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Macrophage Activation Syndrome

A serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T-LYMPHOCYTES and MACROPHAGES. It is seen predominantly in children with systemic onset JUVENILE IDIOPATHIC ARTHRITIS.
Also Known As:
Syndrome, Macrophage Activation
Networked: 537 relevant articles (38 outcomes, 27 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Juvenile Arthritis (Juvenile Idiopathic Arthritis)
2. Adult-Onset Still's Disease
3. Systemic Lupus Erythematosus (Libman-Sacks Disease)
4. Hemophagocytic Lymphohistiocytosis (Hemophagocytic Syndrome)
5. Sepsis (Septicemia)

Experts

1. Grom, Alexei A: 21 articles (08/2021 - 09/2003)
2. Shimizu, Masaki: 19 articles (01/2021 - 09/2010)
3. Yachie, Akihiro: 17 articles (01/2021 - 09/2010)
4. Canna, Scott W: 15 articles (02/2022 - 07/2013)
5. Schulert, Grant S: 13 articles (01/2022 - 04/2014)
6. Ravelli, Angelo: 11 articles (10/2022 - 03/2010)
7. Cron, Randy Q: 10 articles (01/2022 - 02/2011)
8. Mizuta, Mao: 10 articles (01/2021 - 10/2015)
9. Nakagishi, Yasuo: 10 articles (01/2021 - 05/2012)
10. Inoue, Natsumi: 9 articles (01/2021 - 10/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Macrophage Activation Syndrome:
1. Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link
2. Cyclosporine (Ciclosporin)FDA LinkGeneric
3. Adrenal Cortex Hormones (Corticosteroids)IBA
4. Biomarkers (Surrogate Marker)IBA
5. SteroidsIBA
6. canakinumabFDA Link
7. tocilizumab (atlizumab)FDA Link
8. Interleukin-1 (Interleukin 1)IBA
9. MethylprednisoloneFDA LinkGeneric
10. InterleukinsIBA
12/08/2022 - "It is often associated with an early onset (<2 years of age), macrophage activation syndrome and high interleukin (IL)-18 circulating levels. "
10/01/2019 - "Overproduction of interleukin (IL)-18 is closely related to the pathogenesis of adult-onset Still's disease (AOSD) and the development of macrophage activation syndrome (MAS), a life-threating complication of AOSD. "
07/01/2020 - "interleukin-1β-driven macrophage activation syndrome vs. interleukin-6-driven immune dysregulation). "
12/01/2023 - "Interleukin (IL)-18, a pro-inflammatory cytokine, is relatively underestimated as a therapeutic target, despite accumulated evidence indicating the unique roles of IL-18 in acute and chronic inflammatory conditions, such as macrophage activation syndrome. "
10/01/2020 - "Participants with DNMT3A or TET2 CHIP-driver sequence variations displayed increased expression of interleukin 1β (no CHIP-driver sequence variations, 1.6217 normalized Unique Molecular Identifiers [nUMI]; DNMT3A, 5.3956 nUMI; P < .001; TET2, 10.8216 nUMI; P < .001), the interleukin 6 receptor (no CHIP-driver sequence variations, 0.5386 nUMI; DNMT3A, 0.9162 nUMI; P < .001;TET2, 0.5738 nUMI; P < .001), as well as the NLRP3 inflammasome complex (no CHIP-driver sequence variations, 0.4797 nUMI; DNMT3A, 0.9961 nUMI; P < .001; TET2, 1.2189 nUMI; P < .001), plus upregulation of CD163 (no CHIP-driver sequence variations, 0.5239 nUMI; DNMT3A, 1.4722 nUMI; P < .001; TET2, 1.0684 nUMI; P < .001), a cellular receptor capable of mediating infection, macrophage activation syndrome, and other genes involved in cytokine response syndrome. "

Therapies and Procedures

1. Therapeutics
2. Plasma Exchange
3. Critical Care (Surgical Intensive Care)
4. Hematopoietic Stem Cell Transplantation
5. Salvage Therapy