Author(s): Costa, Andreia ; Pais, Cármen ; Cerqueira, Sofia ; Salvador, Fernando
Date: 2018
Origin: Acta Médica Portuguesa
Subject(s): Leishmaniasis; Visceral; Lung Diseases; Lymphohistiocytosis; Hemophagocytic; Lupus Erythematosus; Systemic/complications; Macrophage Activation Syndrome; Treatment Outcome; Doenças do Pulmão; Leishmaniose Visceral; Linfohistiocitose Hemofagocítica; Lúpus Eritematoso Sistémico/complicações; Resultado do Tratamento; Síndrome de Ativação Macrofágica
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 steroidsin weaning scheme.
O lúpus eritematoso sistémico é uma doença autoimune heterogénea e imprevisível, o que pode complicar a sua abordagem e tratamento. A linfohistiocitose hemofagocítica e a hemorragia alveolar difusa são complicações raras da doença. Os autores descrevem o caso de uma mulher de 32 anos, com lúpus e febre de origem indeterminada. Da investigação realizada, o mielograma revelou hemofagocitose e parasitas de Leishmania, pelo que iniciou anfotericina B lipossomal. Manteve febre apesar da terapêutica dirigida e evoluiu com hemorragia alveolar difusa. Repetiu mielograma, mantendo hemofagocitose já sem parasitas, tendo aumentado corticoterapia e iniciado imunoglobulina com melhoria. Dada a presença de síndrome de activação macrofágica e hemorragia alveolar difusa iniciou rituximab. Meses após a alta hospitalar, iniciou novamente febre sustentada e foram novamente identificados parasitas de Leishmania, pelo que reiniciou anfotericina B lipossomal associada a miltefosina. Mantém follow-up, encontrando-se assintomática e com corticóides em esquema de desmame.
