Original ArticlePreliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
Section snippets
Study Design
To identify the features that were suitable candidates as diagnostic criteria for MAS, we followed the classification criteria approach.9, 10 The purpose of this approach is to separate patients with a disease from patients without the disease. Classification criteria, ideally, have a high sensitivity rate for the disease in question and a high specificity rate against other diseases (that is, a high proportion of cases of the disease are found to be positive and a high proportion of cases
Results
Table I shows the frequency of clinical, laboratory, and histopathological features in patients with S-JIA, and MAS who were reported in the literature and were seen at the authors' units and in patients with S-JIA who had active systemic disease. The frequency of features observed in our patients with MAS and in patients reported in the literature was comparable, with the exceptions of bilirubin increase and erythrocyte sedimentation rate decrease, which were more common in patients reported
Discussion
There are no formal and universally accepted criteria for the diagnosis of MAS in S-JIA. The recognition that this syndrome bears close resemblance to the clinical and laboratory picture of hemophagocytic lymphohistiocytosis (HLH) has led many clinicians to use in practice the diagnostic guidelines for this disease.35 There are, however, several problems with the use of HLH criteria in patients with MAS, the first of which is the need for tissue confirmation. As noted in patients with HLH36 and
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