Juvenile-onset systemic lupus erythematosus with multisystem involvement complicated by posterior reversible encephalopathy syndrome (PRES)

A previously healthy girl in her middle childhood of African origin presented with a 2-week history of fever and sore throat. Initial tests showed pancytopenia and proteinuria while the septic screen was negative except for the presence of parvovirus B19 infection. When her symptoms persisted, the possibility of an underlying connective tissue disorder such as systemic lupus erythematosus (SLE) was explored and confirmed. Despite the initiation of glucocorticoids, she went on to develop renal and neuropsychiatric involvement which only responded to high-dose immunosuppression including plasma exchange. This case demonstrates the aggressiveness and rapidity of organ involvement in juvenile-onset SLE and the need for timely appropriate immunosuppression and treatment escalation.

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Publication Details

Journal
BMJ Case Reports
Published
2025-03-01
DOI
https://doi.org/10.1136/bcr-2024-260733
Primary Topic
Systemic Lupus Erythematosus Research
Type
article
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article

Juvenile-onset systemic lupus erythematosus with multisystem involvement complicated by posterior reversible encephalopathy syndrome (PRES)

Erika Cefai, Valerie Said Conti, Cecilia Mercieca, Daniela Grima
BMJ Case Reports
Systemic Lupus Erythematosus Research
article

Juvenile-onset systemic lupus erythematosus with multisystem involvement complicated by posterior reversible encephalopathy syndrome (PRES)

Erika Cefai, Valerie Said Conti, Cecilia Mercieca, Daniela Grima
article en

Abstract

A previously healthy girl in her middle childhood of African origin presented with a 2-week history of fever and sore throat. Initial tests showed pancytopenia and proteinuria while the septic screen was negative except for the presence of parvovirus B19 infection. When her symptoms persisted, the possibility of an underlying connective tissue disorder such as systemic lupus erythematosus (SLE) was explored and confirmed. Despite the initiation of glucocorticoids, she went on to develop renal and neuropsychiatric involvement which only responded to high-dose immunosuppression including plasma exchange. This case demonstrates the aggressiveness and rapidity of organ involvement in juvenile-onset SLE and the need for timely appropriate immunosuppression and treatment escalation.

BMJ Case ReportsVol. 18(3)
Mater Dei Hospital (MT)
Good health and well-being
Openalex Percentile: Top 94%
Systemic Lupus Erythematosus Research
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