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Valeriya Yudina

Sechenov University , Russian Federation

Presentation Title:

Using ILAR and PRINTO criteria in sJIa diagnostics

Abstract

Introduction: Systemic idiopathic juvenile arthritis (sJIA) is an acute and severe autoinflammatory disorder characterized by fever, rash, generalized lymphadenopathy, hepato- and/or splenomegaly, serositis, arthritis, or arthralgias. To verify diagnosis sJIA doctors use the ILAR and PRINTO criteria. According to ILAR criteria all patients should have an arthritis at disease onset. But not all the patients develop arthritis at the onset of the disease, which complicates the diagnosis of sJIA.

Objectives: to identify specificity and sensitivity of both criteria (ILAR and PRINTO) for sJIA

Materials and methods: We’ve analyzed both criteria for children with verified diagnosis sJIa, n=78. As the group of control, we took a database of the history of disease of children with sjia-like symptoms such as (fever, rash, lymphadenopathy, hepato or/and splenomegaly, serositis, arthritis), n=32, with the following diagnoses% multisystem inflammatory disease (61,54%), juvenile idiopathic arthritis (12,82%), Kawasaki disease (10,26%), diffuse connective tissue diseases (5,13%), acute lymphoblastic leukemia (5,13%), reactive arthritis (2,56%), lymphoma (2,56%).  In purpose to identify which of criteria (ILAR or PRINTO) has more specificity and sensitivity we’ve used roc-analyse.

Results: The ILAR criteria are the most accepted for diagnosing sJIA, however, the absence of a mandatory clinical sign - arthritis in a number of patients at the onset of the disease does not allow diagnosing sJIA at the onset of the disease, which led to the development of new PRINTO criteria, in which arthritis is not a mandatory criterion. Comparative analysis of these criteria on the study group of patients showed that when assessing the ILAR criteria, the odds ratio is 6.366 (CI 95% 2.356-17.200; p <0.001). At the same time, this model has a higher specificity - 84.2%, sensitivity - 54.4%. The overall accuracy of the model was 65.1%. The AUC indicator for this model was 0.693±0.052 (CI 95% 0.591 - 0.795; p = 0.001), which indicates the average quality of the prognostic model. When using PRINTO criteria in patients, the odds ratio of having sJIA in a patient is 9.230 (CI 95% 2.753–30.947; p <0.001). According to the results of constructing the ROC curve, the AUC indicator was 0.644±0.059 (CI 95% 0.812-0.759; p=0.012), which corresponds to the average quality of the prognostic model. At the same time, the model has 94.8% sensitivity in terms of predicting whether patients belong to the sJIA group. The specificity of the model was 34.2%. The overall percentage of correct predictions reaches 74.3%. When evaluating the combined use of ILAR and PRINTO criteria, the model with high sensitivity predicts the presence of sJIA in a patient in 98.5% of cases, while the specificity of the model was 34.2%. The overall percentage of correct predictions is 75.5%. According to the results of constructing the ROC curve, the AUC indicator was 0.775±0.048 (CI 95% 0.681-0.869; p<0.001), which indicates the good quality of the prognostic model.

Conclusion: When performing a regression analysis for the PRINTO and ILAR criteria for sJIA, the following conclusions can be made:
• PRINTO criteria have the highest sensitivity of 94.8% versus 54.4% for ILAR.
• ILAR criteria have a higher specificity, which is 84.2% versus 34.8% for PRINTO criteria
• When using both criteria simultaneously, the sensitivity increases to 98.5%.
Taking into account the obtained results, it can be concluded that the PRINTO criteria are the most inclusive, however, the specificity of these criteria is lower than that of the ILAR criteria, and therefore, for higher sensitivity, it is recommended to use both criteria together.

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