# Unleashing the Cytokine Storm: A Case of Macrophage Activation Syndrome in Systemic Lupus Erythematosus

**Authors:** Natalie Shaykh, Oshin Rai, Abhinav Karan, Khushman K Bhullar, Vanshika Tripathi, Pramod Reddy

PMC · DOI: 10.7759/cureus.63167 · Cureus · 2024-06-25

## TL;DR

This paper describes a rare case of macrophage activation syndrome in a young woman with systemic lupus erythematosus, highlighting its unique clinical presentation and management.

## Contribution

The novelty lies in presenting a rare case of MAS in an adult with SLE, emphasizing its distinct clinical features and treatment approach.

## Key findings

- MAS was diagnosed in an 18-year-old female with SLE, a rare occurrence.
- The case exhibited a unique clinical presentation compared to typical MAS cases.
- The patient's treatment and outcome are detailed, offering insights into managing MAS in SLE.

## Abstract

Macrophage activation syndrome (MAS), synonymous with secondary hemophagocytic lymphohistiocytosis (HLH), is a rare and critical complication of rheumatologic disease stemming from the unregulated activation and rapid multiplication of macrophages and T lymphocytes. While it primarily manifests in children diagnosed with systemic juvenile idiopathic arthritis (sJIA), it can arise less frequently in other rheumatologic conditions. Here, we outline the clinical course, treatment, and outcome of MAS diagnosed in an 18-year-old female previously diagnosed with SLE who exhibited a unique clinical presentation.

## Linked entities

- **Diseases:** macrophage activation syndrome (MONDO:0015545), secondary hemophagocytic lymphohistiocytosis (MONDO:0015542), systemic juvenile idiopathic arthritis (MONDO:0019434), systemic lupus erythematosus (MONDO:0007915)

## Full-text entities

- **Diseases:** rheumatologic disease (MESH:D012216), juvenile idiopathic arthritis (MESH:D001171), HLH (MESH:D051359), SLE (MESH:D008180), MAS (MESH:D055501)

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11272981/full.md

## References

20 references — full list in the complete paper: https://tomesphere.com/paper/PMC11272981/full.md

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Source: https://tomesphere.com/paper/PMC11272981