학술논문

Macrophage activation syndrome in a patient with systemic lupus erythematosus undergoing cyclophosphamide treatment: a case report
Document Type
article
Source
Reumatismo, Vol 75, Iss 2 (2023)
Subject
Macrophage activation syndrome
systemic lupus erythematosus
hemophagocytic lymphohistiocytosis
infection
Medicine
Internal medicine
RC31-1245
Language
English
Italian
ISSN
0048-7449
2240-2683
Abstract
Macrophage activation syndrome (MAS) is a disorder related to hemophagocytic lymphohistiocytosis and is a life-threatening complication of rheumatic diseases. The diagnosis is challenging because MAS symptoms are quite similar to those of many active autoimmune diseases or severe sepsis. We describe the case of a female patient with systemic lupus erythematosus that presented with symptoms suggesting acute decompensation of autoimmune disease and sepsis. She was later diagnosed with MAS. Despite an aggressive immunosuppressive treatment, she developed a fatal outcome.