Abstract Details

Title Pediatric Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Presenting as Evans Syndrome and Lupus Cerebritis Mimicking Infectious Encephalitis

Topic Autoimmune Neurology

Presentation(s) P5 - Poster Session 5 (11:45 AM-12:45 PM)

Poster/Presentation
Number 2-011

Objective

To report a rare complex case of neuropsychiatric systemic lupus erythematosus presenting as Evans syndrome and lupus cerebritis mimicking infectious encephalitis, along with diagnostic challenges and management options.

Background Neuropsychiatric involvement affects approximately 20–30% of pediatric SLE patients, yet presentations combining Evans syndrome (autoimmune hemolytic anemia plus thrombocytopenia) and severe central nervous system disease are exceedingly rare. High-dose corticosteroids are the cornerstone of treatment, while escalation to cyclophosphamide, rituximab, or plasmapheresis is indicated for refractory or fulminant disease.

Design/Methods NA

Results We present the case of a 11-year-old boy with a history of nonscarring alopecia and a malar rash who developed progressive fatigue, dyspnea on exertion, jaundice, and acute neuropsychiatric symptoms (irritability and altered sensorium). On admission, he was febrile with nasal and ear bleeding, severe anemia (hemoglobin 2.2 g/dL, reticulocytosis, positive direct Coombs test), and profound thrombocytopenia (platelets 4×10^3/µL). Brain CT showed communicating hydrocephalus. Initial treatment included anti-tubercular therapy (for presumed tubercular meningitis), high-dose corticosteroids, broad-spectrum antibiotics, and daily platelet transfusions. Further evaluation revealed a strongly positive ANA (1:2560, speckled pattern). CSF analysis showed lymphocytic pleocytosis (13 cells/µL) with elevated protein (131 mg/dL) and normal glucose; brain MRI demonstrated diffuse T2/FLAIR hyperintensities. Acyclovir was started pending HSV PCR; both HSV PCR and CSF GeneXpert were negative, and anti-tubercular therapy was discontinued. Despite pulse steroid therapy followed by oral steroids, hydroxychloroquine, and azathioprine, the patient’s neurological status deteriorated. He became comatose (GCS 8), required intubation, and developed refractory seizures despite lacosamide. The patient expired on hospital day 10.

Conclusions This case illustrates a fulminant presentation of suspected systemic lupus erythematosus (SLE) with Evans syndrome and severe neuropsychiatric involvement, highlighting the diagnostic and therapeutic challenges in critical pediatric cases.

Authors/Disclosures
Muhammad Moiz Javed, MD PRESENTER	Dr. Javed has nothing to disclose.
Muhammad S. Raza, Jr., Resident physician	Dr. Raza has nothing to disclose.
Syed Mohsin Raza Bukhari, MBBS	Dr. Bukhari has nothing to disclose.
Aqsa B. Qureshi IV, MBBS	Dr. Qureshi has nothing to disclose.
Hassan Mehdi, MBBS	Dr. Mehdi has nothing to disclose.
Amna Zaheer	Miss Zaheer has nothing to disclose.
Muhammad Salman Sajid, MBBS	Mr. Sajid has nothing to disclose.
Syed Ijlal Ahmed, MD, MBBS	Dr. Ahmed has nothing to disclose.

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