D2.36 - Clinical Outcomes Of Dental Procedures In Patients With Hereditary Angioedema

The study included patients diagnosed with HAE between 1999 and 2024 at a tertiary care adult allergy clinic. Patients underwent retrospective analysis for information regarding dental procedures and the occurrence of AE attacks.

Hereditary angioedema (HAE) is an autosomal dominant disorder caused by a deficiency or dysfunction of C1-esterase inhibitor (C1-INH), resulting in increased vascular permeability mediated by bradykinin. Dental procedures have been identified as potential triggers for acute angioedema (AE) attacks. This study aims to investigate the correlation between AE attacks following dental procedures in HAE patients and to identify associated risk factors.

In total, 28 out of 102 HAE patients were excluded from the study due to incomplete data. The study includes 74 patients, of whom 45 (60.8%) were female. Nineteen (25.7%) patients were receiving long-term prophylactic treatment. Of these, 16 (21.6%) were using danazol, while 3 (4.1%) were using plasma-derived C1-inhibitor concentrate (pdC1-INH).

A total of 85 dental procedures were performed on 47 (63.5%) patients. The most common procedures included restorations (n= , 34.1%), tooth extractions (n= , 50.6%), and root canal treatments (n= , 11.8%). Pre-procedural angioedema prophylaxis was administered in 46 (54.1%) procedures predominantly consisting of pdC1-INH concentrate prophylaxis (n= 78.2%), danazol (n= 15.2%), or icatibant (n= 6.6%). A total of 17 (20%) angioedema attacks were observed, all of which occurred in the oropharyngeal region. There was a significant difference in attack frequency between the groups receiving prophylaxis and those not receiving it (p = 0.022). Attack frequency between patients receiving pdC1-INH concentrate prophylaxis (n=36) and those receiving danazol prophylaxis (n=7) (P= 0.572) was similar. Attack frequency between patients receiving 500 IU (n=9) and those receiving 1000 IU (n=27) of pdC1-INH concentrate prophylaxis was also similar (11.1% and 14.8% attacks, respectively p= 0.781). 

Preprocedural prophylactic treatment significantly reduced the frequency of AE attacks associated with dental procedures. In our cohort, there was no significant difference in the frequency of AE attacks between pdC1-INH and danazol prophylaxis groups. It was notable that the effectiveness of 500 IU, and 1000 IU of pdC1-INH concentrate in preventing dental procedure-related attacks was similar.