A54-01 A Fog of the Mind: Recognizing Neuropsychiatric Lupus in the Critical Care Setting

Abstract Introduction Neuropsychiatric lupus represents a rare but severe manifestation of systemic lupus erythematosus (SLE), characterized by neurological and psychological manifestations. Neuropsychiatric symptoms of SLE are rare, occurring in the early stages of SLE and in less than 40% of patients (1). Case Presentation A 44-year-old woman with hypertension and obesity initially presented with B symptoms, including fevers, arthralgias, and extensive axillary, inguinal, pelvic, and retroperitoneal lymphadenopathy. Inguinal lymph node excision biopsy was significant for acute inflammation and necrosis; the patient was discharged with outpatient follow-up. However, the patient developed bilateral lower extremity weakness and confusion, leading to a secondary hospitalization. She presented with acute encephalopathy, diffuse plaque-like skin lesions, and oral mucosal lesions. Brain MRI demonstrated symmetric T2/FLAIR hyperintensities in the right gangliocapsular and periventricular regions with foci of microhemorrhage and low-lying cerebellar tonsils, raising concern for encephalitis. CSF analysis was negative for viral and bacterial infection. Further infectious workup, including serologies for HSV, HIV, RPR, hepatitis, Q fever, and histoplasmosis, was negative. Autoimmune testing was remarkable for ANA 1:2560 (homogeneous), dsDNA 300, and low complement levels (C3/C4). Kidney biopsy demonstrated full-house immune complex deposition with mesangial hypercellularity and podocyte effacement, consistent with lupus nephritis. The constellation of encephalopathy, microhemorrhages on MRI, skin lesions, and renal involvement supported a diagnosis of neuropsychiatric SLE. She was started on pulse dose steroids with significant improvement in her mental status. She was discharged home with oral prednisone, mycophenolate mofetil, and hydroxychloroquine. Discussion This case highlights the diagnostic complexity of Neuropsychiatric Lupus SLE. While neuropsychiatric manifestations of SLE can mimic other causes of encephalopathy, including infectious etiologies and delirium, early recognition and aggressive management are crucial in the ICU to ensure good outcomes. Additionally, there are no specific biomarkers or tests to diagnose neuropsychiatric SLE. High clinical suspicion, physical exam, imaging, and histopathologic findings were key to establishing a diagnosis quickly. Medical therapy for neuropsychiatric SLE includes early initiation of pulse dose steroids, and in some cases, additional immunosuppressive therapies may be needed depending on the severity of the disease. Conclusion Neuropsychiatric lupus can present with symptoms of psychosis, multifocal inflammatory encephalitis, and microhemorrhages on brain imaging. Early recognition and initiation of immunosuppressive therapy is critical for neurologic recovery. Reference: (1) Ahn GY, Kim D, Won S, Song ST, Jeong H-J, Sohn I-W, Lee S, Joo Y B, Bae S-C. Prevalence, risk factors, and impact on mortality of neuropsychiatric lupus: a prospective, single-center study. This abstract is funded by: None