Clinical characteristics of children with MIS-C fulfilling classification criteria for macrophage activation syndrome

Buda, Piotr; Strauss, Ewa; Januszkiewicz‐Lewandowska, Danuta; Czerwińska, Ewa; Ludwikowska, Kamila; Szenborn, Leszek; Gowin, Ewelina; Okarska‐Napierała, Magdalena; Kuchar, Ernest; Książyk, Janusz

doi:10.3389/fped.2022.981711

Cited by 8 publications

(18 citation statements)

References 55 publications

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“…Studies reported that a higher frequency of gastrointestinal symptoms, shock, and reduced lymphocytes and macrophages is observed in MIS-C than in KD. Moreover, the MIS-C cases were significantly older than the KD patients, with a less rash rate and a higher ICU admission rate ( 11 , 13 , 30 ). The current treatment of MIS-C mimics the treatment of KD, and the use of IVIG and/or high-dose corticosteroids was recommended as first-line therapy in these patients ( 11 , 32 ).…”

Section: Discussionmentioning

confidence: 94%

“…Since the outbreak of COVID-19 infections in December 2019, the SARS-CoV-2-related MIS-C has been reported by several centers ( 11 – 13 , 30 , 31 ). The overall manifestations of MIS-C are similar to those of KD, including fever, conjunctivitis, and elevated inflammatory markers.…”

Section: Discussionmentioning

confidence: 99%

“…Both MIS-C and KD can also develop MAS as the disease progressed. To date, however, it is not clear whether MAS related to MIS-C and MAS related to KD share the same pathogenesis ( 11 , 13 , 33 ). The early differential diagnosis of sJIA, MIS-C, and KD is difficult.…”

Section: Discussionmentioning

confidence: 99%

“…The onset of MIS-C occurs approximately 4 weeks after symptomatic or asymptomatic SARS-CoV-2 infection ( 12 ). If MIS-C is refractory to treatment, the development of MAS should be suspect, which can be fatal if not diagnosed early ( 13 ). The study has reported that higher procalcitonin (PCT), ferritin, and fibrinogen (FIB) levels at admission were the risk factors for MAS in MIS-C. Fortunately, the clinical courses and prognoses of MAS in MIS-C seem better than other rheumatological diseases based on limited data ( 13 ).…”

Section: Introductionmentioning

confidence: 99%

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Platelet and ferritin as early predictive factors for the development of macrophage activation syndrome in children with Kawasaki disease: A retrospective case-control study

Zhang

Xiao²,

Zhou³

et al. 2023

Front. Pediatr.

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ObjectiveTo investigate the early predictive factors for Kawasaki disease complicated with macrophage activation syndrome (KD-MAS)MethodsWe performed a retrospective case-control study in children with KD from August 2017 to August 2022, involving 28 cases with KD-MAS and 112 cases not developing KD-MAS. Based on the univariate analysis, binary logistic regression was used to identify the early predictive factors for KD-MAS development, and the receiver operating characteristic curve (ROC) analysis was carried out to obtain the optimal cut-off value.ResultsTwo predictive factors were associated with the development of KD-MAS, which were PLT (OR = 1.013, 95%CI, 1.001–1.026), and serum ferritin (OR = 0.991, 95%CI, 0.982–0.999). The cut-off value of PLT was 110 × 109/L, and the cut-off value of serum ferritin was 548.4 ng/ml.ConclusionChildren with KD who had a PLT count under 110 × 109/L, and a serum ferritin level over 548.4 ng/ml are more likely to develop KD-MAS.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Platelet and ferritin as early predictive factors for the development of macrophage activation syndrome in children with Kawasaki disease: A retrospective case-control study

Zhang

Xiao²,

Zhou³

et al. 2023

Front. Pediatr.

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“…Another study [ 25 ] showed that 21% of MIS-C patients fulfilled the 2016 classification criteria for MAS complicating sJIA [ 26 ]. Furthermore, in the patients with MIS-C, older age, atypical KD phenotype, and skin erosions were significant factors indicating the risk for MAS besides the well-known laboratory signs of MAS.…”

Section: Discussionmentioning

confidence: 99%

Overlapping clinical features of systemic juvenile idiopathic arthritis and SARS-CoV-2-related multisystem inflammatory syndrome in children

Boyarchuk¹,

Kovalchuk²

2023

Reumatologia

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IntroductionDifferential diagnosis of the systemic juvenile idiopathic arthritis (sJIA) is often complicated, because of the variability in clinical presentation and the absence of specific signs.Material and MethodsThe PubMed/Medline and Scopus databases from the years 2013–2022 were analysed for full articles in English and the following key words were used: “juvenile idiopathic arthritis” and “MIS-C”; “juve-nile idiopathic arthritis” and “Kawasaki disease”. As an example of the problem the case description of a 3-year-old patient is presented.ResultsIn the first step 167 publications were identified; however, after exclusion of duplicated articles and those not relevant to the topic, only 13 were included in the analysis. We analysed studies that de-scribe overlapping clinical features of sJIA and Kawasaki disease (KD) or multisystem inflammatory syndrome in children (MIS-C). The main issues we discussed were the search for the specific fea-tures that would distinguish one disease from another. Fever refractory to intravenous immuno-globulin treatment was the most frequent indicator among the features of clinical courses. Among other clinical signs prolonged, recurrent fever, rash, an incomplete KD phenotype, Caucasian race, splenomegaly, and complicated macrophage activation syndrome also supported sJIA diagnosis. Among laboratory tests, high ferritin and serum interleukin-18 levels were found to be the most useful in differentiation. The present case demonstrates that prolonged, unexplained, recurrent fe-ver with a specific pattern should be the reason to suspect sJIA.ConclusionsOverlapping features of sJIA and SARS-CoV-2-related MIS-C complicates diagnosis in the era of the COVID-19 pandemic. Our case presentation adds symptoms of prolonged, spiking, unexplained, re-current fever with a specific pattern for supporting systemic juvenile idiopathic arthritis diagnosis.

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Clinical and laboratory features associated with macrophage activation syndrome in Still’s disease: data from the international AIDA Network Still’s Disease Registry

Triggianese,

Vitale,

Lopalco

et al. 2023

Intern Emerg Med

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To characterize clinical and laboratory signs of patients with Still’s disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still’s disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still’s Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still’s disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9–52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9–97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still’s disease onset (OR 0.6, 95% CI 0.4–0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01–0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0–0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data.

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Clinical characteristics of children with MIS-C fulfilling classification criteria for macrophage activation syndrome

Abstract: Ksia żyk J ( ) Clinical characteristics of children with MIS-C fulfilling classification criteria for macrophage activation syndrome.

Cited by 8 publications

References 55 publications

Platelet and ferritin as early predictive factors for the development of macrophage activation syndrome in children with Kawasaki disease: A retrospective case-control study

Platelet and ferritin as early predictive factors for the development of macrophage activation syndrome in children with Kawasaki disease: A retrospective case-control study

Overlapping clinical features of systemic juvenile idiopathic arthritis and SARS-CoV-2-related multisystem inflammatory syndrome in children

Clinical and laboratory features associated with macrophage activation syndrome in Still’s disease: data from the international AIDA Network Still’s Disease Registry

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