Editorial: 21st Century Storm Chasers: Defining Macrophage Activation Syndrome

Canna, Scott; Nigrović, Peter A.

doi:10.1002/art.39329

Cited by 7 publications

(1 citation statement)

References 15 publications

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“…The inflammatory responses induced by PAMPs or DAMPs can be either acute when the perturbation is rapidly resolved, and the homeostasis is restored, or chronic and pathologic when rapid clearance mechanisms fail. Finally, activating mutations in NLRP1, NLRP3, NLRC4, or MEFV cause inflammasome assembly independently of PAMPs or DAMPs [9][10][11][12][13][14][15].…”

Section: Introductionmentioning

confidence: 99%

Omnipresence of inflammasome activities in inflammatory bone diseases

Alippe

Mbalaviele

2019

Semin Immunopathol

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The inflammasomes are intracellular protein complexes that are assembled in response to a variety of perturbations including infections and injuries. Failure of the inflammasomes to rapidly clear the insults or restore tissue homeostasis can result in chronic inflammation. Recurring inflammation is also provoked by mutations that cause the constitutive assembly of the components of these protein platforms. Evidence suggests that chronic inflammation is a shared mechanism in bone loss associated with aging, dysregulated metabolism, autoinflammatory, and autoimmune diseases. Mechanistically, inflammatory mediators promote bone resorption while suppressing bone formation, an imbalance which over time leads to bone loss and increased fracture risk. Thus, while acute inflammation is important for the maintenance of bone integrity, its chronic state damages this tissue. In this review, we discuss the role of the inflammasomes in inflammation-induced osteolysis.

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Section: Introductionmentioning

confidence: 99%

Omnipresence of inflammasome activities in inflammatory bone diseases

Alippe

Mbalaviele

2019

Semin Immunopathol

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Inflammasomes in Bone Diseases

Mbalaviele

Veis

2018

Experientia Supplementum

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Clinical utility of soluble interleukin-2 receptor in hemophagocytic syndromes: a systematic scoping review

et al. 2017

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The serum-soluble interleukin-2 receptor (sIL-2r) level is considered an important diagnostic test and disease marker in hemophagocytic syndromes/hemophagocytic lymphohistiocytosis (HPS/HLH). However, this cytokine receptor is rarely measured in clinical practice and has been excluded from recent diagnostic/classification criteria such as the HScore and macrophage activation syndrome (MAS) 16. We performed a systematic scoping review of 64 articles (1975-2016) examining the clinical utility of sIL-2r in HPS/HLH. Twenty-two articles describe sIL-2r as a sensitive diagnostic marker for HLH, but only three distinct datasets actually address sensitivity. The original HLH-2004 Guidelines reported sensitivity of 93% and specificity of 100% for sIL-2r ≥ 2400, based on a pediatric dataset (n = 152) which is published for the first time in this review. Two pediatric studies reported sensitivity of 89% for sIL-2r ≥ 2400 in diagnosis of MAS complicating juvenile idiopathic arthritis (JIA) (n = 27) and 88% for secondary HLH in acute liver failure (n = 9). Twenty articles described sIL-2r as a dynamic marker of disease activity that falls with response to treatment, and 15 described high initial sIL-2r levels >10,000 U/mL as a poor prognostic marker. The ability of sIL-2r to distinguish between subtypes of HPS/HLH was inconsistent. This review confirms the importance of soluble IL-2r as a diagnostic and disease marker in HPS/HLH, but also reveals the need for more primary data about its performance characteristics, particularly in adults. More emphasis should be made in including this simple, inexpensive test in clinical practice and studies of HPS/HLH.

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Editorial: 21st Century Storm Chasers: Defining Macrophage Activation Syndrome

Cited by 7 publications

References 15 publications

Omnipresence of inflammasome activities in inflammatory bone diseases

Omnipresence of inflammasome activities in inflammatory bone diseases

Inflammasomes in Bone Diseases

Clinical utility of soluble interleukin-2 receptor in hemophagocytic syndromes: a systematic scoping review

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