Saccomanno et al reported a score for evaluating extra-articular systemic manifestations in a patient with systemic juvenile idiopathic arthritis. This can serve as a measure of disease activity. The authors are from multiple institutions in Italy and Brazil.
Patient selection: systemic juvenile idiopathic arthritis
Parameters:
(1) temperature in °C (fever)
(2) evanescent erythematous rash
(3) generalized lymphadenopathy (>= 3 lymph node stations)
(4) hepatomegaly and/or splenomegaly
(5) serositis (pleuritis, pericarditis, or peritonitis)
(6) hemoglobin in g/dL (anemia)
(7) platelet count in 10^9/L
(8) serum ferritin in ng/mL
Parameter |
Finding |
Points |
body temperature |
< 37°C |
0 |
|
37 to 38 °C |
1 |
|
38.1 to 39 °C |
2 |
|
39.1 to 40 °C |
3 |
|
> 40°C |
4 |
erythematous rash |
no |
0 |
|
yes |
1 |
generalized lymphadenopathy |
no |
0 |
|
yes |
1 |
hepatomegaly or splenomegaly |
no |
0 |
|
yes |
1 |
serositis |
no |
0 |
|
yes |
1 |
hemoglobin |
>= 9 g/dL |
0 |
|
< 9 g/dL |
1 |
platelet count or ferritin |
PC <= 600 * 10^9/L AND serum ferritin <= 500 ng/mL |
0 |
|
PC > 600 * 10^9/L |
1 |
|
serum ferritin > 500 ng/mL |
1 |
where:
• The temperature cut-points as published overlapped at 38°C and 39°C.
• Tibaldi et al used a temperature range of 37.5 to 38°C instead of 37 to 38°C
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 10
• The higher the score the greater the systemic activity.
Specialty: Immunology/Rheumatology