Description

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

 


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