Macrophage activation syndrome in juvenile systemic lupus erythematosus: A multinational multicenter study of thirty‐eight patients
Abstract
Objective: To describe the clinical and laboratory features of macrophage activation syndrome as a complication of juvenile systemic lupus erythematosus (SLE). Methods: Cases of juvenile SLE–associated macrophage activation syndrome were provided by investigators belonging to 3 pediatric rheumatology networks or were found in the literature. Patients who had evidence of macrophage hemophagocytosis on bone marrow aspiration were considered to have definite macrophage activation syndrome, and those who did not have such evidence were considered to have probable macrophage activation syndrome. Clinical and laboratory findings in patients with macrophage activation syndrome were contrasted with those of 2 control groups composed of patients with active juvenile SLE without macrophage activation syndrome. The ability of each feature to discriminate macrophage activation syndrome from active disease was evaluated by calculating sensitivity, specificity, and area under the receiver operating characteristic curve. Results: The study included 38 patients (20 with definite macrophage activation syndrome and 18 with probable macrophage activation syndrome). Patients with definite and probable macrophage activation syndrome were comparable with regard to all clinical and laboratory features of the syndrome, except for a greater frequency of lymphadenopathy, leukopenia, and thrombocytopenia in patients with definite macrophage activation syndrome. Overall, clinical features had better specificity than sensitivity, except for fever, which was highly sensitive but had low specificity. Among laboratory features, the best sensitivity and specificity was achieved using hyperferritinemia, followed by increased levels of lactate dehydrogenase, hypertriglyceridemia, and hypofibrinogenemia. Based on the results of statistical analysis, preliminary diagnostic guidelines for macrophage activation syndrome in juvenile SLE were developed. Conclusion: Our findings indicate that the occurrence of unexplained fever and cytopenia, when associated with hyperferritinemia, in a patient with juvenile SLE should raise the suspicion of macrophage activation syndrome. We propose preliminary guidelines for this syndrome in juvenile SLE to facilitate timely diagnosis and correct classification of patients.
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MAS is recognized as a complication of systemic lupus erythematosus.
“MAS is recognized also as a complication of systemic lupus erythematosus (34), a disease that has been linked to TLR9 overstimulation by self DNA (35)(36)(37)(38).”
From Repeated TLR9 stimulation results in macrophage activation syndrome–like disease in mice (2011) · cites this paper as [34]
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- Authors
- Parodi, A., Davì, S., Pringe, A. B., Pistorio, A., Ruperto, N., Magni‐Manzoni, S., Miettunen, P., Bader‐Meunier, B., Espada, G., Sterba, G., Ozen, S., Wright, D., Magalhães, C. S., Khubchandani, R., Michels, H., Woo, P., Iglesias, A., Guseinova, D., Bracaglia, C., Hayward, K., Wouters, C., Grom, A., Vivarelli, M., Fischer, A., Breda, L., Martini, A., & Ravelli, A.
- Affiliations
- Istituto Giannina Gaslini (IT)
- Hospital General de Niños Ricardo Gutierrez (AR)
- Policlinico San Matteo Fondazione (IT)
- Université Paris Descartes (FR)
- Hospital Clínico Universitario de Caracas (VE)
- Children's Hospital Central California (US)
- Jaslok Hospital (IN)
- German Center for Pediatric and Adolescent Rheumatology (DE)
- University College London (GB)
- Universidad Nacional de Colombia (CO)
- Children's Clinical University Hospital (LV)
- Bambino Gesù Children's Hospital (IT)
- Seattle Children's Hospital (US)
- Universitair Ziekenhuis Leuven (BE)
- Cincinnati Children's Hospital Medical Center (US)
- University of Chieti-Pescara (IT)
- University of Genoa (IT)
- ORCID
- Davì, Sergio
- Pringe, Alejandra Beatriz
- Pistorio, Angela
- Ruperto, Nicolino
- Magni‐Manzoni, Silvia
- Bader‐Meunier, Brigitte
- Espada, Graciela
- Khubchandani, Raju
- Woo, Patricia
- Iglesias, Antonio
- Bracaglia, Claudia
- Hayward, Kristen
- Wouters, Carine
- Grom, Alexei
- Vivarelli, Marina
- Breda, Luciana
- Martini, Alberto
- Ravelli, Angelo
- Venue
- Arthritis & Rheumatism
- Publisher
- Wiley
- Published
- 2009-10-29
- Volume
- 60
- Issue
- 11
- Pages
- 3388-3399
- ISSN
- 0004-3591 (ISSN-L)
- 1529-0131
- OpenAlex
- W2005218341
- Cited by
- 291
- References
- 33
- Citations by year
- 2026: 13 · 2025: 8 · 2024: 25 · 2023: 13 · 2022: 16 · 2021: 21 · 2020: 14 · 2019: 26 · 2018: 24 · 2017: 11 · 2016: 20 · 2015: 21 · 2014: 26 · 2013: 16 · 2012: 17
- FWCI
- 8.19
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Arthritis & Rheumatism (publishedVersion)
- PubMed (publishedVersion)
- Acervo Digital da Universidade Estadual Paulista (Universidade Estadual Paulista) (submittedVersion)
- BOA (University of Milano-Bicocca) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- UNESP Institutional Repository (São Paulo State University) (submittedVersion)
- Funders
- Alpha Scholarship from the European Union (AML/B7-311/970666/II-0246-FI)
- Topics
- Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
- Health Sciences › Medicine › Surgery › Kawasaki Disease and Coronary Complications
- Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
- Keywords
- macrophage activation syndrome, pediatric lupus, hemophagocytosis, hyperferritinemia, fever, cytopenia, thrombocytopenia, leukopenia, hypofibrinogenemia, hypertriglyceridemia, lactate dehydrogenase, diagnostic criteria
- MeSH terms
- Adolescent, Child, Female, Ferritins, Fever, Humans, International Cooperation, Leukopenia, Lupus Erythematosus, Systemic, Male, Retrospective Studies, Thrombocytopenia, Macrophage Activation Syndrome
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