 Points from case  ? When to give Epi pen to patients with allergic Rxn’s/ angioedema  Documentation ( how to RTN to ER ?)  Admission criteria for allergic Rxn’s ( wouldn’t help with this case)  Beware bowel edema as manifestation of allergic Rxn  Earlier airway intervention  ?surgical

 Angioedema is characterized by painless, nonpruritic, nonpitting, and well- circumscribed areas of edema due to increased vascular permeability

 most apparent in the head and neck, including the face, lips, floor of the mouth, tongue, and larynx, but edema may involve any portion of the body  may involve the gastrointestinal tract, leading to intestinal wall edema

 This deficiency in functioning C1-INH leads to autoactivation of the complement system and release of kininlike mediators, resulting in edema of the subcutaneous or submucosal tissues C’ Pathway (-) C1-INH(-) kallikrein high molecular weight–kinogen bradykinin

 1) Hereditary angioedema (HAE)  2) Acquired angioedema (AAE)  3) Angioedema associated with allergic reactions (which is often associated with urticaria)  4) Angioedema secondary to medications  ACE / ARB  5) Idiopathic angioedema

 C1 Esterase Inhibitor Deficiency  3 Types  1) Low levels of C1-INH (80-85%)  2) Normal Levels but dysfunctional  3) Normal levels and function – only women?  X-linked dominant inheritance

 Precipitants of HAE angioedema  Mental and physical stress  Trauma  Dental or surgical procedures  Infections  Menstruation  Pregnancy  Oral contraceptives containing estrogens

 Epinephrine, corticosteroids, and antihistamines are NOT effective in patients with HAE, AAE, and ACE inhibitor–induced angioedema.  These agents are recommended as second-line therapy. (in cases of angioedema due to allergic causes, these medications are first-line therapies.)

 1 st line treatment  Vapor-heated C1-INH concentrate  ( U IV)  FFP ( may worsen attack?)  2U IV  Other  tranexamic acid  epsilon-aminocaproic acid ( inhibit plasmin – plays role in initiating C’ cascade)

 1) Hereditary angioedema (HAE)  2) Acquired angioedema (AAE)  3) Angioedema associated with allergic reactions (which is often associated with urticaria)  4) Angioedema secondary to medications  ACE / ARB  5) Idiopathic angioedema

 Rare  Type I – lymphomas / lymphoproliferative dz  Type II - autoantibodies ? cause

 1) Hereditary angioedema (HAE)  2) Acquired angioedema (AAE)  3) Angioedema associated with allergic reactions (which is often associated with urticaria)  4) Angioedema secondary to medications  ACE / ARB  5) Idiopathic angioedema

 Allergy-induced angioedema  an IgE-mediated hypersensitivity reaction  Causes  Medications  Food  Environmental allergens (insect bites)

 Vasopressin ( Level C)  4U bolus  10U diluted in 10mls ( titrate to effect)  Surviving Sepsis Campaign guidelines  Recommend an AVP dosage of 0.03– 0.04 IU/min, a recent study suggested that IU/min (4 IU/h)

 1) Hereditary angioedema (HAE)  2) Acquired angioedema (AAE)  3) Angioedema associated with allergic reactions (which is often associated with urticaria)  4) Angioedema secondary to medications  ACE / ARB  5) Idiopathic angioedema

 % of patients treated with ACE inhibitors develop angioedema  Idiosyncratic Rxn  14 fold increased risk in first month of treatment  Has occurred >1 yr after initiation

 94% of angioedemas in ED due to meds  Most of these due to ACE Inhibitors  As many as 22 % require intubation  11% mortality  ARB’s also cause but incidence unknown (case reports)  Mainly losarten

 Airway Intervention ( Zirkle et al 2000)  Increasing age  Symptoms ( eg. stridor, hoarseness, dyspnea)  Not correlated  Rapidity of onset of sx  Cause of angioedema  Gender  Previous history

 Preferred techniques  Awake nasotracheal ( orotracheal )  Cricothyrotomy  Tracheostomy