Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

Minoia, Francesca; Davì, Sergio; Horne, AnnaCarin; Bovis, Francesca; Demirkaya, Erkan; Akikusa, Jonathan; Ayaz, Nuray Aktay; Al‐Mayouf, Sulaiman M.; Barone, Patrizia; Bica, Bianca; Bolt, Isabel; Breda, Luciana; Cunto, Carmen De; Enciso, Sandra; Gallizzi, Romina; Griffin, Thomas A.; Hennon, Teresa; Horneff, Gerd; Jeng, Michael; Kapovic, Ageza M; Lipton, Jeffrey M.; Magni‐Manzoni, Silvia; Rumba-Rozenfelde, Ingrīda; Magalhães, Cláudia Saad; Sewairi, Wafaa Mohamed Saad; Stine, Kimo C.; Vougiouka, Olga; Weaver, Lehn K.; Dāvidsone, Zane; Arocena, Jaime de Inocencio; Ioseliani, Maka; Lattanzi, Bianca; Tezer, Hasan; Buoncompagni, Antonella; Picco, Paolo; Ruperto, Nicolino; Martini, Alberto; Cron, Randy Q.; Ravelli, Angelo

doi:10.3899/jrheum.141261

Cited by 66 publications

(45 citation statements)

References 22 publications

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“…The design, inclusion criteria and data collection procedures of this portion of the project have been described in detail previously 16 17 26. Briefly, international paediatric rheumatologists and paediatric haematologists were invited to participate in a retrospective cohort study of patients with systemic JIA-associated MAS or with one of two conditions that could potentially be confused with MAS, that is, active systemic JIA not complicated by MAS and systemic infection.…”

Section: Methodsmentioning

confidence: 99%

“…Paediatric subspecialists who provided these data are listed in Appendix A. The features of the patients with MAS and the comparison patients have been described elsewhere 16 17 26…”

Section: Methodsmentioning

confidence: 99%

“…Recent studies indicate a mortality rate of 8%,16 17 making timely diagnosis and prompt initiation of appropriate treatment imperative. However, early recognition of MAS is often challenging, given the lack of a single pathognomonic clinical or laboratory feature.…”

Section: Introductionmentioning

confidence: 99%

“…In addition, features of MAS may be difficult to distinguish from other conditions that may present with overlapping manifestations, such as flares of systemic JIA or systemic infections. Recently, a wide disparity in the frequency and severity of the classic clinical and laboratory features across patients has been described 16 17…”

Section: Introductionmentioning

confidence: 99%

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2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

et al. 2016

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To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ=0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.

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Section: Methodsmentioning

confidence: 99%

“…Paediatric subspecialists who provided these data are listed in Appendix A. The features of the patients with MAS and the comparison patients have been described elsewhere 16 17 26…”

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

et al. 2016

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“…MAS can result in progressive multi-organ failure and eventually a fatal outcome if unrecognized. Recent studies indicate a mortality rate of 8% (Minoia et al, 2014, 2015). In 2016, classification criteria for MAS complicating sJIA have been published (Table 2) (Ravelli et al, 2015, 2016).…”

Section: Clinical Characteristics Of Sjiamentioning

confidence: 99%

IL-1 Inhibition in Systemic Juvenile Idiopathic Arthritis

et al. 2016

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Systemic juvenile idiopathic arthritis (sJIA) is the form of childhood arthritis whose treatment is most challenging. The demonstration of the prominent involvement of interleukin (IL)-1 in disease pathogenesis has provided the rationale for the treatment with biologic medications that antagonize this cytokine. The three IL-1 blockers that have been tested so far (anakinra, canakinumab, and rilonacept) have all been proven effective and safe, although only canakinumab is currently approved for use in sJIA. The studies on IL-1 inhibition in sJIA published in the past few years suggest that children with fewer affected joints, higher neutrophil count, younger age at disease onset, shorter disease duration, or, possibly, higher ferritin level may respond better to anti-IL-1 treatment. In addition, it has been postulated that use of IL-1 blockade as first-line therapy may take advantage of a “window of opportunity,” in which disease pathophysiology can be altered to prevent the occurrence of chronic arthritis. In this review, we analyze the published literature on IL-1 inhibitors in sJIA and discuss the rationale underlying the use of these medications, the results of therapeutic studies, and the controversial issues.

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

Canna

Nigrović

2016

Arthritis & Rheumatology

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Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

Abstract: 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.

Cited by 66 publications

References 22 publications

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

IL-1 Inhibition in Systemic Juvenile Idiopathic Arthritis

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

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