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Start Over Descriptor "Macrophage Activation Syndrome pathology" Journal pediatric rheumatology online journal
2 results on '"Macrophage Activation Syndrome pathology"'

1. Proceedings from the 2 nd Next Gen Therapies for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome symposium held on October 3-4, 2019.

Author

Canna SW, Schulert GS, de Jesus A, Pickering A, Brunner H, Gadina M, Levine S, Goldbach-Mansky R, Boutelle J, Sinha R, DeBenedetti F, and Grom A

Subjects
Arthritis, Juvenile diagnosis, Arthritis, Juvenile pathology, Biomarkers, Child, Child, Preschool, Disease Progression, Humans, Infant, Macrophage Activation Syndrome diagnosis, Macrophage Activation Syndrome pathology, Arthritis, Juvenile therapy, Macrophage Activation Syndrome therapy
Abstract

For reasons poorly understood, and despite the availability of biological medications blocking IL-1 and IL-6 that have markedly improved overall disease control, children with Systemic Juvenile Idiopathic Arthritis (SJIA) are now increasingly diagnosed with life-threatening chronic complications, including hepatitis and lung disease (SJIA-LD). On October 3-4, 2019, a two-day meeting, NextGen Therapies for Systemic Juvenile Idiopathic Arthritis (SJIA) & macrophage activation syndrome (MAS) organized by the Systemic JIA Foundation ( www.systemicjia.org/ ) in Washington, DC brought together scientists, clinicians, parents and FDA representatives with the objectives (1) to integrate clinical and research findings in MAS and SJIA-LD, and (2) to develop a shared understanding of this seemingly new pulmonary complication of SJIA. The current manuscript summarizes discussions and conclusions of the meeting.

Published
2020
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2. Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.

Author

Shimizu M, Mizuta M, Okamoto N, Yasumi T, Iwata N, Umebayashi H, Okura Y, Kinjo N, Kubota T, Nakagishi Y, Nishimura K, Mohri M, Yashiro M, Yasumura J, Wakiguchi H, and Mori M

Subjects
Case-Control Studies, Child, Child, Preschool, Female, Humans, Macrophage Activation Syndrome classification, Macrophage Activation Syndrome etiology, Macrophage Activation Syndrome pathology, Male, Retrospective Studies, Treatment Outcome, Antibodies, Monoclonal, Humanized therapeutic use, Antirheumatic Agents therapeutic use, Arthritis, Juvenile complications, Macrophage Activation Syndrome drug therapy
Abstract

Background: This study aimed to determine the influence of tocilizumab (TCZ) in modifying the clinical and laboratory features of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA). Furthermore, we assessed the performance of the 2016 MAS classification criteria for patients with s-JIA-associated MAS while treated with TCZ., Methods: A panel of 15 pediatric rheumatologists conducted a combination of expert consensus and analysis of real patient data. Clinical and laboratory features of s-JIA-associated MAS in 12 TCZ-treated patients and 18 untreated patients were evaluated. Possible MAS was defined as having characteristic laboratory features but lack of clinical features of MAS, or atypical MAS, or early treatment that prevented full-blown MAS., Results: Clinically, the TCZ-treated patients with s-JIA-associated MAS were less likely febrile and had significantly lower ferritin, triglyceride, and CRP levels than the untreated patients with s-JIA-associated MAS. Other laboratory features of MAS including lower platelet counts and lower fibrinogen were more pronounced in TCZ-treated patients. The TCZ-treated patients with s-JIA-associated MAS were less likely to be classified as MAS based on the MAS classification criteria (25% vs 83.3%, p < 0.01). This is ascribed to the absence of fever or insufficient ferritin elevation, compared with the untreated patients., Conclusion: TCZ could modify the clinical and laboratory features of s-JIA-associated MAS. When evaluating the s-JIA patients while treated with TCZ, it is not applicable to use MAS classification criteria. Care must be taken to not underdiagnose MAS based on the MAS classification criteria.

Published
2020
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