Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose:
Macrophage activation syndrome (MAS) – is a severe life-threatening hematological condition, mostly complicated systemic juvenile idiopathic arthritis (SJIA). Early detection of MAS can lead to appropriate therapeutic interventions and change the outcomes. There are no strict criteria for early MAS detection in SJIA. Currently applied HLH criteria can determinate only advanced stage of MAS, which lead to delay diagnosis, late start of specific treatment and associated with poor outcomes. There are several sets of preliminary criteria of MAS in SJIA.
The aim of our study was to detect early clinical and laboratorial signs able to discriminate MAS from active SJIA without MAS.
Methods:
our retrospective study was based on reviewing of medical charts of children who were admitted to our rheumatology department in 2005-2013 with SJIA and definite MAS (n=18) and active SJIA without MAS (n=40). We utilized the A.Ravelli criteria (2002, 2005, 2011) for detecting MAS. We evaluated demographic data, data related to SJIA and MAS. We used the main characteristic clinical and laboratorial markers of MAS only at the moment of MAS confirmation. We calculated cutoff points for MAS parameters (ROC-analysis), performed analysis of sensitivity and specificity and identified predictors.
Results:
Several clinical signs were relevant to MAS in SJIA: oligoarthicular disease course (OR=5.6 [95%CI:1.6-19.4], p=0.005), splenomegaly (OR=67.6 [3.8-1205.9], p=0.000004), coagulopathy (OR=7.0 [1.9-26.1], p=0.006), lung (OR=11.3 [2.8-45.2], p=0.0001) and kidney involvement, realized in proteinuria<1.0g/24 h (OR=42.1 [2.2-801.2], p=0.0001). The involvement of wrist (OR=0.2 [0.1-0.8], p=0.03), MCP (OR=0.1 [0.0-0.9], p=0.02) and PIP joints (OR=0.1 [0.0-0.6], p=0.005) were protective against MAS development. The best cutoffs for laboratorial parameters, related to MAS are in table.
|
Parameter |
Sensitivity |
Specifisity |
OR (95%CI) |
AUC (95%CI) |
p |
|
Hb≤90 g/l |
72.2 |
80.0 |
10.4 (2.9-37.8) |
0.77 (0.64-0.87) |
0.0001 |
|
WBC≤9.9*109/l |
83.3 |
90.0 |
35.0 (7.4-165.6) |
0.92 (0.81-0.97) |
0.0000001 |
|
PLT≤211*109/l |
88.9 |
100.0 |
534.6 (24.3-1747.8) |
0.98 (0.9-0.997) |
0.0000001 |
|
ALT>72.9 U/l |
64.7 |
85.0 |
10.4 (2.8-38.9) |
0.81 (0.68-0.9) |
0.0002 |
|
AST>59.7 U/l |
82.4 |
92.1 |
54.4 (9.8-302.9) |
0.88 (0.76-0.95) |
0.0000001 |
|
LDH>882 U/l |
75.0 |
100.0 |
158.3 (7.9-3169.1) |
0.91 (0.79-0.976) |
0.0000001 |
|
GGTP>35 U/l |
83.3 |
60.0 |
7.5 (1.2-47.1) |
0.68 (0.47-0.85) |
0.047 |
|
ALP>736.2 |
27.3 |
100.0 |
30.1 (1.4-638.4) |
0.58 (0.43-0.72) |
0.01 |
|
Total protein≤63g/l |
64.7 |
97.5 |
71.5 (7.8-658.5) |
0.84 (0.71-0.92) |
0.0000001 |
|
Albumin≤29.3 g/l |
100.0 |
92.5 |
375.0 (18.4-7661.8) |
0.98 (0.9-0.997) |
0.0000001 |
|
Prothrombin≤77% |
71.4 |
92.3 |
30.0 (2.9-313.5) |
0.81 (0.61-0.93) |
0.0008 |
|
Fibrinogen≤1.8 g/l |
64.3 |
100.0 |
46.6 (2.3-947.8) |
0.88 (0.7-0.97) |
0.001 |
|
Ferritin>400 μg/l |
100.0 |
76.0 |
87.0 (4.5-1671.7) |
0.92 (0.78-0.98) |
0.000005 |
|
CRP>113 mg/l |
52.9 |
89.5 |
9.6 (2.3-39.1) |
0.59 (0.45-0.72) |
0.001 |
|
ESR≤10 mm/h |
61.1 |
92.5 |
19.4 (4.3-87.8) |
0.78 (0.65-0.88) |
0.0001 |
|
Na+ ≤137 mmol/l |
66.7 |
81.1 |
8.0 (2.1-31.0) |
0.79 (0.65-0.0.89) |
0.003 |
|
Active joints≤6 |
83.3 |
59.0 |
7.2 (1.8-29.0) |
0.74 (0.61-0.85) |
0.003 |
Conclusion:
we detected clinical and laboratorial markers which can help to early recognition of
MAS in children with active SJIA.
Disclosure:
M. Kostik,
None;
M. Dubko,
None;
L. Snegireva,
None;
V. Masalova,
None;
T. Kornishina,
None;
N. Abramova,
None;
I. Chikova,
None;
N. Glebova,
None;
E. Kuchinskaya,
None;
E. Balbotkina,
None;
O. Kalshnikova,
None;
V. Chasnyk,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/identification-of-best-cutoff-points-and-clinical-signs-specific-for-early-recognition-of-macrophage-activation-syndrome-in-active-systemic-juvenile-idiopathic-arthritis/