Home|Journals Follow on Twitter| Subscribe to List

Directory for Medical Articles
 

Open Access

2



Toward the Development of New Diagnostic Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis

Sergio Davě, Bianca Lattanzi, Erkan Demirkaya, Silvia Rosina, Giulia Bracciolini, Antonia Novelli, Nicolino Ruperto, Alberto Martini, Randy Q Cron, Angelo Ravelli.

Cited by (8)

Abstract
Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) with features of hemophagocytosis, leading to coagulopathy, pancytopenia, and liver and central nervous system dysfunction. MAS is overt in about 10% of children with sJIA but may occur subclinically in another 30-40% of patients. The diagnosis of MAS in sJIA can be challenging because it may mimic a flare of the underlying disease or be confounded with an infectious complication or a medication side effect. So far, universally agreed upon diagnostic criteria for MAS are not available. A multinational collaborative effort is under way that is aimed to generate new criteria for diagnosing MAS as a complication of sJIA. The first step of the project has led to the identification of, by means of a Delphi survey, the clinical, laboratory and histopathologic features of MAS that were felt to be most important by a large sample of international pediatric rheumatologists with experience with MAS in children with sJIA. The diagnostic performance of potential criteria will be scrutinized further in the second phase of the study, through the analysis (ongoing) of real patient data. The final set of criteria is meant to be established in a consensus conference of experts using a combination of statistical and consensus formation techniques. The ultimate goal of the project is to develop a core set of criteria that is both highly sensitive and specific. These criteria will assist physicians in MAS diagnosis in children with sJIA and enable quick institution of appropriate therapy.

Key words: Macrophage activation syndrome, systemic juvenile idiopathic arthritis, hemophagocytic syndrome, hemophagocytic Iymphohistiocytosis, diagnostic criteria



Share this Article


Advertisement
Journal of Environmental and Occupational Science

SUBMIT YOUR ARTICLE NOW


ScopeMed Home
Follow ScopeMed on Twitter
BiblioCAM
Article Tools
eJPort Journal Hosting
About ScopeMed
License Information
Terms & Conditions
Privacy Policy
Suggest a Journal
Publisher Login
Contact Us

The articles in Scopemed are open access articles licensed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License (https://creativecommons.org/licenses/by-nc-sa/4.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.
ScopeMed is a Database Service for Scientific Publications. Copyright © ScopeMed® Information Services.
Scopemed Buttons