
New-onset Systemic Lupus Erythematosus presenting with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage: a case-based review
Author(s) -
Gonçalo Cruz,
Mariana Sousa,
Sara Vilela,
Rita Gouveia
Publication year - 2022
Publication title -
brazilian journal of case reports
Language(s) - English
Resource type - Journals
ISSN - 2763-583X
DOI - 10.52600/2763-583x.bjcr.2022.2.2.89-101
Subject(s) - macrophage activation syndrome , diffuse alveolar hemorrhage , medicine , cyclophosphamide , complication , macrophage , alveolar macrophage , immunology , hemophagocytic lymphohistiocytosis , pathology , chemotherapy , arthritis , biochemistry , chemistry , disease , in vitro
Macrophage activation syndrome is a form of secondary hemophagocytic lymphohistiocytosis in the setting of autoimmune diseases. It’s a rare, hyperinflammatory complication requiring prompt institution of therapy to prevent organ dysfunction and death. We report a young female presenting with macrophage activation syndrome with multiorgan failure and diffuse alveolar hemorrhage in the setting of inaugural systemic lupus erythematosus. The patient was successfully treated with steroids, plasma exchange and cyclophosphamide.