Pediatric Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) Presenting as Evans Syndrome and Lupus Cerebritis Mimicking Infectious Encephalitis
Muhammad Raza1, Syed Mohsin Raza Bukhari2, Aqsa Qureshi1, Hassan Mehdi2, Amna Zaheer3, Muhammad Salman Sajid2, Muhammad Moiz Javed4, Syed Ijlal Ahmed5
1Indus Hospital - Recep Tayyip Erdogan Campus, Muzaffargarh, 2Nishtar Medical University and Hospital, Multan, 3Liaquat National Hospital and Medical College, 4Department of Neurology, Geisinger Wyoming Valley Medical Center, 5Department of Neurology, Saint Francis Medical Center
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.
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.
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