Macrophage Activation Syndrome in Children: Diagnosis and Management

Bagri, Narendra Kumar; Gupta, Latika; Sen, Ethan S; Ramanan, Athimalaipet V

doi:10.1007/s13312-021-2399-8

Cited by 9 publications

(11 citation statements)

References 44 publications

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“…Macrophage activation a variant HLH, most often accompanies systemic juvenile idiopathic arthritis (sJIA) [8] and systemic lupus erythematosus (SLE) [9], but can be encountered in a wide range of autoimmune disorders [10]. The only child in our series with autoimmune disease who was diagnosed with HLH suffered from ANCA-positive systemic vasculitis.…”

Section: Discussionmentioning

confidence: 95%

Soluble interleukin-2 receptor in pediatric patients investigated for hemophagocytic lymphohistiocytosis: A single-center, 10-year-long experience

et al. 2023

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Introduction/Objective. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, splenomegaly and cytopenias. Diagnosis of HLH requires at least five of the eight criteria set by the Histiocyte Society and poses a significant challenge to physicians. HLH-2004 criteria include measurement of plasma levels of soluble receptor for interleukin-2 (sIL-2R), an invaluable tool in the diagnosis of HLH, particularly because it can be measured swiftly and inexpensively. Methods. We retrospectively analyzed medical records of 45 pediatric patients (28 boys and 17 girls, median age 8.1 years) who were investigated for suspected HLH in University Children?s Hospital, Belgrade, in the period 2012-2022. Results. Ten children were diagnosed with HLH, while 35 did not have HLH. All ten HLH patients had secondary HLH: eight suffered from infection or inflammatory condition, one from an autoimmune disease, and one from malignancy. Level of sIL-2R was above the HLH-2004 cutoff value of 2400 IU/ml in 9/10 patients with HLH (sensitivity 90%) and 9/35 of patients who did not have HLH (specificity 74.2%). Conclusion. Soluble IL-2 receptor measurement is valuable in children suspected to have HLH. Sensitivity and specificity of this analysis can be further improved by strict patient selection and a comprehensive diagnostic approach.

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Section: Discussionmentioning

confidence: 95%

Soluble interleukin-2 receptor in pediatric patients investigated for hemophagocytic lymphohistiocytosis: A single-center, 10-year-long experience

et al. 2023

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“…In children with sJIA, occult MAS is reported to be at least three times more common than overt MAS [ 9 , 10 ]. sJIA patients with occult MAS show clinical manifestations that do not currently meet the diagnostic criteria for MAS [ 13 , 26 ].…”

Section: Discussionmentioning

confidence: 99%

“…Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis (HLH), is an inflammatory phenomenon caused by excessive activation of T cells and macrophages secondary to infections, malignancies, or rheumatic diseases [ 8 , 9 , 10 ]. It is characterized by unremitting fever, hepatosplenomegaly, cytopenia, and organ dysfunction [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

“…It is characterized by unremitting fever, hepatosplenomegaly, cytopenia, and organ dysfunction [ 9 ]. The leading causes of MAS in children are systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE) [ 10 ]. With increasing reports of KD complicated by MAS, KD is considered the third most common cause of MAS in children [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

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Under-Recognized Macrophage Activation Syndrome in Refractory Kawasaki Disease: A Wolf in Sheep’s Clothing

et al. 2022

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Recognition of macrophage activation syndrome (MAS) in patients with refractory Kawasaki disease (KD) can be challenging. This study aimed to investigate the incidence of MAS in patients with refractory KD and to compare the characteristics of refractory KD and MAS. Medical records of 468 patients diagnosed with KD from January 2010 to December 2019 were retrospectively reviewed. Of the 468 KD patients, 63 were enrolled in the study as a refractory KD group (n = 59) and an MAS group (n = 4). The incidence of MAS was 0.8% (4/468) in patients with KD and 6.3% (4/63) in patients with refractory KD. Compared to the refractory KD group, the MAS group had higher frequencies of incomplete KD, hepatosplenomegaly, third-line treatment, and MAS screening, and showed lower levels of albumin. No significant differences were found in other clinical and laboratory findings. In addition to four patients with MAS, five patients with refractory KD who received third-line treatment showed severe systemic inflammation and organ dysfunction, but only one in five patients underwent MAS screening, including ferritin levels. In conclusion, given the relatively high incidence of MAS in children with refractory KD and the similar phenotype between refractory KD and MAS, we propose that MAS screening should be included in routine laboratory tests for refractory KD.

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“…In a study of sJIA [19], the median interval between sJIA diagnosis and MAS onset was 4 months, and the male to female ratio of patients was 4:6. In summarizing the epidemiologic studies on pediatric MAS, the approximate incidence would be listed in the following order: sJIA (~10%) > SLE (~5%) > KD (~2%) > JDM (18 cases reported worldwide) [20][21][22][23][24]. A similar incidence of MAS has been reported in an adult study [25]: adult-onset Still's disease (AOSD, 11.5%), SLE (5.1%), systemic sclerosis (2.3%), dermatomyositis/polymyositis (DM/PM, 2.0%), and rheumatoid arthritis (RA, 1.5%).…”

Section: Epidemiologymentioning

confidence: 99%

Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment

Lee,

Bae,

Rhim

et al. 2024

Children

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Macrophage activation syndrome (MAS) is potentially fatal; so, early diagnosis and timely treatment are essential. However, detecting MAS is sometimes challenging because its principal features can be observed in other pediatric diseases that cause severe inflammation. Cytokine storm due to immune dysregulation represents the clinical and laboratory features of MAS that are included in the diagnostic criteria. Most cases of MAS occur as an underlying condition worsens and progresses. Therefore, a patient with autoimmune or autoinflammatory disease who shows unexplained clinical deterioration despite appropriate management should be considered at high risk for MAS (i.e., occult MAS). The basic principles of treatment are control of triggering factors, supportive care, and relief of hyperinflammation. Systemic steroids and cyclosporine A are frequently used as a first-line treatment. For the treatment of refractory MAS, cytokine-specific biologic agents such as anakinra have recently become preferred over traditional immunosuppressive agents such as etoposide. MAS might be underrecognized in pediatric patients with infectious and inflammatory diseases due to its diverse clinical presentations. Clinical suspicion of MAS is of the utmost importance for early recognition of the disease.

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Macrophage Activation Syndrome in Children: Diagnosis and Management

Cited by 9 publications

References 44 publications

Soluble interleukin-2 receptor in pediatric patients investigated for hemophagocytic lymphohistiocytosis: A single-center, 10-year-long experience

Soluble interleukin-2 receptor in pediatric patients investigated for hemophagocytic lymphohistiocytosis: A single-center, 10-year-long experience

Under-Recognized Macrophage Activation Syndrome in Refractory Kawasaki Disease: A Wolf in Sheep’s Clothing

Macrophage Activation Syndrome in Children: Update on Diagnosis and Treatment

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