Ozen et al modified the score of Pras et al for describing the severity of Familial Mediterranean Fever (FMF) in children. The authors are from Hacettepe University (Ankara), University of Duisburg-Essen and Charite University Hospital Berlin.
Differences from original Pras score:
(1) age range for age of onset truncated
(2) colchicine dose adjusted
Patient selection: pediatric patient with FMF
Parameters:
(1) age of onset in years
(2) number of attacks per month
(3) arthritis
(4) erysipelas-like erythema
(5) amyloidosis
(6) dose of colchicine
Parameter |
Finding |
Points |
age of onset |
< 6 years |
4 |
|
6 to 10 years |
3 |
|
11 to 20 years |
2 |
number of attacks |
< 1 per month |
1 |
|
1 or 2 per month |
2 |
|
> 2 per month |
3 |
arthritis |
none |
0 |
|
acute |
2 |
|
protracted |
3 |
erysipelas-like erythema |
no |
0 |
|
yes |
2 |
amyloidosis |
no |
0 |
|
yes |
3 |
dose of colchicine |
less than appropriate dose |
0 |
|
appropriate dose |
1 |
|
more than appropriate dose |
2 |
where:
• Starting colchicine dose: 0.5 mg/day if < 5 years old; 1.0 mg/day if 5-10 years; 1.5 mg/day if > 10 years old.
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 3
• maximum score: 27
• The higher the score the more severe the disease.
Total Score |
Severity |
3 to 5 |
mild |
6 to 9 |
moderate |
>= 10 |
severe |
Specialty: Immunology/Rheumatology