1 - Pediatric Rheumatology Clinics, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, E.P.E.;
2 - Pediatric Hematology Clinics, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, E.P.E.;
3 - Intensive Care Unit, Hospital Dona Estefânia, Centro Hospitalar Lisboa Central, E.P.E.
4 - Pediatric Cardiology, Hospital da Cruz Vermelha, Portugal;
5 - Pediatric Rheumatology Unit, Instituto Português de Reumatologia (IPR), Lisbon, Portugal;
6 - Department of Pediatrics, Hospital Prof. Doutor Fernando Fonseca E.P.E, Amadora, Portugal.
- Ann Paediatr Rheum. 2012; 1(1): 71-76 doi: 10.5455/apr.120120111141.
Although sickle cell disease (SCD) and systemic lupus erythematosus (SLE) are two distinct and relatively common chronic diseases, coexistence of these two conditions in the same patient appears to be rare. The authors report an eight-year-old child with SCD who developed a severe form of parvovirus B19-associated SLE, with secondary severe immune hemolytic anemia related to drugs, Libman-Sacks endocarditis complicated by severe aortic regurgitation, dilated left ventricle with impaired function and myocardial ischemia, with further decompensation culminating in cardiac arrest during an infectious intercurrence, which led inevitably to death. This patient displayed a broad spectrum of musculoskeletal, hematologic and cardiovascular complications, which could be associated with either SCD or SLE. Conclusion: Because of a substantial overlap between the clinical manifestations of these two disorders, the diagnosis of SLE in a patient with a previous known diagnosis of SCD may be difficult and is often delayed. Our report illustrates the importance of considering other disease processes, like autoimmune diseases when clinical features or its evolution are atypical of SCD and emphasizes some of the diagnostic difficulties encountered during the diagnosis and management of these patients.
Key words: Systemic lupus erythematosus, sickle cell disease, child, Libman-Sacks endocarditis, parvovirus B19.