Agostoni et al reported criteria for evaluation of disease severity in a patient with hereditary angioedema based on findings over the previous year. This can help identify patients who may benefit from more aggressive or novel management. The authors are members of PREHAEAT (which stands for Novel Methods of Predicting, Preventing and Treating Attacks in Patients with Hereditary Angioedema).
Evaluation period: over the previous year
Parameters:
(1) duration of mild attacks (those that do not disrupt normal daily activities
(2) duration of moderate attacks (those that reduce normal daily activities without causing complete disability)
(3) duration of severe attacks (those which are totally disabling)
(4) emergency treatments
(5) long term prophylaxis
time for each type of attack in days =
= (average time per event in hours) * (number of events) / 24
|
Parameter |
Finding |
Points |
|
mild attacks |
|
0.5 * (number of mild attack days) |
|
moderate attacks |
|
(number of moderate attack days) |
|
severe attacks |
|
2 * (number of severe attack days) |
|
emergency treatments |
none |
0 |
|
|
conservative |
5 * (number of treatments) |
|
|
invasive |
25 * (number of treatments) |
|
long term prophylaxis |
< 3 months |
0 |
|
|
3 to 6 months |
12.5 |
|
|
> 6 months |
25 |
where:
• Conservative treatment involves infusion of FFP or C1-INH.
• Invasive treatment includes intubation or tracheostomy.
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: > 100
|
Total Score |
Severity Class |
Descriptor |
|
0 |
5 |
asymptomatic |
|
1 to 10 |
4 |
minimal |
|
11 to 20 |
3 |
mild |
|
21 to 30 |
2 |
moderate |
|
> 30 |
1 |
severe |
Specialty: Immunology/Rheumatology
ICD-10: ,
