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Macrophage activation syndrome complicating rheumatic diseases in adults: case-based review

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Abstract

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatologic diseases. Data regarding the clinical course, management and outcome of adults with MAS is limited. Therefore, we aimed to describe the clinical features, treatment and outcome of adult patients with MAS, and review the literature for previous cohorts. We retrospectively reviewed patients with MAS complicating rheumatologic diseases between the years 2007 and 2017. Through Pubmed, Medline and Scopus literature search we identified previous cases of adult patients with MAS. We identified 7 patients with MAS complicating rheumatologic diseases (5 females and 2 males). The median age of diagnosis was 32 (range 26–57) years. The median follow-up was 30 months (range 6.95–36.5) months. The underlying rheumatologic disease was adult onset Still’s disease (AOSD) in 3 patients, systemic juvenile idiopathic arthritis (sJIA) in 2 patients, systemic lupus erythematosus (SLE) in 1 patient, and systemic vasculitis in 1 patient. Four patients developed MAS concurrently with the clinical development of the rheumatologic disease. All the patients were treated with systemic corticosteroids. Five patients were treated with cyclosporine A, one of which received combination therapy with anakinra, and one received tocilizumab. Two patients deceased during the hospitalization. We identified 92 patients from literature cohorts, 73 (79%) of them with AOSD. MAS developed concurrently with the underlying rheumatologic disease in 25 (27%) patients, and 30 (33%) patients deceased. Our cohort and previous cohorts demostrate that MAS often presents concurrently with the underlying rheumatologic disease and is associated with a high mortality rate. Further larger prospective studies are needed to determine the optimal management of MAS.

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Author notes

  1. Mayan Gilboa and Gil Bornstein equally contributed to this study and are co-first.

Authors and Affiliations

  1. Department of Internal Medicine D, The Chaim Sheba Medical Center, Affiliated to Sackler Faculty of Medicine, Tel Aviv University, Tel-Hashomer, Ramat Gan, Israel

    Mayan Gilboa

  2. Department of Internal Medicine B, Tel Aviv Sourasky Medical Center, Affiliated to Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

    Gil Bornstein

  3. Department of Internal Medicine F and the Rheumatology Unit, The Chaim Sheba Medical Center, Affiliated to Sackler Faculty of Medicine, Tel-Aviv University, Tel Hahsomer, 52621, Ramat Gan, Israel

    Ilan Ben-Zvi & Chagai Grossman

Authors
  1. Mayan Gilboa
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  2. Gil Bornstein
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  3. Ilan Ben-Zvi
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  4. Chagai Grossman
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Contributions

MG: data curation, validation and formal analysis. GB: methodology, investigation. IB-Z: supervision, writing—review and editing. CG: writing—original draft, investigation. All co-authors take full responsibility for the integrity of the study and all parts of the final manuscript.

Corresponding author

Correspondence to Chagai Grossman.

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Conflict of interest

Mayan Gilboa declares she has no conflict of interest. Gil Bornstein declares he has no conflict of interest. Ilan Ben-Zvi declares he has no conflict of interest. Chagai Grossman declares he has no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Gilboa, M., Bornstein, G., Ben-Zvi, I. et al. Macrophage activation syndrome complicating rheumatic diseases in adults: case-based review. Rheumatol Int 40, 663–669 (2020). https://doi.org/10.1007/s00296-019-04393-7

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  • DOI: https://doi.org/10.1007/s00296-019-04393-7

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