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
Submitted: 12 March 2022
Accepted: 1 January 2023
Published: 21 March 2023
Accepted: 1 January 2023
Abstract Views: 1103
PDF: 534
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
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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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