Overview
- Definition: anaphylaxis is an acute, potentially fatal systemic hypersensitivity reaction caused by rapid, massive release of mediators from mast cells and basophils, producing multi-system involvement within minutes of exposure.
- Cardinal presentation: sudden urticaria or angioedema (present in about 90 percent) with respiratory compromise and/or hypotension after contact with an allergen.
- Why it is an emergency: it can kill quickly through upper-airway obstruction and distributive shock, and delayed epinephrine is the strongest predictor of death.
- First-line treatment: intramuscular epinephrine given immediately is the only intervention shown to reduce mortality; antihistamines and glucocorticoids are adjuncts, not substitutes.
- Late hazard: a biphasic reaction can recur hours after apparent resolution, so patients are observed.
Epidemiology
- Common triggers: foods (peanut, tree nuts, shellfish, milk, egg), Hymenoptera stings, drugs (beta-lactams, NSAIDs), and latex; a proportion is idiopathic.
- Age pattern: food is the leading trigger in children, whereas drugs and insect stings predominate in adults.
- At-risk groups: prior anaphylaxis, atopy, and asthma, which worsens severity.
Risk factors
- Prior anaphylaxis or known allergy: the strongest predictor of a future episode.
- Asthma, especially poorly controlled: associated with more severe, fatal reactions.
- Specific exposures: foods, Hymenoptera stings, beta-lactams and NSAIDs, latex, perioperative agents, and radiocontrast media.
- Mast cell disease: systemic mastocytosis or an elevated baseline tryptase.
- Beta-blocker or ACE inhibitor use: more severe reactions that are harder to treat.
Pathophysiology
- IgE-mediated mechanism (type I): allergen cross-links specific IgE on mast cells and basophils, triggering degranulation.
- Non-IgE direct activation: agents such as opioids, vancomycin, and radiocontrast can directly activate mast cells and produce an identical picture.
- Mediator release: histamine, prostaglandin D2, leukotrienes, and tryptase drive vasodilation, increased vascular permeability, and bronchoconstriction.
- Distributive shock: hypotension arises from peripheral vasodilation and capillary leak in a hyperdynamic state, not from impaired cardiac contractility.
Clinical features
- Skin and mucosa (about 90 percent): urticaria, flushing, pruritus, and angioedema.
- Respiratory: throat tightness, stridor, wheeze, dyspnea, and hypoxemia from laryngeal edema and bronchospasm.
- Cardiovascular: hypotension, tachycardia, syncope, and shock.
- Gastrointestinal: crampy abdominal pain, vomiting, and diarrhea.
- Tempo: onset within minutes to a few hours, and the faster the onset the more severe the reaction.
- Biphasic course: symptoms can recur hours after initial resolution without re-exposure.
Differential diagnosis
- Vasovagal syncope: bradycardia and pallor without urticaria, bronchospasm, or true shock.
- Bradykinin-mediated angioedema (hereditary or ACE inhibitor): swelling without urticaria or pruritus and a poor response to epinephrine (Angioedema).
- Acute asthma exacerbation: wheeze and dyspnea without urticaria or hypotension (Asthma).
- Septic or other distributive shock: fever and an infection source rather than acute allergen exposure (Sepsis).
- Scombroid poisoning: histamine from spoiled fish producing a flushing and urticarial mimic, but affecting others who ate the same fish and resolving without epinephrine.
- Panic attack or vocal cord dysfunction: subjective throat closure without objective skin or airway findings.
- Carcinoid syndrome: episodic flushing and diarrhea over a chronic course.
- Mast cell activation syndrome or mastocytosis: recurrent episodes with elevated tryptase.
- Acute coronary syndrome or arrhythmia: cardiovascular collapse without allergic features.
Diagnosis
- Diagnose clinically and treat first, using the standard criteria (any one of three fulfills the diagnosis):
- Acute onset over minutes to hours of skin or mucosal involvement plus either respiratory compromise or hypotension or end-organ dysfunction.
- Two or more of the following after exposure to a likely allergen: skin or mucosal signs, respiratory compromise, hypotension, or persistent gastrointestinal symptoms.
- Hypotension after exposure to a known allergen for that patient.
- Supportive laboratory test, serum tryptase: draw within 1 to 2 hours of onset (it peaks about 1 hour and can stay elevated for roughly 5 hours); an elevated level supports the diagnosis, but a normal level does not exclude it and is common in food-triggered anaphylaxis.
- After recovery, identify the trigger: refer to allergy for specific IgE or skin testing, and measure a baseline tryptase to screen for mastocytosis.
- Never delay treatment for testing: epinephrine is given on clinical grounds.
Clinical pearl. Anaphylaxis is a treat-first diagnosis: give intramuscular epinephrine the moment criteria are met. A normal tryptase never rules it out because it is frequently normal in food-triggered reactions, and waiting for a lab result or for a second organ system to declare itself is how patients die.
Management
- First, give intramuscular epinephrine to stabilize the patient: 0.3 to 0.5 mg IM (0.01 mg/kg, 1 mg/mL concentration) into the anterolateral thigh, repeated every 5 to 15 minutes as needed; there is no absolute contraindication in anaphylaxis.
- Position and oxygenate: lay the patient supine with legs elevated (avoid sudden upright posture, which can precipitate fatal empty-ventricle arrest) and give high-flow oxygen.
- Restore volume: 1 to 2 L of IV crystalloid bolus for hypotension.
- If refractory to repeated IM dosing: start an epinephrine IV infusion 0.05 to 0.5 mcg/kg/min, titrated to response, and add another vasopressor if shock persists.
- If on a beta-blocker and refractory: glucagon 1 to 5 mg IV over 5 minutes, then a 5 to 15 mcg/min infusion.
- Secure the airway early: prepare for intubation when there is stridor or airway angioedema.
- Add adjuncts (never in place of epinephrine): an H1 antihistamine (diphenhydramine 25 to 50 mg IV) with an H2 blocker (famotidine 20 mg IV) for cutaneous symptoms, inhaled albuterol for bronchospasm, and a glucocorticoid (methylprednisolone 1 to 2 mg/kg IV) with no acute mortality benefit.
- Observe for a biphasic reaction: monitor for 4 to 6 hours, longer after severe or refractory episodes.
- Discharge safely: prescribe two epinephrine autoinjectors, provide an anaphylaxis action plan, arrange allergy referral, and counsel trigger avoidance.
Complications
- Death: from upper-airway obstruction or refractory distributive shock, usually with delayed epinephrine.
- Biphasic reaction: recurrence hours after initial recovery.
- Hypoxic-ischemic injury and arrhythmia: consequences of prolonged hypoxia or shock.
- Protracted anaphylaxis: symptoms persisting despite treatment.
Prognosis
- Excellent with prompt epinephrine: outcomes are strongly tied to how quickly epinephrine is given.
- Fatal reactions cluster: in asthmatics, in adolescents and young adults with food allergy, and when treatment is delayed.
- Recurrence risk is lifelong: for the identified trigger, mandating a carried autoinjector.
Prevention
- Avoid the identified trigger: the foundation after allergy evaluation.
- Carry two epinephrine autoinjectors at all times: and train the patient and caregivers in their use.
- Venom immunotherapy for Hymenoptera-sting anaphylaxis: given over 3 to 5 years, reduces the risk of a future systemic reaction to roughly 5 percent.
- Document drug allergies and use graded challenge, premedication, or desensitization when an implicated agent is unavoidable.
- Optimize asthma control: to reduce the severity of any future reaction.
Key points
- Anaphylaxis is a clinical, treat-first diagnosis made by the three-criteria set, not by laboratory testing.
- Intramuscular epinephrine 0.3 to 0.5 mg in the anterolateral thigh, repeated every 5 to 15 minutes, is first-line and mortality-reducing; antihistamines and steroids are adjuncts only.
- Skin findings occur in about 90 percent, but their absence does not exclude anaphylaxis.
- Watch for biphasic reactions and observe for 4 to 6 hours.
- Discharge with two autoinjectors and allergy referral, and offer venom immunotherapy for sting-triggered anaphylaxis.