Visceral Leishmaniasis Associated with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage in a Lupus Patient

Andreia Costa, Cármen Pais, Sofia Cerqueira, Fernando Salvador


Systemic lupus erythematosus is a heterogeneous and unpredictable autoimmune disease which can be complicated to approach and treat. Hemophagocytic lymphohistiocytosis and diffuse alveolar hemorrhage are rare disease complications. The authors describe a clinical case of a 32-year-old woman with lupus and fever of unknown origin. From the investigations performed, the myelogram revealed hemophagocytosis and Leishmania parasites, therefore liposomal amphotericin B was then started. In addition to directed therapy, she maintained fever that evolved with diffuse alveolar hemorrhage. The myelogram was repeated and showed that she still had hemophagocytosis but now without parasites. Corticotherapy was increased and intravenous Immunoglobulin was started, with improvement. Rituximab was started as a result of macrophage activation syndrome and diffuse alveolar hemorrhage. Months after discharge, she began once again to have sustained fever and Leishmania parasites were found again, therefore liposomal amphotericin B was started once more associated with miltefosine. She continues being followed-up as she is asymptomatic and using steroids
in weaning scheme.


Leishmaniasis, Visceral; Lung Diseases; Lymphohistiocytosis, Hemophagocytic; Lupus Erythematosus, Systemic/complications; Macrophage Activation Syndrome; Treatment Outcome

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