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ANAPHYLAXIS, URTICARIA, & ANGIOEDEMA
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ESSENTIALS OF DIAGNOSIS
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Anaphylaxis is a systemic reaction with cutaneous symptoms, associated with dyspnea, visceral edema, and hypotension Urticaria is characterized by large, irregularly shaped, pruritic, erythematous wheals Angioedema is painless, deep, subcutaneous swelling, often involving periorbital, circumoral, and facial regions These disorders may be diagnosed clinically, especially in the context of allergen exposure; detection of specific IgE or elevated serum tryptase can confirm the diagnosis
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General Considerations
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Certain allergens especially drugs, insect venoms, and foods may induce an IgE antibody response, causing a generalized release of mediators from mast cells and resulting in systemic anaphylaxis This potentially fatal condition affects both nonatopic and atopic persons Isolated urticaria and angioedema are more common cutaneous forms of anaphylaxis with a better prognosis Food allergies cause an estimated 150 fatalities per year in the United States, most cases being due to ingestion of peanuts, tree nuts, shellfish, and fish Common childhood food allergies such as milk, soy, wheat, and egg are often outgrown over time if strict avoidance is practiced -Lactam antibiotics may be involved in 400 800 fatalities per year, and stinging insect venom causes about 50 fatalities per year Chronic relapsing urticaria, angioedema, and, less commonly, anaphylaxis, however, are not always due to IgE-mediated hypersensitivity In a minority of cases perhaps 10% or less underlying systemic disorders such as systemic mastocytosis or subclinical infection or inflammatory disorders may be manifested by episodic urticaria or angioedema Idiopathic causes are commonly responsible for chronic or relapsing symptoms, suggesting that some cases may be associated with autoimmune processes including the production of histamine-liberating autoantibodies directed against Fc mast cell membrane receptors A review of 593 patients with recurrent episodes of anaphylaxis seeking medical attention at a university medical center revealed that most (70%) anaphylactic episodes in adults were classified as idiopathic in nature In contrast, the bulk (35 55%) of anaphylactic reactions in children are caused by food allergies Twenty percent of the population will experience urticaria or angioedema during their lifetime, and the estimated prevalence of idiopathic anaphylaxis is 34,000 patients in the United States
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NONALLERGIC RHINITIS 1 Vasomotor Rhinitis
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Vasomotor rhinitis can be the primary cause of chronic nasal congestion, rhinorrhea or, less commonly, sneezing paroxysms About one-fifth of patients who seek medical attention at an allergy/immunology subspecialty clinic complaining of chronic rhinitis will actually have nonallergic, vasomotor rhinitis; one-third of patients in whom allergic rhinitis is diagnosed will also have a vasomotor component to the disease Nonspecific nasal hyperreactivity leads to symptoms triggered by cold temperature, inhaled irritants, strong odors, spicy food, and other nonallergic environmental stimuli Intranasal corticosteroids (eg, fluticasone), azelastine, and ipratropium, and oral decongestants may be effective as monotherapy or in combination
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2 Nasal Polyposis
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Compared with the general population, nasal polyps are not found more frequently in patients with allergic rhinitis, but they are associated with aspirin sensitivity syndrome, chronic sinusitis, allergic fungal sinusitis, Churg-Strauss syndrome, and cystic fibrosis Benign inflammatory outgrowths of nasosinus mucosa, nasal polyps can lead to nasal airway obstruction, hyposmia, and chronic sinusitis Compared with placebo, topical corticosteroids have been shown to improve polyposis, but the response rate to topical agents alone is commonly inadequate and treatment with systemic corticosteroids is usually required Since concomitant chronic sinusitis is almost always present, cotreatment with appropriate antibiotics is usually recommended Some cases will require surgical intervention, and regardless of the treatment, recurrence is a frequent complication
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Clinical Findings
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A Symptoms and Signs
The manifestations are: (1) hypotension or shock from widespread vasodilation or dysrhythmia, (2) respiratory distress from bronchospasm or laryngeal edema, (3) gas-
Allergic & Immunologic Disorders
trointestinal and uterine muscle contraction, and (4) flushing, pruritus, urticaria, and angioedema
CMDT 2008
B Laboratory Findings
In vivo allergy skin testing and in vitro RAST testing can detect allergen-specific IgE for a variety of foods, hymenoptera (bee, wasp, hornet, fire ant) venom, latex, and some medicines Skin testing for food allergy is appropriate only if the patient has symptoms consistent with IgE-mediated allergy (eg, urticaria, angioedema, or anaphylaxis) within 2 hours after eating the suspect food Determination of serum tryptase can be used to identify recent anaphylactic reactions or other reactions due to systemic mast cell activation Tryptase is a mast cell-derived neutral protease with a half-life of 60 90 minutes Elevated tryptase levels have been associated with anaphylaxis, systemic mastocytosis, and non-IgE-mediated diseases characterized by mast cell degranulation ( anaphylactoid reactions ) Histamine is released during these disorders and has a very short serum half-life but may be briefly detectable during symptomatic periods If IgE-mediated hypersensitivity is not found and symptoms become relapsing or chronic (over 6 weeks in duration), a screening battery of laboratory tests may be done after a thorough history and physical examination Appropriate diagnostic testing should follow any positive findings on examination or review of systems Patients suffering from recurrent angioedema should also be tested for C1-esterase inhibitor deficiency Measuring a serum C4 level is an easy screening test for C1-esterase inhibitor deficiency/hereditary angioedema because it will be low in most cases
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