The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency

Orphanet Journal of Rare Diseases

Henriette Farkas¹  Lilian Varga¹  George Fust¹  György Temesszentandrási¹  Zsuzsanna Nébenführer¹  Ibolya Czaller¹  Erika Szabó¹  Dorottya Csuka¹  Zsuzsanna Zotter¹ 

[1] 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary

关键词: Subcutaneous/submucosal attack;    Trigger factor;    C1-Inhibitor deficiency;    Hereditary angioedema;   

Others  :  863221
DOI  :  10.1186/1750-1172-9-44

 received in 2013-12-02, accepted in 2014-03-20,  发布年份 2014

【 摘 要 】

Background

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.

Methods

In 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.

Results

During 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%.

Conclusion

This 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.

【 授权许可】

   
2014 Zotter et al.; licensee BioMed Central Ltd.

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