Download - Angioedema 11/12/2010 BY: MOHAMMED ALSAIDAN
Angioedema 11/12/2010
BY: MOHAMMED ALSAIDAN
Angioedema
• Abrupt and short-lived swelling of the skin, mucous membranes, or both including the upper respiratory and intestinal epithelial linings
• The swelling is nonpitting, erythematous or skin-colored
• Areas where the skin is lax
• Pain is variable, but rarely itching.
• There is no desquamation or staining of the skin although scratching or rubbing may cause bruising.
Pathophysiology
• Increase in permeability of the submucosal or subcutaneous capillaries and postcapillary venules, causing local plasma extravasation and consequent swelling.
• A variety of vasoactive molecular mediators , by mast cells:• Preformed ? synthesized ?
• There is a paucity of cellular infiltration in angioedema.
Epidemiology
• 49% of all patients with urticaria also had angioedema
• Angioedema occurred in 93 of 107 (87%) patients with chronic urticaria
• Women are more frequently affected than men
• Most commonly affects those 40 to 50 years old
Acute allergic angioedema
• Almost always accompanied by urticaria , within 1 to 2 hours of exposure to the offending allergen.
• It is commoner in patients who are atopic or allergic to foods or medications
• Pathophysiology = urticaria
• (the g-chains of FceR1 tyrosine kinase activation protein kinase C activation increase in intracellular calcium mast cell degranulation )
Acute allergic angioedema
Acute allergic angioedema
• Clinical features: swelling can occur anywhere
• Risk of anaphylaxis
• Skin prick test vs. RAST
TREATMET• ABC• EPI PEN
Adult Mild angioedema without signs of circulatory compromise0.3-0.5 mg of 1:1000 SCModerate-to-severe angioedema with signs of shock:Adult dose is 0.3-0.5 mg of 1:10,000 IV Pediatric0.15-0.3 mg (depending on the patient's weight) of
1:1000 solution SC• Diphenhydramine (50 mg) I.V. or I.M • Hydrocortisone (200 mg) I.V• 24 h. Observation
NSAID-induced angioedema• Clinical picture resemble allergic urticaria or angioedema and
are often termed ‘‘pseudoallergic”
• Only COX 1 inhibitors cause pseudoallergic angioedema
• Skin prick testing has no value
• urticaria/angioedema to NSAIDS vs. NSAID-induced asthma
• Rx: Emergency measures are as for acute allergic angioedema +/- leukotriene antagonists?
Angioedema of ACE inh.
• Not associated with urticaria
• Usually involve face + orophaynx
• Incidence = 0.1% to 0.2% and is 5 x more common in African Americans than white patients
• The most common cause of acute angioedema
• Rx: as for allergic but with risk of relapses after recovery
Angioedema of ACE inh.
• Angiotensin II receptor antagonists are tolerated by patients who have reacted to ACE inhibitors
• Screen for HAE
Physical urticarias
Idiopathic acquired angioedema
• Chronic and relapsing, and usually associated with urticaria
• In 30% to 50% : urticaria and the angioedematous lesions are a result of an autoimmune process
• Angioedema and urticaria occurred together in 49% to 87% and angioedema alone in 9 to 11%
• Pathophysiology = idiopathic urticaria
• ASST might be helpful, basopenia
Idiopathic acquired angioedema
• Avoid provoking factors :• Aspirin• Overtiredness• Overexcitement• overvigorous exercise• alcohol overconsumption
• Antihistamines vs. steroids vs. others?
Gleich syndrome
• Episodic angioedema, urticaria, fever, weight gain, peripheral blood and skin eosinophilia
• Patients responded well to systemic steroids
• Probably closely related to the hypereosinophilic syndrome
Hereditary angioedema (HAE)• Dominantly inherited defect in chromosome 11 (11q12-
q13.1)
• Affects about 1:50,000 person
• (type 1) (The classic type) is a quantitative defect in (C1 INH)
• (type 2) is functional defect in (C1 INH)
• (type 3) in women with quantitatively and functionally normal C1 INH activity with a relationship to estrogenic activity
To be clinically expressed , the C1 INH plasma level should be quantitatively or functionally less than 40% of normal
Hereditary angioedema (HAE)• Increase in activation of C1, leading to consumption of C2 and
C4, high level of bradykinin
• Patients are usually asymptomatic up to puberty
• precipating factors• minor injury such as dental maneuvers (>50%)• vigorous exercise• alcohol consumption• emotional stress• hormonal factors
Hereditary angioedema (HAE)
• There could be transitory prodromal nonpruritic urticarial eruption in some patients, persist for 3 to 4 days
• Coadministration of ACE inhibitors and estrogens is contraindicated in HAE.
• Associated diseases:• Glomerulonephritis• Sjogren’s syndrome• thyroiditis,• Lupus• coagulopathies
Treatment
• ABCs (usually not life threatening)
• Antihistamines and corticosteroids are ineffective
• S.C. adrenaline (0.3 mg every 10 minutes) usually not effective but maybe helpful
• The mainstay treatment is: I.V. FFP or C1 inhibitor concentrate• 550 plasma U in a 10-mL vial to be administered at a dose of 25
plasma U/kg body weight to a total of 1000 plasma U repeated once if necessary, It is usually effective within 3 to 4 hours, and often within minutes.
Treatment
• There have been reports of improvement of acute symptoms with icatibant, a specific B2 kinin antagonist
• Anabolic steroids • increase the circulating levels of normal functional C1 INH in both
type 1 and type 2 HAE • risk of hepatotoxicity and liver adenomas• stanazolol 2-4 mg/d• danazol 50-300 mg/d• women : hirsutism, acne, menstrual cycle irrigularity, deep voice
Prophylaxis
• C1 inh concentrate before surgery, especially when intubation or tooth extraction is necessary
• For minor surgical procedures, tranexamic acid (1 g four times daily in adults or 500 mg four times daily in children) for 48 hours before and after the procedure
• increase in established maintenance doses of tranexamic acid or anabolic steroids
Acquired C1 inh. deficiency
• Type 1: immune complex mediated C1 and C1 inh consumption
• associated diseases :• B-cell lymphoma (the most common)• Other haematologic malignancies
• Type 2 : autoantibodies against C1 inh.
Treatment
• Treatment of the underlying disease
• Plasma or C1 inhibitor concentrate used for emergency
• For chronic disease:• Plasmapheresis • Cytotoxic agent • Androgenic compounds• e-aminocaproic acid• Tranexamic acid for type II
Thank You