Epression than the general population, with 42.5 of patients showing at least
Epression than the general population, with 42.5 of patients showing at least mild symptoms of depression.36 Data suggest that patients with more severe disease are more likely to experience depression than those with mild or infrequent attacks.36 Patients with HAE are nearly twice as likely to report taking psychotropic or antidepressant medication compared with the general population.DIAGNOSISIdentifying HAE may be difficult because of variability in clinical presentation. HAE should be suspected in patients presenting with any of the characteristic symptoms, especially in the presence of a positive family history.25 Screening for genetic mutations is not generally performed for diagnostic reasons. Instead, laboratory testing is indicated for patients with suspected HAE. Biochemical markers used for the diagnosis of HAE are serum complement factor 4 (C4), C1-esterase inhibitor antigen, functional C1-esterase inhibitor, and C1q antigenic protein.25 The most cost-effective?2011 World Allergy Organizationmethod to screen for HAE is measurement of C4 levels.2 If C4 is normal, as can occur in some patients between attacks, the measurement should be repeated during an acute attack.25 Whether to obtain C1-esterase inhibitor antigen and functional C1-esterase inhibitor studies at the time of C4 collection or at a subsequent time can be based on the index of suspicion. Low levels of C4, C1-esterase inhibitor antigenic protein, and functional C1-esterase inhibitor are consistent with a type I HAE diagnosis but should be confirmed via a second measurement. Low levels of C4 and functional C1esterase inhibitor with normal or elevated C1-esterase inhibitor antigenic protein are indicative of type II HAE.25 C1q antigenic protein is normal in HAE.25 It should be noted that variability in symptoms is not related to PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/29072704 the levels of biochemical markers and that patients with lower levels of the markers outlined here do not necessarily exhibit more severe HAE symptoms than those with higher levels.2 A recent study, however, found a significant correlation between severity scores and baseline functional C1-esterase inhibitor levels, which suggests the potential significance of monitoring functional C1-esterase levels in relation to clinical disease course.TREATMENT APPROACHESThe goals of treatment for HAE are focused on PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/26240184 life-saving efforts, slowing the progression and severity of attacks, and reducing the BX795 biological activity number of attacks and their impact on patient quality of life. Because of large variations in clinical presenta-SGower et alWAO Journal ?February 2011, Supplementtion and severity of disease, HAE treatment is individualized and based on a close collaboration between physician and patient. The pharmacotherapy of HAE can be categorized into 3 approaches: acute treatment, short-term prophylaxis, and long-term prophylaxis. Acute treatment options for HAE include C1-esterase inhibitor (human or recombinant) replacement therapy, icatibant, or ecallantide (Table 1)2,9,25,39 ?48; however, it should be noted that not all agents are currently approved in all countries. Infusions of C1-esterase inhibitor concentrate have been shown to increase functional levels of C1-esterase inhibitor and C4 to near-normal levels.49 Icatibant is a bradykinin B2-receptor antagonist that reverses increased vascular permeability and inhibits vasodilation and extravasation.40 Ecallantide is a human plasma kallikrein inhibitor that treats HAE symptoms by directly inhibiting plasma kalli.
