Dissecting the heterogeneity of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
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.
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.
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.
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2015 |
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| Erschienen: |
2015 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:42 |
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| Enthalten in: |
The Journal of rheumatology - 42(2015), 6 vom: 25. Juni, Seite 994-1001 |
| Sprache: |
Englisch |
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| Links: |
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| Themen: |
Comparative Study |
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| Anmerkungen: |
Date Completed 22.04.2016 Date Revised 02.06.2015 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.3899/jrheum.141261 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM248086626 |
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| 100 | 1 | |a Minoia, Francesca |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Dissecting the heterogeneity of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis |
| 264 | 1 | |c 2015 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
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| 500 | |a Date Completed 22.04.2016 | ||
| 500 | |a Date Revised 02.06.2015 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Comparative Study | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Multicenter Study | |
| 650 | 4 | |a HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS | |
| 650 | 4 | |a HEMOPHAGOCYTIC SYNDROMES | |
| 650 | 4 | |a MACROPHAGE ACTIVATION SYNDROME | |
| 650 | 4 | |a SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS | |
| 700 | 1 | |a Davì, Sergio |e verfasserin |4 aut | |
| 700 | 1 | |a Horne, AnnaCarin |e verfasserin |4 aut | |
| 700 | 1 | |a Bovis, Francesca |e verfasserin |4 aut | |
| 700 | 1 | |a Demirkaya, Erkan |e verfasserin |4 aut | |
| 700 | 1 | |a Akikusa, Jonathan |e verfasserin |4 aut | |
| 700 | 1 | |a Ayaz, Nuray A |e verfasserin |4 aut | |
| 700 | 1 | |a Al-Mayouf, Sulaiman M |e verfasserin |4 aut | |
| 700 | 1 | |a Barone, Patrizia |e verfasserin |4 aut | |
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| 700 | 1 | |a Enciso, Sandra |e verfasserin |4 aut | |
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| 700 | 1 | |a Magni Manzoni, Silvia |e verfasserin |4 aut | |
| 700 | 1 | |a Rumba-Rozenfelde, Ingrida |e verfasserin |4 aut | |
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