Comparison of Different Diagnostic Guidelines for the Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: Single Centre Experience.

Comparison of Different Diagnostic Guidelines for the Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: Single Centre Experience. Acta Clin Croat. 2018 Jun;57(2):307-311 Authors: Jelušić M, Kronja M, Frković M, Sršen S, Huljev Frković S, Štekić Novački K Abstract Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA), caused by exaggerated but ineffective immune response. The aim of the study was to compare the capacity of the HLH-2004 guidelines with the capacity of the MAS guidelines from 2005, and with the new set of classification criteria from 2016 in diagnosing MAS complicating sJIA. The study included 35 children aged 1-18 diagnosed with sJIA according to ILAR criteria and treated at the Department of Pediatrics, Division of Immunology and Rheu-matology, Zagreb University Hospital Centre, in the period from 2009 to 2015. Out of 35 patients diagnosed with sJIA, there were 12 girls and 23 boys, with the mean age at disease onset (±SD) 5.51±3.65 years. Eight patients had flare of disease. With the guidelines from 2005, MAS was diag-nosed in six (17.1%) patients with sJIA. With the new set of classification criteria from 2016, MAS was diagnosed in four (11.4%) patients with sJIA. MAS was not diagnosed with the HLH-2004 guidelines. In our study, four out of six patients had MAS at the onset of sJIA, and in the...
Source: Acta Clinica Croatica - Category: General Medicine Tags: Acta Clin Croat Source Type: research