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Sensitivity and specificity of current diagnostic guidelines in children with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Authors
Journal
Pediatric Rheumatology
1546-0096
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Volume
9
Identifiers
DOI: 10.1186/1546-0096-9-s1-o5
Keywords
  • Oral Presentation
Disciplines
  • Medicine

Abstract

Sensitivity and specificity of current diagnostic guidelines in children with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis ORAL PRESENTATION Open Access Sensitivity and specificity of current diagnostic guidelines in children with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis Sergio Davì*, Bianca Lattanzi, Silvia Rosina, Erkan Demirkaya, Nicolino Ruperto, Alberto Martini, Randy Q Cron, Angelo Ravelli From 18th Pediatric Rheumatology European Society (PReS) Congress Bruges, Belgium. 14-18 September 2011 Background Early diagnosis of macrophage activations syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) may be challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. However, the diagnostic value of the guidelines for hemophagocytic lymphohis- tiocytosis (HLH) (1) or sJIA-associated MAS (2) has sel- dom been examined. Objective To investigate the sensitivity and specificity of diagnostic guidelines for HLH and sJIA-associated MAS in patients with sJIA who developed MAS. Methods The study sample included 155 children with sJIA who had MAS (diagnosed and treated as such by the attend- ing physician) and 2 control groups with potentially “confusable” conditions, including active sJIA without MAS (n=303) and a systemic febrile infection requiring hospitalization (n=191). Diagnostic guidelines for HLH and sJIA-associated MAS were applied to all MAS and control patients. Because no patient had NK-cell activity and soluble CD25 determination available and bone marrow aspirate was performed in only a few patients, these 3 criteria were excluded from HLH guidelines. HLH criteria were, therefore, met when at least 4 of the 5 remaining variables were present. sJIA-associated MAS criteria were met when at least 2 laboratory criteria or at least 1 laboratory criterion and 1 clinical criterion were present. Sensitivity and specifi

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