Hereditary angioedema and post traumatic stress disorder: a reciprocal relationship?

BACKGROUND

Hereditary angioedema (HAE) patients endure repeated unpredictable trauma associated with attacks of disfiguring swelling, severe abdominal pain, risk of asphyxiation or experiences with loss of a loved one with HAE. Our study investigated the prevalence of presumptive post-traumatic stress disorder (PTSD) for HAE due to C1 inhibitor deficiency (HAE-C1INH).

RESULTS

Participants ≥ 18 with HAE-C1INH were recruited from the US HAE Association: Cohort 1, self-referred; Cohort 2, randomized from the HAEA membership. Participants completed 8 on-line questionnaires: (1) HAE survey; (2) SF-36; (3) Childhood Trauma Questionnaire (CTQ); (4) Beck Depression Inventory-II (BDI-II); (5) Life Events Checklist form for HAE; (6) PTSD checklist-civilian version (PCL-C); (7) Peritraumatic Dissociative Experiences Questionnaire (PDEQ) and (8) Peritraumatic Distress Inventory (PDI). The prevalence of presumptive PTSD based on the PLC-C outcomes was between 20 and 25% in HAE-C1INH participants (n = 77). Significant score correlations were found between: PLC-C and BDI-II (p < 0.0001, R2 = 0.23), total CTQ (p = 0.001, R2 = 0.13), PDEQ (p < 0.0001, R2 = 0.23) and PDI (p < 0.0001, R2 = 0.39). SF-36 total mental health scores were significantly associated with presumptive PTSD at all PLC-C cut-off scores as were all mental health subdomains, early age of HAE symptom onset and requiring emergency department visits for HAE attacks.

CONCLUSION

Our study identified a high prevalence of presumptive PTSD among HAE-C1INH participants. Diagnosis was correlated with high rates of depression and reduced SF-36 mental domain performance. The interrelationship between PTSD and HAE may represent reciprocal risk factors enhancing disease severity.