Expert Opinion on Symptoms, Diagnosis, Treatment, and Clinical Cases-
Hereditary angioedema (HAE) is a rare, autosomal dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. HAE is usually associated with low levels or impaired function of C1 inhibitor.1 The rarity and complexity of the presentation can be easily mistaken for allergic and non-allergic reactions alike. Furthermore, a laboratory diagnosis of HAE requires an understanding of the underlying mechanisms of the different types of HAE. Although 50% of patients become symptomatic by age 10, accurate diagnosis is often delayed by decades.2 Traditional treatment options, which may include anabolic steroids and antifibrinolytics, are associated with troublesome side effects.3 However, new medications are available for prophylaxis and acute treatment and can now be incorporated into patient-centric treatment plans for HAE. Through increased awareness of HAE symptoms, diagnostics, and new and emerging therapies, healthcare professionals will be able to reduce the time to diagnosis and improve patient outcomes.
Describe important clinical features of hereditary angioedema (HAE)
Differentiate HAE from allergic angioedema
Identify diagnostic considerations in HAE
Develop treatment plans for acute HAE attacks and prophylaxis
Outline treatment goals for HAE patients
Hereditary angioedema (HAE) is a rare, autosomal dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. HAE is usually associated with low levels or impaired function of C1 inhibitor.1 The rarity and complexity of the presentation can be easily mistaken for allergic and non-allergic reactions alike. Furthermore, a laboratory diagnosis of HAE requires an understanding of the underlying mechanisms of the different types of HAE. Although 50% of patients become symptomatic by age 10, accurate diagnosis is often delayed by decades.2 Traditional treatment options, which may include anabolic steroids and antifibrinolytics, are associated with troublesome side effects.3 However, new medications are available for prophylaxis and acute treatment and can now be incorporated into patient-centric treatment plans for HAE. Through increased awareness of HAE symptoms, diagnostics, and new and emerging therapies, healthcare professionals will be able to reduce the time to diagnosis and improve patient outcomes.
Describe important clinical features of hereditary angioedema (HAE)
Differentiate HAE from allergic angioedema
Identify diagnostic considerations in HAE
Develop treatment plans for acute HAE attacks and prophylaxis
Outline treatment goals for HAE patients
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