Abstract
BackgroundHereditary angioedema (HAE) resulting from C1-inhibitor deficiency is characterized by attacks of subcutaneous and submucosal edema. Many factors have been presumed to induce edema. Our study analyzed these factors in a fairly large patient population.MethodsIn the first stage of our study, we analyzed the data recorded by 92 subjects in their patient diaries over seven years. The second phase included 27 HAE patients, who had been completing the diary entry ‘Trigger factors’ every day for seven months whether or not they had experienced an attack.ResultsDuring the initial stage, 91% of the subjects described some factor possibly related to the onset of an attack. They could identify a trigger factor – most commonly (21%) mental stress – in 30% of the 3176 attacks. We found a significant (p < 0.001) difference in the distribution of the trigger factors of the edematous attacks of different locations. The 27 participants of the second phase identified 882 potential trigger factors and recorded 365 attacks. Of these, 246 (67%) occurred on days when the patients identified a potential trigger factor. The likelihood of edema-formation associated with the latter was as follows: menstruation – 63%, infection – 38%, mental stress – 26%, physical exertion – 25%, meteorological changes – 21%, fatigue – 17%.ConclusionThis analysis of the trigger factors explored, for the first time, their potential role in inducing HAE attacks. Our findings might open new perspectives in extending the indications for edema-prophylaxis, and could contribute to a better understanding of the pathomechanism of HAE attacks.
Highlights
Hereditary angioedema (HAE) due to C1-INH deficiency (HAE-C1-INH) is a rare autosomal dominant disorder
The objective of our study was to appraise the incidence of various trigger factors, along with their effect on edematous attacks
Each patient received a standardized Patient Diary. The latter was intended for recording the date of onset, severity, location, and potential trigger factors of the attacks according to standard criteria, along with the treatment received
Summary
Hereditary angioedema (HAE) due to C1-INH deficiency (HAE-C1-INH) is a rare autosomal dominant disorder. The deficiency of C1-INH may cause activation of four closely interrelated (complement, contact-kinin, coagulation, and fibrinolytic) enzyme cascade systems. This may lead to the release of bradykinin, resulting in recurrent, paroxysmal angioedema in the subcutis and/or in the submucosa of the gastrointestinal tract and of the upper airways. The diagnosis, management, and follow-up of HAE-C1-INH patients are guided by international recommendations and consensus guidelines based on the latest scientific findings [6,7,8]. Hereditary angioedema (HAE) resulting from C1-inhibitor deficiency is characterized by attacks of subcutaneous and submucosal edema. Many factors have been presumed to induce edema Our study analyzed these factors in a fairly large patient population
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