Journal of

Case Reports and Images in Orthopedics and Rheumatology

 
     
Case Report
 
Macrophage activation syndrome: A nearly fatal presentation, and review of diagnosis and treatment guidelines
Luis W. Dominguez1, Valentin D. Marian2
1Department of Internal Medicine, Jersey City Medical Center
2Department of Rheumatology and Assistant Program Director, Jersey City Medical Center Jersey City Medical Center, Jersey City NJ, USA

Article ID: 100006Z14LD2017
doi:10.5348/Z14-2017-6-CR-3

Address correspondence to:
Luis W. Dominguez MD
MPH. Department of Internal
Medicine, Jersey City Medical Center
355 Grand St. Jersey City
NJ. 07302. USA

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How to cite this article:
Dominguez LW, Marian VD. Macrophage activation syndrome: A nearly fatal presentation, and review of diagnosis and treatment guidelines. J Case Rep Images Orthop Rheum 2017;2:12–19.


ABSTRACT
Introduction: Macrophage activation syndrome (MAS) is a rare but life-threatening systemic inflammatory complication, usually arising from superimposed viral infections or flares of underlying rheumatological conditions. It is characterized by an uncontrolled immune response, involving the expansion of T-Cells, Macrophages, and the hypersecretion of pro-inflammatory cytokines. The pathophysiology of this disease is not fully understood, and the diagnostic criteria have been debated. In 2016, an international panel of experts released new guidelines for the classification criteria of MAS, with greater sensitivity, specificity, and laboratory guidelines. Our case provides support for these new guidelines, and is possibly the first published after their release. Furthermore, treatment using glucocorticoids and alternative immunosuppressants is also reviewed.
Case Report: A 33-year-old Hispanic male without significant history presented with an unremitting fever for five days, associated with myalgias, fatigue, night sweats, and a rash. Upon admission, he was tachycardic, hypotensive and febrile at 103°F (39.4°C). Physical examination revealed a diffuse, non-blanching, maculopapular rash sparing the face, palms and soles. Labs revealed pancytopenia, transaminitis, elevated ferritin, and low fibrinogen. He was started on empiric antibiotics. Blood cultures were drawn and returned negative, yet he continued to spike fevers. On 4th day, he suffered acute respiratory distress syndrome (ARDS), requiring mechanical ventilation. He was transferred to the intensive care unit (ICU) where he continued to deteriorate. Given the persistent fever, pancytopenia, transaminitis, low fibrinogen and elevated ferritin, MAS was considered, based on recent classification criteria. He was started on high-dose methylprednisolone. He rapidly recovered, and was extubated within 24 hours and discharged later that week.
Conclusion Macrophage activation syndrome is a rare condition with a debated definition, and a mortality rate of up to 30%. As a result, it remains a diagnostic challenge. This case supports the new 2016 guidelines and confirms methylprednisolone as a first-line treatment. Furthermore, MAS seems to have occurred in the absence of underlying rheumatological disease.


Keywords: Adult still’s disease, Diagnostic criteria for macrophage activation syndrome, Macrophage activation syndrome (MAS)

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Author Contributions
Luis W. Dominguez – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Valentin D. Marian – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2017 Luis W. Dominguez et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



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