Abstract
The aim of treatment of hereditary angioedema (HAE) due to C1 esterase inhibitor deficiency (HAE-C1-INH) is either treating acute attacks or preventing attacks by using prophylactic treatment. For treating acute attacks, plasma-derived C1 inhibitor (C1-INH) concentrates, a bradykinin B2 receptor antagonist, and a recombinant human C1-INH are available in Europe. In the United States, a plasma-derived C1-INH concentrate, a bradykinin B2 receptor antagonist, and a plasma kallikrein inhibitor were approved for the treatment of acute attacks. Fresh frozen plasma is also available for treating acute attacks. Short-term prophylactic treatment focuses on C1-INH and attenuated androgens. Long-term prophylactic treatments include attenuated androgens such as danazol, stanozolol, and oxandrolone, antifibrinolytics, and a plasma-derived C1-INH concentrate. Plasma-derived C1-INH and a bradykinin B2 receptor antagonist are admitted for self-administration and home therapy. So the number of management options increased considerably within the last few years thus helping to diminish the burden of HAE.
Highlights
With regard to pathogenesis, clinical picture, and therapy
Hereditary angioedema caused by C1 inhibitor deficiency
Hereditary angioedema caused by C1 inhibitor deficiency ––Type I ––Type II Hereditary angioedema with normal C1 inhibitor ––Hereditary angioedema caused by factor XII gene mutations ––Hereditary angioedema with unknown genetic background Angioedemas caused by acquired C1 inhibitor deficiency Angioedema caused by ACE inhibitor or other drugs
Summary
Angioedemas (former terms: angioneurotic edema, Quincke’s edema) are demarcated edemas that last for 1 – 7 days and recur at irregular intervals. Angioedemas are edemas of the subcutaneous tissue, and hives are edemas of the dermis. Both have to be considered manifestations of a common underlying pathomechanism at different locations (“histamine-mediated angioedemas”). Numerous cases of asphyxia have been described of this form of the disease, and the quality of life of many patients is impaired due to frequent edematous attacks. For this disease, almost all steps of pathogenesis from the underlying genetic defect to the clinical symptom “angioedema” could be elucidated. Hereditary angioedema caused by C1 inhibitor deficiency ––Type I (reduced activity and concentration of C1 inhibitor in the plasma) ––Type II (reduced activity with normal or increased concentration of C1 inhibitor in the plasma) Hereditary angioedema with normal C1 inhibitor ––Hereditary angioedema caused by factor XII gene mutations ––Hereditary angioedema with unknown genetic background Angioedemas caused by acquired C1 inhibitor deficiency Angioedema caused by ACE inhibitor or other drugs
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