Hyperferritinemia Wins Again: Defining Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus

Smitherman, Emily A.; Cron, Randy Q.

doi:10.3899/jrheum.210024

Cited by 3 publications

(3 citation statements)

References 15 publications

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“…In this study also retrospectively sought data on hospitalizations within 12 months, all cases of MAS occurred within 30 days of SLE diagnosis. In this study, there were no data on the strength of the association between ferritin and MAS [14]. Another study by Almutairi et al, found serum ferritin levels to be significantly correlated with SLEDAI (p<0.0001) and MAS markers (LDH, AST, triglycerides and CD25) [4].…”

Section: Resultscontrasting

confidence: 53%

“…In addition to assessing disease activity in the SLE, checking ferritin levels can detect early severe complications in SLE in the form of MAS that SLEDAI scores or others cannot assess. In a pediatric study by Smitherman et al [14], ferritin 627 g/L has been shown to be 89% specific with a sensitivity of 95% for identifying patients with MAS. In this study also retrospectively sought data on hospitalizations within 12 months, all cases of MAS occurred within 30 days of SLE diagnosis.…”

Section: Resultsmentioning

confidence: 96%

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Increase in Serum Ferritin Level as a Marker of Disease Activity in Pediatric Sys-temic Lupus Erythematosus (pSLE) Patients

Wulandari,

Barlianto,

Nugroho

et al. 2023

J.Trop.Life.Science

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Ferritin is an acute-phase reactant that is elevated in autoimmune disorders, including systemic lupus erythematosus (SLE). However, their correlation with disease activity scores has not been confirmed. Pandemic Covid-19 makes children visitation to hospital to get the treatment of SLE were delayed. This study aimed to evaluate correlation between serum ferritin and disease activity and its role in screening for flare in pediatric SLE (pSLE) patients during pandemic Covid-19. This is a cross-sectional study conducted in Saiful Anwar General Hospital Malang. Sampling was carried out sequentially on pediatric patients who met the criteria for Systemic Lupus International Collaborating Clinics (SLICC) and were recorded between July 2021-May 2022. All patients were interviewed and assessed for disease activity using SLE Disease Activity Index 2000 (SLEDAI-2k). A score <4 was categorized as inactive disease. Biochemical, serological tests including markers of disease activity and ferritin level were measured by standard laboratory procedure. Comparison, correlation and ROC curve analyses were performed with SPSS software. There were 38 females pSLE participated in this study. The mean age of the patients were 12.6 ± 3.02 years. Serum ferritin significantly higher in active disease compared to inactive disease 84.50 ng/mL (68.00-151.75 ng/mL) ng/mL and 815.00 ng/mL (451.25-1570.00 ng/mL), a value of p<0.05 was determined to be statistically significant. A significant correlation was found between serum ferritin with SLEDAI 2K (r = 0.890, p = 0.000). Correlation was also found between serum ferritin and IgM anti-double stranded-DNA (r = 0.325, p = 0.046), but not with other laboratory and serological parameters. In ROC curve analysis, we found that Area Under The Curve (AUC) 0.989, 95%CI 0.964-1.014, p value 0,000, with cut off value 297.50 with sensitivity 85% and specificity 94.4%. Ferritin was increased in active disease as compared to inactive disease and correlated with SLEDAI score and IgM-dsDNA. Thus, ferritin may be potential as an affordable and available marker of disease activity in pSLE during pandemic Covid-19.

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Section: Resultscontrasting

confidence: 53%

Section: Resultsmentioning

confidence: 96%

Increase in Serum Ferritin Level as a Marker of Disease Activity in Pediatric Sys-temic Lupus Erythematosus (pSLE) Patients

Wulandari,

Barlianto,

Nugroho

et al. 2023

J.Trop.Life.Science

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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: 96%

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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COVID-19 and the hyperferritinemic syndromes

Zandman-Goddard

Tocut

2023

Autoimmunity, COVID-19, Post-Covid19 Syndrome and COVID-19 Vaccination

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Hyperferritinemia Wins Again: Defining Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus

Cited by 3 publications

References 15 publications

Increase in Serum Ferritin Level as a Marker of Disease Activity in Pediatric Sys-temic Lupus Erythematosus (pSLE) Patients

Increase in Serum Ferritin Level as a Marker of Disease Activity in Pediatric Sys-temic Lupus Erythematosus (pSLE) Patients

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

COVID-19 and the hyperferritinemic syndromes

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