Description

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.


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