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Dissecting the heterogeneity of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.
- Source :
-
The Journal of rheumatology [J Rheumatol] 2015 Jun; Vol. 42 (6), pp. 994-1001. Date of Electronic Publication: 2015 Apr 15. - Publication Year :
- 2015
-
Abstract
- Objective: To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey.<br />Methods: International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course.<br />Results: A total of 362 patients were included by 95 investigators from 33 countries. Demographic, clinical, laboratory, and histopathologic features were comparable among patients seen in diverse geographic areas or by different pediatric specialists. Patients seen in North America were given biologics more frequently. Patients entered by pediatric hemato-oncologists were treated more commonly with biologics and etoposide, whereas patients seen by pediatric rheumatologists more frequently received cyclosporine. Patients with demonstration of hemophagocytosis had shorter duration of sJIA at MAS onset, higher prevalence of hepatosplenomegaly, lower levels of platelets and fibrinogen, and were more frequently administered cyclosporine, intravenous immunoglobulin (IVIG), and etoposide. Patients with severe course were older, had longer duration of sJIA at MAS onset, had more full-blown clinical picture, and were more commonly given cyclosporine, IVIG, and etoposide.<br />Conclusion: The clinical spectrum of MAS is comparable across patients seen in different geographic settings or by diverse pediatric subspecialists. There was a disparity in the therapeutic choices among physicians that underscores the need to establish uniform therapeutic protocols.
- Subjects :
- Adolescent
Age Distribution
Arthritis, Juvenile diagnosis
Child
Child, Preschool
Cohort Studies
Comorbidity
Databases, Factual
Female
Humans
Internationality
Macrophage Activation Syndrome diagnosis
Male
Multivariate Analysis
Prevalence
Prognosis
Retrospective Studies
Severity of Illness Index
Sex Distribution
Survival Analysis
Arthritis, Juvenile epidemiology
Arthritis, Juvenile therapy
Macrophage Activation Syndrome epidemiology
Macrophage Activation Syndrome therapy
Subjects
Details
- Language :
- English
- ISSN :
- 0315-162X
- Volume :
- 42
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- The Journal of rheumatology
- Publication Type :
- Academic Journal
- Accession number :
- 25877504
- Full Text :
- https://doi.org/10.3899/jrheum.141261