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Pediatric Education and Research 13(2):p 51-52, July -Dec 2025. | DOI: 10.21088/per.2321.1644.13325.2
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Macrophage Activation Syndrome Presenting Unusually with Facial Edema in a Known Case of Mixed Connective Tissue Disease
Rupali Rokade, Supriya Kushwah, Somashekhar B
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Attribution-Non-commercial 4.0 International (CC BY-NC 4.0)This license enables reusers to distribute, remix, adapt, and build upon the material in any medium or format for noncommercial purposes only, and only so long as attribution is given to the creator.
Pediatric Education and Research 13(2):p 51-52, July -Dec 2025. | DOI: 10.21088/per.2321.1644.13325.2
How Cite This Article:
Supriya Kushwah, Somashekhar B., Rupali Rokade. Macrophage Activation Syndrome Presenting Unusually with Facial Edema in a Known Case of Mixed Connective Tissue Disease. Pediatr. Edu. Res. 2025; 13(2): 51-52.Timeline
Received : November 20, 2025
Accepted : December 19, 2025
Published : December 30, 2025
Abstract
Macrophage Activation Syndrome (MAS) is an unusual and severe complication in Mixed Connective Tissue Disease (MCTD). It involves multi-organ system and can be fatal if identified late.1,2 A 17-year-old female, diagnosed with MCTD one year prior and maintained on hydroxychloroquine, mycophenolate mofetil, and low-dose steroids, presented with 7 days of fever and facial puffiness with vitals stable. On admission child had pallor, skin thickening, digital pitting edema. On admission MAS workup was negative. After 3 days of admission, the patient developed neuropsychiatric symptoms and seizures suggestive of neurolupus and MAS that was confirmed on repeat workup. She was managed with intravenous immunoglobulin (2 g/kg over 5 days), broad-spectrum antibiotics, and pulse methylprednisolone therapy followed by high-dose oral prednisolone. Cyclosporine was added for disease activity control. Child developed concurrent renal involvement in the form of proteinuria and positive dsDNA antibodies, started on intravenous cyclophosphamide. This case is notable for MAS at any phase of disease along with disease flare in MCTD, complicated by Central nervous system and renal lupus activity. MAS is a rare but life-threatening hyperinflammatory syndrome, more frequently reported in systemic juvenile idiopathic arthritis than in MCTD. Prompt recognition and aggressive immunosuppression are essential to prevent irreversible organ damage. Our case underscores the importance of considering MAS in any patient with connective tissue disease presenting with unexplained fever, cytopenias, hyperferritinemia, and organ dysfunction. Early diagnosis and initiation of targeted immunosuppressive therapy can be lifesaving.3,4 Child is on monthly cyclophophamide and asymptomatic.
References
- 1. Yamaoka H., Hada I., Watanabe M, et al. Macrophage Activation Syndrome as an Atypical Manifestation of Mixed Connective Tissue Disease in a 14-Year-Old Girl: A Case Report. Cureus. 2025; 17(3): e80206. doi:10.7759/cureus.80206.
- 2. Prakash J., Porel R., Kumar N., Biswas R., Ojha V.S. Macrophage Activation Syndrome Secondary to Mixed Connective Tissue Disease: A Rare Case in a Tertiary Care Hospital of Eastern India. International Journal of Innovative Science and and Research Technology. 2023; 8(8).
- 3. Gouda W., Alsaqabi F., Moshrif A., et al. Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with dexamethasone sodium phosphate, IVIG, and cyclosporine. Journal of Medical Case Reports. 2021; 15: 497.
- 4. Dong Y., Wang T., Wu H. Heterogeneity of macrophage activation syndrome and treatment progression. Front Immunol. 2024; 15: 1389710
Data Sharing Statement
There are no additional data available. All raw data and code are available upon request.
Funding
This research received no funding.
Author Contributions
All authors contributed significantly to the work and approve its publication.
Ethics Declaration
This article does not involve any human or animal subjects, and therefore does not require ethics approval.
Acknowledgements
We would like to express our gratitude to the patients, their families, and all those who have contributed to this study.
Conflicts of Interest
No conflicts of interest in this work.
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Cite this article
Supriya Kushwah, Somashekhar B., Rupali Rokade. Macrophage Activation Syndrome Presenting Unusually with Facial Edema in a Known Case of Mixed Connective Tissue Disease. Pediatr. Edu. Res. 2025; 13(2): 51-52.
Licence:
Attribution-Non-commercial 4.0 International (CC BY-NC 4.0)This license enables reusers to distribute, remix, adapt, and build upon the material in any medium or format for noncommercial purposes only, and only so long as attribution is given to the creator.
| Received | Accepted | Published |
|---|---|---|
| November 20, 2025 | December 19, 2025 | December 30, 2025 |
DOI: 10.21088/per.2321.1644.13325.2
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Timeline
| Received | November 20, 2025 |
| Accepted | December 19, 2025 |
| Published | December 30, 2025 |
licence
Attribution-Non-commercial 4.0 International (CC BY-NC 4.0)
This license enables reusers to distribute, remix, adapt, and build upon the material in any medium or format for noncommercial purposes only, and only so long as attribution is given to the creator.
