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- Minoia, F, et al.
(författare)
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Dissecting the heterogeneity of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
- 2015
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Ingår i: The Journal of rheumatology. - : The Journal of Rheumatology. - 0315-162X .- 1499-2752. ; 42:6, s. 994-1001
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Tidskriftsartikel (refereegranskat)abstract
- 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.
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| 3. |
- Struglics, André, et al.
(författare)
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Aggrecanase cleavage in juvenile idiopathic arthritis patients is minimally detected in the aggrecan interglobular domain but robust at the aggrecan C-terminus.
- 2012
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Ingår i: Arthritis and Rheumatism. - : Wiley. - 1529-0131 .- 0004-3591.
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: To understand aggrecan degradation in juvenile idiopathic arthritis (JIA), the pattern and abundance of aggrecan fragments in synovial fluid aspirates from JIA patients were analysed and compared with aggrecan fragments in synovial fluids from patients with other arthritides, juvenile knee injury and a knee-healthy reference group. METHODS: The concentration of sulphated glycosaminoglycans in synovial fluid was measured by the Alcian blue precipitation assay. Aggrecan fragments were purified by dissociative CsCl density gradient centrifugation, deglycosylated and analysed by Western blot using antibodies specific for either aggrecanase-derived ARGS, SELE and KEEE neoepitopes, or the aggrecan G3-domain. RESULTS: The concentration of sulphated glycosaminoglycans in JIA synovial fluids was significantly lower compared with the levels in fluids from OA (P<0.001), juvenile knee injury (P=0.006) and knee-healthy reference (P=0.022) groups. Western blot analysis detected KEEE, SELE, and G3 fragments generated by aggrecanase cleavage in the chondroitin sulphate-rich region of JIA aggrecan. The pattern of JIA aggrecan fragments was not identical to that in synovial fluids pooled from OA patients, although there were notable similarities. Surprisingly, aggrecanase-derived ARGS fragments were barely detectable in the JIA synovial fluids, in marked contrast to the levels of ARGS fragments in OA synovial fluids. CONCLUSIONS: Aggrecanases appear to cleave minimally in the interglobular domain of aggrecan in JIA patients despite robust levels of cleavage in aggrecan's chondroitin-sulphate rich region. The results suggest that unlike other arthritides, aggrecanase cleavage in the aggrecan interglobular domain might not be a major pathogenic event in JIA. © 2012 American College of Rheumatology.
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