Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report

Table 1 Quality of Life and PRO assessments to evaluate disease burden and control

Assessment	Application	Number of items	Recall period(s)	Advantages	Disadvantages
Angioedema Activity Score (AAS) [51]	Recurrent angioedema	5	24 h	Brief Good internal consistency	Prospective nature limits compliance
HAE Activity Score (HAE-AS) [52]	HAE-C1-INH	12	1 month and 6 months	Allows for assessment of attack variability over time	Requires accurate recall over 6 months
Angioedema Quality of Life (AE-QoL) Questionnaire [57]	Recurrent angioedema	17	4 weeks	Good psychometric properties Good internal consistency	Time-consuming Not HAE specific
Hereditary angioedema quality of life (HAE-QoL) [61]	HAE-C1-INH	25	6 months	HAE specific Good internalconsistency	Time-consuming Requires accurate recall over 6 months
United States Hereditary Angioedema Association Quality of Life (HAEA-QoL) Survey [65]	HAE-C1-INH	27	NR	NR	Validation studies are needed
36-Item Short-Form Health Survey (SF-36) [54, 93]	Generic health status	36	1 week and 4 weeks	Useful for comparisons	Less specific Low sensitivity
EuroQol 5-Dimensions Survey (EQ-5D) [54, 94]	Generic health status	5	No recall period	Useful for comparisons Easy to administer	Less specific Low sensitivity
Angioedema Control Test (AECT) [59]	Recurrent angioedema	4	4 weeks and 3 months	Brief Simple scoring	Validation studies are needed

HAE, hereditary angioedema; HAE-C1-INH, HAE with C1-esterase inhibitor deficiency; NR, not reported; PRO, patient-reported outcome

ISSN: 1710-1492