Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: Report of five cases
Abstract
Objectives To evaluate the efficacy of cyclosporine A in the treatment of macrophage activation syndrome (MAS) occurring in children with juvenile arthritis.Study design MAS developed in two boys and three girls with systemic juvenile arthritis (four) and polyarticular juvenile arthritis (one). In three children whose condition was life-threatening, increased parenteral administration of corticosteroids failed to improve their condition; therefore cyclosporine A (2 to 5 mg/kg per day) was added. In two other patients with less severe clinical manifestations, cyclosporine A alone (2 to 8 mg/kg per day) was given.Results After the introduction of cyclosporine A, rapid improvement was obtained in all patients and apyrexia occurred within 24 to 48 hours. The biologic abnormalities disappeared more slowly (up to 5 weeks for liver enzymes).Conclusions These observations underline the usefulness of cyclosporine A in this complication. The use of this drug may circumvent the need for increased doses of corticosteroids in some patients. The mechanism of action of cyclosporine A remains speculative, but these results indicate indirectly that T-helper lymphocytes may play a role in the pathogenesis of MAS.
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Nonablative inhibition of T cells with cyclosporine can also be helpful.
“Nonablative inhibition of T cells with cyclosporine can also be helpful.77”
From Cytokine storm (2020) · cites this paper as [77]
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- Authors
- Mouy, R., Stephan, J.-L., Pillet, P., Haddad, E., Hubert, P., & Prieur, A.-M.
- Affiliations
- Hôpital Necker-Enfants Malades (FR)
- ORCID
- Hubert, Philippe
- Venue
- The Journal of Pediatrics
- Publisher
- Elsevier BV
- Published
- 1996-11
- Volume
- 129
- Issue
- 5
- Pages
- 750-754
- ISSN
- 0022-3476 (ISSN-L)
- OpenAlex
- W2062603648
- Cited by
- 244
- References
- 25
- Citations by year
- 2026: 4 · 2025: 3 · 2024: 8 · 2023: 2 · 2022: 6 · 2021: 6 · 2020: 13 · 2019: 7 · 2018: 7 · 2017: 9 · 2016: 13 · 2015: 15 · 2014: 11 · 2013: 9 · 2012: 9
- FWCI
- 2.60
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Pediatrics (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
- Health Sciences › Medicine › Surgery › Kawasaki Disease and Coronary Complications
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Keywords
- cyclosporine, macrophage activation syndrome, juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, corticosteroid-refractory disease, T helper cells, liver enzymes
- MeSH terms
- Arthritis, Juvenile, Child, Child, Preschool, Female, Humans, Immunosuppressive Agents, Macrophage Activation, Male, Syndrome, Cyclosporine, Treatment Outcome, Antirheumatic Agents
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