce attack severity
•
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When to seek help
why see an allergy & immunology
Care should be coordinated through an allergy and immunology specialist with HAE expertise, ideally at a designated comprehensive care center. Specialists handle confirmatory testing, family cascade screening, individualized prophylaxis selection, pregnancy planning, dental and surgical pre-medication, and access to expensive targeted therapies.
01Laryngeal asphyxiation — historically responsible for one in four untreated HAE deaths; modern emergency on-demand therapy and self-administration training have largely prevented it.
02Unnecessary abdominal surgery (appendectomy, laparotomy) before diagnosis, occurring in up to 30% of patients in older series and contributing to diagnostic delay.
03Hypovolemic shock from third-space fluid losses during severe abdominal attacks.
04Adverse effects from chronic androgen therapy: weight gain, hirsutism, hepatic adenoma, dyslipidemia, mood change.
05Anaphylaxis after ecallantide (3-4% of doses) requires administration in monitored settings.
06Pregnancy complications including increased attack rate in the third trimester, particularly in HAE-nC1INH; pdC1-INH-supported deliveries are very safe.
HAE type 1 (low C1-INH antigen and function)85% of HAE cases. SERPING1 mutations produce truncated or rapidly degraded C1-INH protein; both antigenic level and functional activity are below the lower limit of normal.
HAE type 2 (normal antigen, low function)15% of HAE cases. SERPING1 missense mutations, typically near the reactive-center loop, produce a dysfunctional but normally-secreted C1-INH protein. Antigenic level is normal or even elevated; functional activity is reduced.
HAE with normal C1-INH (HAE-nC1INH)Approximately 5-10% of clinical HAE. Includes FXII-HAE, PLG-HAE, KNG1-HAE, and several other genetic subtypes plus a substantial proportion of unknown genetic origin (HAE-U). Predominantly affects women; estrogen exposure is a major trigger.
Acquired angioedema (AAE-1 and AAE-2)Not inherited but mechanistically related: C1-INH consumption by lymphoproliferative disorders (AAE-1) or anti-C1-INH autoantibodies (AAE-2). Usually presents after age 40 without family history and warrants malignancy workup.
Living with Hereditary Angioedema
Timeline
An acute attack treated early with on-demand C1-INH, icatibant, or ecallantide begins to improve within 30-90 minutes and fully resolves within 4-24 hours. Untreated attacks last 2-5 days. Long-term prophylaxis reaches steady-state attack reduction within 4-8 weeks of starting subcutaneous C1-INH, lanadelumab, or berotralstat. Quality of life typically improves within 3-6 months of effective prophylaxis.
Lifestyle
01Avoid ACE inhibitors lifelong; document the allergy/contraindication clearly in the medical record.
02Switch from combined oral contraceptives or estrogen-containing HRT to progestin-only or non-hormonal alternatives.
03Plan pregnancies with an allergist and high-risk obstetrician; pdC1-INH is the preferred on-demand and prophylactic option in pregnancy.
04Build a written action plan that lists triggers, on-demand therapy choice, dose, contact numbers, and escalation criteria.
05Educate household, school, workplace, and dentist about HAE and how to respond to laryngeal warning signs.
06Maintain a diary of attacks and triggers to refine prophylactic strategy and dose.
Daily management
01Take long-term prophylaxis on schedule (lanadelumab fortnightly, berotralstat daily, subcutaneous C1-INH twice weekly) without missed doses.
Complementary approaches
Helicobacter pylori eradicationIn patients with predominantly abdominal HAE attacks and confirmed H. pylori infection, eradication has been associated with reduced attack frequency in observational studies. Treat as per local antimicrobial guidance.
Tranexamic acid 1-3 g/dayAntifibrinolytic that reduces attack frequency modestly; cheap and widely available. Useful in HAE-nC1INH and as adjunct in resource-limited settings; thrombosis risk requires caution.
Choosing a doctor
Look for an allergist or immunologist who is part of a HAE comprehensive care center (US HAEA Angioedema Center of Excellence or equivalent international designation), follows at least 10 HAE patients, and has 24-hour access to on-demand therapy. Pediatric HAE warrants a pediatric allergist with HAE experience and an established airway plan.
Patient support resources
US HAE Association (HAEA) →Patient organization that runs Angioedema Centers of Excellence, supports research, and provides emergency-card and travel guidance.
HAE International (HAEi) →Global federation of HAE patient organizations and reference centers; multilingual disease information and country directory.
Hereditary angioedema (HAE) is a rare inherited disease that causes recurrent attacks of non-itchy swelling of the skin, abdomen, and airway driven by excess bradykinin. Most cases are due to deficient or dysfunctional C1-esterase inhibitor. It is treated with on-demand and long-term prophylactic therapies specific to bradykinin pathways.
How is HAE different from an allergic reaction?▾▴
Allergic reactions are histamine-mediated, present with urticaria and itch, and respond to adrenaline, antihistamines, and steroids. HAE is bradykinin-mediated, has no hives or itch, and does not respond to those drugs. Recognizing the difference is critical because using allergy drugs alone can delay life-saving HAE-specific treatment.
What causes HAE attacks?▾▴
Attacks are triggered by minor trauma, dental procedures, surgery, infections, psychological stress, menstruation, hormonal contraceptives, and ACE inhibitors. Spontaneous attacks without an identifiable trigger occur in about 30% of episodes. Recognizing personal triggers is part of long-term management.
Is HAE life-threatening?▾▴
Yes when laryngeal swelling occurs without treatment — historic mortality from asphyxiation was 25-30% of untreated patients. Modern self-administered on-demand therapy with C1-inhibitor concentrate, icatibant, or ecallantide has reduced HAE mortality close to general-population levels.
How is HAE diagnosed?▾▴
Diagnosis combines clinical history with three blood tests: C4 (low in 88-92% of patients between attacks), antigenic C1-INH (low in type 1, normal in type 2), and functional C1-INH (low in both). Tests are repeated after 4 weeks to confirm. Genetic testing identifies SERPING1 mutations and HAE with normal C1-INH variants.
Can HAE be cured?▾▴
There is no cure for the inherited gene defect, but modern treatment controls the disease so well that most patients live normal lives. Lanadelumab, subcutaneous C1-INH, and berotralstat reduce attack frequency by 44-95%; over 70% of lanadelumab patients have 12-month attack-free intervals.
What is on-demand treatment in HAE?▾▴
On-demand therapy is medication given at the start of an HAE attack to shorten its duration. Options are intravenous plasma-derived or recombinant C1-INH, subcutaneous icatibant (bradykinin B2 antagonist), and subcutaneous ecallantide (kallikrein inhibitor). All patients should carry two doses at all times.
When should long-term prophylaxis be considered in HAE?▾▴
Long-term prophylaxis is recommended when attacks are frequent, severe, or have involved the airway, when on-demand therapy is unavailable, or when quality of life is significantly impaired. Options include subcutaneous C1-INH, lanadelumab every 2 weeks, oral berotralstat daily, and (second-line) attenuated androgens.
Is HAE inherited?▾▴
Yes. HAE is autosomal dominant — each child of an affected parent has a 50% chance of inheriting the condition. About 25% of cases have no family history and arise from new (de novo) SERPING1 mutations or are HAE with normal C1-INH. Genetic counseling is recommended for family screening.
Can children have HAE?▾▴
Yes. Two-thirds of patients have their first attack between ages 5 and 15. Pediatric HAE is treated with the same agents as adult HAE; plasma-derived C1-INH is approved for all ages, and icatibant is approved in many countries down to age 2. Pediatric airway attacks require urgent action.
Why are ACE inhibitors dangerous in HAE?▾▴
ACE inhibitors block bradykinin breakdown by angiotensin-converting enzyme, raising bradykinin levels and provoking severe attacks even in mild HAE. They are contraindicated in HAE; angiotensin receptor blockers (ARBs) are generally tolerated and preferred when antihypertensive therapy is needed.
Are hormonal contraceptives safe in HAE?▾▴
Combined oral contraceptives containing estrogen worsen HAE attacks in many women and should be avoided. Progestin-only methods (mini-pill, implant, IUD, depot injection) are generally safe and may reduce attack frequency. Choice should be made with an allergist and gynecologist.
Can pregnancy affect HAE?▾▴
Pregnancy often changes attack frequency — some women improve, others worsen, particularly in the third trimester. Plasma-derived C1-INH is the on-demand and prophylactic option of choice in pregnancy. Vaginal delivery is preferred; cesarean section warrants C1-INH pre-medication. Breastfeeding is safe.
Do antihistamines help HAE?▾▴
No. HAE attacks are driven by bradykinin, not histamine. Antihistamines, corticosteroids, and adrenaline do not shorten HAE attacks. Relying on these drugs delays effective HAE-specific therapy and is associated with worse outcomes, especially in laryngeal involvement.
What is short-term prophylaxis for HAE procedures?▾▴
Short-term prophylaxis is C1-INH concentrate (1000-2000 IU IV) given 1-6 hours before a high-risk event such as dental work, intubation, endoscopy, or surgery. It reduces provoked attack rate from 20-30% to under 10%. Tranexamic acid and danazol are alternatives where C1-INH is unavailable.
How long does an HAE attack last?▾▴
Untreated attacks build over 12-36 hours and resolve over 2-5 days. Treated attacks improve within 30 minutes to 2 hours after on-demand C1-INH, icatibant, or ecallantide and fully resolve within hours rather than days.
Can stress cause HAE attacks?▾▴
Psychological stress is a recognized trigger in 60-70% of patients. Mechanisms likely involve cortisol and autonomic changes affecting the contact system. Stress management (CBT, mindfulness, exercise) reduces attack frequency in observational studies but is an adjunct, not a replacement for medical therapy.
Is acquired angioedema the same as HAE?▾▴
No. Acquired angioedema (AAE) is not inherited; it usually presents after age 40 and is caused by C1-INH consumption from lymphoproliferative disorders (AAE-1) or autoantibodies (AAE-2). C1q is typically low in AAE and normal in HAE, helping distinguish them; AAE requires malignancy workup.
What is HAE with normal C1 inhibitor?▾▴
HAE-nC1INH is a subtype with the same clinical picture but normal C4 and C1-INH testing. Gain-of-function mutations in F12, PLG, KNG1, ANGPT1, MYOF, and HS3ST6 have been identified. It is predominantly seen in women, is highly estrogen-responsive, and is treated with the same on-demand agents.
Are there oral medications for HAE?▾▴
Yes. Berotralstat (Orladeyo) 150 mg daily, approved in 2020, is the first oral plasma kallikrein inhibitor for long-term HAE prophylaxis, reducing attack rate by 44% versus placebo. Attenuated androgens (danazol) and tranexamic acid are older oral options used as second-line or in resource-limited settings.
How can family members be tested for HAE?▾▴
All first-degree relatives of a confirmed HAE patient should be offered C4 and C1-INH antigenic/functional testing, regardless of symptoms, and ideally before age 1 with confirmation after age 1. If the family mutation is known, genetic testing provides definitive diagnosis. Early identification saves lives by enabling pre-emptive emergency planning.
A flat, serpiginous, non-pruritic skin rash (erythema marginatum) preceding 25-50% of attacks by hours to a day.
05Tingling, tightness, or fullness in the affected area as a prodrome 4-24 hours before visible swelling.
06Triggers identifiable in 60-70% of attacks — minor trauma (dental work, intubation, surgery), psychological stress, infections, menstruation, hormonal contraceptives or hormone replacement therapy, ACE inhibitors, and some antibiotics.
07Mood symptoms (irritability, fatigue, low mood) commonly preceding an attack, particularly in adolescents.
08Headache and vomiting from gastrointestinal attacks or, rarely, transient cerebral edema.
09Pelvic and perineal swelling with severe pain in women; can mimic ovarian or appendicular pathology.
10Bladder or urethral swelling causing urinary retention in rare attacks.
early warning signs
•Erythema marginatum: a non-pruritic, serpiginous, blanching, ring-shaped rash that may precede an attack by hours
•Tingling, prickling, or tightness in a body area that goes on to swell
•Sudden unexplained mood change, anxiety, or fatigue several hours before a typical attack
•Vague abdominal discomfort that escalates rapidly into severe colic and vomiting
•Mild voice change or sensation of a lump in the throat — high-risk warning for laryngeal involvement
● emergency signs
•Voice change, hoarseness, stridor, difficulty swallowing saliva, or dyspnea — call emergency services and self-administer on-demand HAE therapy immediately; airway intervention before swelling progresses can be life-saving
•Severe abdominal pain with vomiting and hypotension — bowel-wall edema can cause hypovolemic shock from third-space losses
•Facial swelling involving the lips, tongue, or floor of mouth, even when initially mild — risk of rapid progression to airway obstruction
•Failure of an attack to begin to resolve within 2-4 hours of on-demand treatment — repeat dose and seek emergency care
•Any HAE attack in a child under 5 years — pediatric airway is small and progression can be rapid
04
Serum C1qDistinguishes acquired angioedema (AAE, often with low C1q) from hereditary angioedema (normal C1q)
05
Genetic testing (SERPING1; F12, PLG, KNG1, ANGPT1, MYOF, HS3ST6 for HAE-nC1INH)Confirms diagnosis when biochemical results are equivocal, identifies HAE-nC1INH, and supports family cascade screening
06
Abdominal CT or ultrasound during an attackDemonstrates bowel-wall thickening, ascites, and absence of obstruction — supports diagnosis when clinical picture is unclear
Outlook
Modern targeted therapy has transformed HAE outlook. Pre-1980s case series reported mortality from laryngeal asphyxiation in 25-30% of untreated patients; post-2010 cohorts on consistent on-demand therapy and long-term prophylaxis show mortality close to general-population rates. The HELP, COMPACT, and APeX-2 trials each demonstrated attack-rate reductions of 44-95% with modern prophylactic agents, and over 70% of patients on lanadelumab achieve a 12-month attack-free interval. Quality-of-life scores rise substantially: AE-QoL scores improve by 20-30 points on prophylaxis in trial data. Childbearing is generally safe with planning and pdC1-INH support. Major unmet needs include diagnostic delay, equitable access in low- and middle-income countries, and HAE with normal C1-INH, where biomarkers and trial evidence remain limited.
genetic
75% of patients have an affected parent; spontaneous SERPING1 mutations explain the remaining 25%. Any first-degree relative with recurrent swelling, unexplained abdominal pain, or sudden death from airway swelling raises suspicion.
Female sex (especially HAE-nC1INH)non-modifiable
Women have more frequent and severe attacks than men with the same mutation, driven by estrogen-mediated upregulation of the contact system. HAE-nC1INH affects women disproportionately.
Estrogens increase plasma factor XII and high-molecular-weight kininogen; combined hormonal contraception can transform a previously mild HAE phenotype into severe disease. Progestin-only contraception is generally safe.
ACE inhibitor therapymodifiable
Contraindicated in HAE. ACE inhibitors block bradykinin degradation and can precipitate severe, life-threatening laryngeal attacks even in patients previously well controlled.
Pediatric onsetnon-modifiable
Earlier first attack predicts a more severe lifetime course; over 50% of patients with childhood-onset HAE describe their disease as severe by adulthood.
Trauma and surgerymodifiable
Dental procedures, intubation, endoscopy, abdominal and pelvic surgery, and ENT procedures precipitate up to 30% of attacks. Pre-procedural short-term prophylaxis with C1-INH concentrate reduces attack rate to under 10%.
Helicobacter pylori infectionmodifiable
Several studies link H. pylori infection to increased HAE attack frequency. Eradication reduces attack rate in some patients, particularly those with predominantly abdominal attacks.
Vitamin D 800-2000 IU/day, especially in patients on long-term androgen therapy
foods to avoid
•Alcohol in excess — small association with attack frequency in observational data and interactions with danazol and tranexamic acid
•Smoking, which appears to increase attack frequency in some cohorts
•Recreational stimulants that raise blood pressure (cocaine, amphetamines), which can amplify symptoms
•Foods or supplements linked to attacks in the personal diary — variable between individuals
choosing the right hospital
01Functional and antigenic C1-INH testing available locally or by reliable referral
02On-site emergency stocks of plasma-derived or recombinant C1-INH, icatibant, and ecallantide where licensed
03Anesthesia and ENT cover for emergency airway
04Designated HAE comprehensive care or Angioedema Center of Excellence
05Patient education program with self-administration training and home-supply pathway
Essential facilities
HAEA Angioedema Centers of ExcellenceHAEi reference centers worldwideAllergy and immunology comprehensive care centersPediatric tertiary allergy unitsHigh-risk obstetrics services for pregnant HAE patients
02Inspect injection sites for redness or induration and rotate sites for subcutaneous therapies.
03Track every attack — location, time of onset, treatment time, response time — for clinic review.
04Keep on-demand therapy at room temperature in a labeled travel kit; replace before expiry.
05Hydrate well; rest after any minor trauma.
06Update the personal HAE emergency card with current treatment and contact details every year.
Exercise
Regular moderate exercise is encouraged and does not provoke attacks for most patients. Avoid contact sports that involve repeated facial trauma if attacks are frequent. Pre-treat with on-demand therapy before high-risk activities in particularly trigger-sensitive patients. Stress-management approaches (CBT, mindfulness) reduce psychological triggers in observational studies.