Visceral Leishmaniasis Associated with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage in a Lupus Patient
| Autor: | Fernando Salvador, Sofia Cerqueira, Andreia Costa, Cármen Pais |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Adult
Lung Diseases Pathology medicine.medical_specialty lcsh:Medicine Hemorrhage 030204 cardiovascular system & hematology Lymphohistiocytosis Hemophagocytic 03 medical and health sciences 0302 clinical medicine medicine Humans Lupus Erythematosus Systemic Fever of unknown origin lcsh:R5-920 Hemophagocytic lymphohistiocytosis Systemic lupus erythematosus business.industry Macrophage Activation Syndrome lcsh:R Diffuse alveolar hemorrhage General Medicine medicine.disease Lupus Erythematosus Systemic/complications Pulmonary Alveoli Treatment Outcome Visceral leishmaniasis 030220 oncology & carcinogenesis Macrophage activation syndrome Leishmaniasis Visceral Female Rituximab Hemophagocytosis lcsh:Medicine (General) business medicine.drug |
| Zdroj: | Acta Médica Portuguesa, Vol 31, Iss 10, Pp 593-596 (2018) |
| ISSN: | 1646-0758 0870-399X |
| DOI: | 10.20344/amp.9028 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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