Objective:

To present a rare case of primary NPSLE with severe encephalitis and meningitis, highlighting its clinical features, management challenges, and outcomes. This case underscores the importance of early diagnosis and aggressive treatment of this condition.

Background:

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a complication of SLE that affects the nervous system through inflammation or thrombosis (primary NPSLE) or due to immunosuppression or medication side effects (secondary NPSLE). Diagnostic findings include SLE diagnosis with symptoms including altered cognition, neuropsychiatric changes, or neuropathy, and supportive features such as a high SLE Disease Activity Index (SLEDAI) and presence of specific autoantibodies. Treatment involves immunosuppression with glucocorticoids, cyclophosphamide, or anti-B-cell therapy, with intravenous immunoglobulin or plasmapheresis in selected cases. This case highlights a noteworthy case of primary NPSLE's clinical features, management, and outcome.

Design/Methods:

NA

Results:

A 31-year-old female with systemic lupus erythematosus (SLE) presented with fulminant neuropsychiatric SLE (NPSLE), manifesting as encephalitis, meningitis, and confusion. After initial improvement with corticosteroids, she returned with worsening confusion and seizures. Lab results showed elevated lupus serologies, and cerebrospinal fluid (CSF) analysis revealed high protein levels and increased opening pressure. MRI showed bilateral frontal lobe infarcts, with overall evaluation consistent with NPSLE. She received cyclophosphamide, plasmapheresis, and steroids. Complications included cerebral edema, hemophagocytic lymphohistiocytosis (HLH), and candidemia. After intensive treatment, her condition improved, and she was discharged to rehab, showing further progress with outpatient care and rehabilitation.

Conclusions:

This case highlights the need for early recognition and aggressive treatment of NPSLE. Inadequate intervention led to disease progression, requiring cyclophosphamide, plasmapheresis, and high-dose steroids with complications including cerebral edema, HLH, and candidemia. The patient's recovery emphasizes the importance of intensive immunosuppression and early rehabilitation for better outcomes. Prompt diagnosis and aggressive management are crucial in severe NPSLE and recent advances in assessment and therapies may help facilitate this in the future.