Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


An International Consensus Survey of the Diagnostic Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis.

Davi4,  Sergio, Consolaro4,  Alessandro, Guseinova4,  Dinara, Pistorio4,  Angela, Martini3,  Alberto, Cron1,  Randy Q., Ravelli2,  Angelo

Children's Hospital of Alabama and University of Alabama at Birmingham, Birmingham, AL
IRCCS G. Gaslini and Università di Genova, Genova, Italy
IRCCS G. Gaslini and Università di Genova, Genova, Italy
IRCCS G. Gaslini, Genova, Italy

Background:

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of systemic juvenile idiopathic arthritis (sJIA). Because MAS is a serious condition that can follow a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are critical. However, diagnosis of MAS can be difficult and hard to distinguish from sepsis-like syndromes. In addition, subclinical forms of MAS in sJIA underscore the importance of establishing criteria sensitive enough to identify MAS from routine disease flare.

Objective:

To select the clinical, laboratory and histopathologic features which are more suitable as diagnostic criteria for sJIA-associated MAS through an international Delphi questionnaire survey.

Methods:

All members of the Paediatric Rheumatology International Trials Organisation (PRINTO), the Childhood Arthritis & Rheumatology Research Alliance (CARRA), and the Pediatric Rheumatology Collaborative Study Group (PRCSG) were submitted a questionnaire that listed the 28 most typical features of MAS and were first asked to select the 10 features that they deemed most important in the diagnosis of the syndrome, and then to rank order the 10 selected features by assigning 10 to the most important, and end with 1 as the least important.

Results:

Of the 505 pediatric rheumatologists who were contacted, 232 (45.9%) participated in the survey. The table shows, for the features selected by more than 30% of respondents, the percentage of investigators who selected the feature and its median rank.

Results are reported for all respondents and for respondents categorized by geographic area. Falling platelet count, hyperferritinemia, evidence of hemophagocytosis in the bone marrow, increased liver enzymes, and falling leukocyte count were the 5 most frequently selected features. Only evidence of hemophagocytosis in the bone marrow, hyperferritinemia, and persistent fever reached a median rank >7. Overall, investigators from Europe and other parts of the world tended to give more weight to clinical manifestations than North American investigators, whereas North American investigators appeared to rely more on laboratory abnormalities than investigators from Europe and other parts of the world.

Conclusions:

We identified the features of MAS that were agreed upon by the majority of international pediatric rheumatologists. The ability of each feature to discriminate MAS from potentially "confusable" conditions and the optimal diagnostic threshold for laboratory tests will be assessed through a large-scale data collection, which is ongoing. Altogether, these processes will lead to the development of a new and robust set of criteria for MAS complicating sJIA.

FeatureNo. (%) of respondents who selected the featureMedian rankMean (SD) rank% of respondents giving rank 8–10 to the feature% of respondents giving rank 5–10 to the feature
Falling platelet count201 (86.6)6.56.1 (2.3)24.661.6
Hyperferritinemia194 (83.6)76.5 (3.0)39.253.9
Bone marrow hemophagocytosis188 (81.0)96.9 (3.6)44.855.2
Increased liver enzymes174 (75)55.0 (2.4)1340.9
Falling leukocyte count172 (74.1)55.6 (2.5)20.346
Persistent continous fever >= 38°C158 (68.1)76.0 (3.4)30.240.1
Falling erythrocite sedimentation rate142 (61.2)65.5 (2.7)15.938.8
Hypofibrinogenemia142 (61.2)55.4 (2.4)12.936.6
Hypertriglic eridemia135 (58.2)55.1 (2.7)16.831
Central nervous system dysfunction104 (44.8)55.0 (2.9)11.623.7
Falling hemoglobin level100 (43.1)54.8 (2.3)4.323.3
Prolongation of clotting times81 (34.9)4.54.5 (2.3)4.717.2
Increased D-dimer76 (32.8)55 (2.6)7.319.4
Hemorragic manifestations72 (31.0)55.3 (3.0)1017.7
Liver enlargement71 (30.6)44.8 (2.8)7.314.2

To cite this abstract, please use the following information:
Davi, Sergio, Consolaro, Alessandro, Guseinova, Dinara, Pistorio, Angela, Martini, Alberto, Cron, Randy Q., et al; An International Consensus Survey of the Diagnostic Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :208
DOI: 10.1002/art.27977

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