Features, Treatment, and Outcomes of Macrophage Activation Syndrome in Childhood‐Onset Systemic Lupus Erythematosus

Borgia, R. Ezequiel; Gerstein, Maya; Levy, Deborah M.; Silverman, Earl D.; Hiraki, Linda T.

doi:10.1002/art.40417

Cited by 79 publications

(65 citation statements)

References 27 publications

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“…While MAS is most often associated with sJIA and AOSD, it is increasingly recognized in other rheumatologic conditions. In a recent retrospective series, close to 10% of childhoodonset SLE patients had MAS, a proportion similar to sJIA [41]. MAS has been reported in KD (1-2% of patients) but less frequently than in sJIA and SLE [42,44].…”

Section: Rheumatologicmentioning

confidence: 86%

“…Preliminary SLE-specific MAS criteria have also been developed by Parodi and collaborators based on the study of 38 patients with SLE and MAS; however, this study did not have a replication cohort [105]. A recent retrospective review of a different cohort of 403 pediatric SLE patients, including 38 patients with MAS, showed that all SLE patients diagnosed with MAS by the treating physician also met the Parodi MAS criteria [41].…”

Section: Classification Criteria For Macrophage Activation Syndrome (mentioning

confidence: 99%

“…Traditionally, it was thought that about 10% of children with sJIA developed MAS; however, it is now understood that many more (30-40%) sJIA patients have subclinical features of the disease [37][38][39]. Further, MAS has been described in rheumatologic diseases other than sJIA including systemic lupus erythematosus (SLE) and Kawasaki disease (KD) [40][41][42][43][44]. The prevalence of HLH/MAS in critically ill patients with sepsis physiology is almost certainly underdiagnosed by large margins, particularly in patients with cytopenias, coagulopathy, and/or hepatobiliary dysfunction.…”

Section: Introductionmentioning

confidence: 99%

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Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management

2019

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Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis, is a frequently fatal complication of a variety of pediatric inflammatory disorders. MAS has been most commonly associated with systemic juvenile idiopathic arthritis (sJIA), as approximately 10% of children with sJIA develop fulminant MAS, with another 30-40% exhibiting a more subclinical form of the disease. Children with other rheumatologic conditions such as systemic lupus erythematosus and Kawasaki disease are also at risk for MAS. Moreover, MAS also complicates various genetic autoinflammatory disorders such as gain of function mutations in the cytosolic inflammasome NLRC4, pediatric hematologic malignancies (e.g., T-cell lymphoma), and primary immunodeficiencies characterized by immune dysregulation. Disease-specific and broadly inclusive diagnostic criteria have been developed to facilitate the diagnosis of MAS. Recently, simple screening tools such as the serum ferritin to erythrocyte sedimentation rate ratio have been proposed. Early diagnosis and rapid initiation of immunosuppression are essential for the effective management of MAS. With a better understanding of the pathophysiology of MAS and the advent of novel therapeutics, a broad immunosuppressive approach to treatment is giving way to targeted anti-cytokine therapies. These treatments include agents that block interleukin-1 (IL-1), IL-6, IL-18, interferon-γ, as well as inhibitors of downstream targets of cytokine signaling (e.g., Janus kinases). Increased early recognition of MAS among pediatric inflammatory disorders combined with the use of effective and less toxic cytokine-targeted therapies should lower the mortality of this frequently fatal disorder.

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Section: Rheumatologicmentioning

confidence: 86%

Section: Classification Criteria For Macrophage Activation Syndrome (mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management

2019

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“…Among systemic autoimmune diseases, MAS has been described mainly in association with systemic JIA. However, it has been increasingly recognized as a complication of juvenile SLE (jSLE), with studies showing that the incidence of MAS in jSLE ranges from 5.5 to 9% [3, 4].…”

Section: Introductionmentioning

confidence: 99%

“…Recently, Parodi et al [7] published preliminary diagnostic guidelines for MAS as a complication of jSLE. These guidelines were shown to be clinically relevant by Borgia et al [4], who found that all 38 patients in their study of clinically diagnosed childhood-onset SLE also met the criteria of Parodi et al [7] . Moreover, they reported that in 68% of patients, jSLE and MAS were diagnosed simultaneously.…”

Section: Introductionmentioning

confidence: 99%

Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus

Sato¹,

Uejima²,

Arakawa

et al. 2019

Rheumatology Advances in Practice

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Objectives Macrophage activation syndrome (MAS) is a severe complication of juvenile systemic lupus erythematosus (jSLE). However, little is known about the association between these conditions, especially in terms of MAS as the initial manifestation of jSLE. The aim of this study was to determine the clinical features of MAS as the initial manifestation of jSLE. Methods We carried out a retrospective review of the clinical features of MAS cases diagnosed concomitantly with jSLE from 2004 to 2016. Data from these patients were compared with those from a control group consisting of jSLE patients without MAS. Results Eleven (23.9%) of the 46 patients recruited for this study were diagnosed with MAS during the initial stage of jSLE. The between-group comparisons demonstrated that fever, leucopenia, hyperferritinaemia and increased aspartate aminotransferase were more frequently observed in jSLE patients with MAS than in controls ( P <0.01). Importantly, neurological symptoms were significantly more common in patients with MAS than in controls ( P <0.01), with 6 (54.6%) of the 11 MAS patients affected. For treatment, all 11 patients with both jSLE and MAS were administered CSs upon diagnosis, and 7 received immunosuppressants. No patient involved in this study died. Conclusion MAS can develop as the initial manifestation of jSLE. MAS with jSLE should be suspected in patients with fever, hyperferritinaemia, cytopenia and liver disorder. In addition, we found that jSLE patients with MAS had more neurological symptoms than those without. All patients with MAS were successfully treated with CSs. Early diagnosis and intensive therapy are essential in improving clinical outcomes.

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To “Lump” or to “Split” in Macrophage Activation Syndrome and Hemophagocytic Lymphohistiocytosis

Nikiforow

Berliner

2019

Arthritis & Rheumatology

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Features, Treatment, and Outcomes of Macrophage Activation Syndrome in Childhood‐Onset Systemic Lupus Erythematosus

Cited by 79 publications

References 27 publications

Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management

Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management

Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus

To “Lump” or to “Split” in Macrophage Activation Syndrome and Hemophagocytic Lymphohistiocytosis

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