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Procedure-Specific Risk of Acute Attacks in Hereditary Angioedema: A 410-Procedure Cohort Analysis.

Source: PubMed, NCBI / U.S. National Library of Medicine

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & ImmunologyAy Ecem, Kural Ragıp Fatih, Bahadir Adil, et al.Published 6/1/2026Last synced 6/13/2026Status: syncedPMID: 42229680DOI: 10.1016/j.anai.2026.05.030

Hereditary angioedema (HAE) is characterized by recurrent, potentially life-threatening attacks often triggered by surgical procedures. Standard risk assessments frequently overlook procedural invasiveness and tissue trauma, limiting individualized perioperative management. To evaluate procedure-specific risk of acute HAE attacks and assess prophylactic strategy effectiveness across diverse surgical and interventional categories. This retrospective cohort study analyzed 410 procedures in 58 patients with C1-inhibitor-deficient HAE. Interventions were categorized as major surgery, minor surgery, diagnostic, obstetric, or minimally invasive cosmetic procedures based on invasiveness and airway involvement. Attacks within 72 hours post-procedure were recorded. Overall, procedure-related attacks occurred in 12.9% (53/410) of cases. Major surgery carried the highest risk (34.7%, 17/49), with attack rates reaching 47.8% (11/23) without prophylaxis versus 23.1% (6/26) with prophylaxis. Minor surgery showed an 8.9% (4/45) attack rate; notably, no attacks occurred in patients receiving prophylaxis (0/12) compared to 13.0% (3/23) without it. For circumcision, the rate was 13.3% overall. Diagnostic procedures had an 18.18% (2/11) attack rate, occurring only without prophylaxis. Minimally invasive cosmetic procedures posed the lowest risk (1.94%, 5/258). Statistical analysis confirmed major surgery risk significantly exceeded minor surgery (p=.023) and cosmetic procedures (p<.001). Additi

Abstract

Hereditary angioedema (HAE) is characterized by recurrent, potentially life-threatening attacks often triggered by surgical procedures. Standard risk assessments frequently overlook procedural invasiveness and tissue trauma, limiting individualized perioperative management. To evaluate procedure-specific risk of acute HAE attacks and assess prophylactic strategy effectiveness across diverse surgical and interventional categories. This retrospective cohort study analyzed 410 procedures in 58 patients with C1-inhibitor-deficient HAE. Interventions were categorized as major surgery, minor surgery, diagnostic, obstetric, or minimally invasive cosmetic procedures based on invasiveness and airway involvement. Attacks within 72 hours post-procedure were recorded. Overall, procedure-related attacks occurred in 12.9% (53/410) of cases. Major surgery carried the highest risk (34.7%, 17/49), with attack rates reaching 47.8% (11/23) without prophylaxis versus 23.1% (6/26) with prophylaxis. Minor surgery showed an 8.9% (4/45) attack rate; notably, no attacks occurred in patients receiving prophylaxis (0/12) compared to 13.0% (3/23) without it. For circumcision, the rate was 13.3% overall. Diagnostic procedures had an 18.18% (2/11) attack rate, occurring only without prophylaxis. Minimally invasive cosmetic procedures posed the lowest risk (1.94%, 5/258). Statistical analysis confirmed major surgery risk significantly exceeded minor surgery (p=.023) and cosmetic procedures (p<.001). Additionally, cosmetic procedures carried lower risk than minor (p=.046) and diagnostic interventions (p=.043). HAE attack risk follows an invasiveness-dependent gradient. Major surgeries and obstetric procedures carry the highest risk, while minor and aesthetic interventions demonstrate significantly lower rates, emphasizing the need for stratified prophylactic protocols.

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