Demirkaya et al reported the International Severity Scoring System (ISSF) for a patient with familial Mediterranean Fever (FMF). The authros are from the FMF Arthritis Vasculitis and Orphan disease Research in pediatric rheumatology (FAVOR).
Patient selection: Familial Mediterranean Fever (FMF)
Parameters:
(1) chronic sequelae (amyloidosis, growth retardation, anemia, splenomegaly)
(2) FMF-related organ dysfunction (nephrotic range proteinuria, other)
(3) FMF-related organ failures (heart, renal, other)
(4) average frequency of attacks per month
(5) acute phase reactants (serum amyloid A, erythrocyte sedimentation rate, fibrinogen), >= 2 weeks after the last attack, measured at least 2 times 1 month apart
(6) number of sites involved during an individual attack (pericarditis, pleuritis, peritonitis, erysipelas-like erythema, testis involvement, myalgia, etc)
(7) different types of attacks during the course of the disease (fever, pericarditis, pleuritis, peritonitis, erysipelas-like erythema, testis involvement, myalgia, etc)
(8) duration of attacks
(9) exertional leg pain (pain after exercising and/or after prolonged standing, not due to other cause)
|
Parameter |
Finding |
Points |
|
chronic sequelae |
absent |
0 |
|
|
present |
1 |
|
FMF-related organ dysfunction |
absent |
0 |
|
|
present |
1 |
|
FMF-related organ failure |
absent |
0 |
|
|
present |
1 |
|
average frequency of attacks per month |
< 1 |
0 |
|
|
1 to 2 |
1 |
|
|
> 2 |
2 |
|
acute phase reactants |
absent |
0 |
|
|
present |
1 |
|
number of sites involved |
0 to 2 |
0 |
|
|
>= 3 |
1 |
|
different types of attacks during course of disease |
0 to 2 |
0 |
|
|
>= 3 |
1 |
|
duration of attacks |
> 72 hours in >= 3 attacks in a year |
1 |
|
|
other |
0 |
|
exertional leg pain |
absent |
0 |
|
|
present |
1 |
total score =
= SUM(points for all 9 parameters)
Interpretation:
• minimum score: 0
• maximum score: 10
• The higher the score the greater the disease severity.
|
Total Score |
Disease Severity |
|
0 to 2 |
mild |
|
3 to 5 |
intermediate |
|
6 to 10 |
severe |
Specialty: Immunology/Rheumatology, Genetics, Infectious Diseases, Pedatrics
