https://doi.org/10.4081/reumatismo.2022.1488

Unmasking of systemic lupus erythematosus in a patient with hemophagocytic lymphohistiocytosis- macrophage activation syndrome (HLA-MAS) and diffuse alveolar hemorrhage

Vol. 74 No. 4 (2022)
Submitted: 12 March 2022
Accepted: 1 January 2023
Published: 21 March 2023
Hemophagocytic lymphohistiocytosis, systemic lupus erythematosus, ferritin, diffuse alveolar hemorrhage, pancytopenia
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Authors

P. Sukhadiya
Department of Internal Medicine, All India Institute of Medical Sciences, Jodhpur, India.
P. Kumar
Department of Internal Medicine, All India Institute of Medical Sciences, Jodhpur, India.
D.S. Meena
dsmims14@gmail.com
https://orcid.org/0000-0001-9524-5278
Department of Internal Medicine, All India Institute of Medical Sciences, Jodhpur, India.
A. Kumar P.H.
Department of Internal Medicine, All India Institute of Medical Sciences, Jodhpur, India.
N. Vijayan
https://orcid.org/0000-0003-0066-6470
Department of Internal Medicine, All India Institute of Medical Sciences, Jodhpur, India.
P. Garg
Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, India.
M.K. Garg
Department of Internal Medicine, All India Institute of Medical Sciences, Jodhpur, India.

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by macrophages and cytotoxic T cells with aberrant activation. The primary (genetic) form, which is caused by mutations that affect lymphocyte cytotoxicity and immune regulation, is most prevalent in children, whereas the secondary (acquired) form is prevalent in adults. Secondary HLH is commonly caused by infections or cancers, but it can also be caused by autoimmune disorders, in which case it is known as macrophage activation syndrome (MAS; or MAS-HLH). A 25-year-old female presented with a high-grade fever that lasted for two weeks. His laboratory results revealed pancytopenia, neutropenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Based on the clinical presentation and laboratory findings, a provisional diagnosis of HLH has been made. A HLH protocol was utilized to treat the patient. During the course of hospitalization, systemic lupus erythematosus (SLE) was identified as the underlying cause. She improved dramatically after receiving an immunosuppressive regimen of etoposide, cyclosporine, and dexamethasone according to HLH protocol-2004 with individualized modifications. The clinician should be aware that HLH may be the initial manifestation of underlying SLE. Early diagnosis and aggressive, individualized treatment are the key to improving outcomes.

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How to Cite

Sukhadiya, P., Kumar, P., Meena, D., Kumar P.H., A., Vijayan, N., Garg, P., & Garg, M. (2023). Unmasking of systemic lupus erythematosus in a patient with hemophagocytic lymphohistiocytosis- macrophage activation syndrome (HLA-MAS) and diffuse alveolar hemorrhage. Reumatismo, 74(4). https://doi.org/10.4081/reumatismo.2022.1488
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