Case report and literature review: neuropsychiatric systemic lupus erythematosus presenting as massive intracerebral hemorrhage

Neuropsychiatric systemic lupus erythematosus (NPSLE) encompasses a spectrum of central and peripheral nervous system manifestations. While cerebrovascular events occur in 5%-15% of NPSLE patients, intracerebral hemorrhage (ICH) as the initial presentation of systemic lupus erythematosus (SLE) is exceptionally rare and diagnostically challenging. A 17-year-old female presented with nephrotic syndrome and suddenly developed massive left frontal ICH requiring emergency craniotomy. Vascular malformations were excluded by digital subtraction angiography and intraoperative inspection. Subsequent immunological workup revealed a single positive antinuclear antibody, elevated anti-dsDNA antibodies, positive anti-ribosomal P antibody, positive lupus anticoagulant (LA), and hypocomplementemia, supporting the diagnosis of SLE with active lupus nephritis. She achieved multisystem recovery following aggressive immunosuppressive therapy. In the literature review, 12 clinical studies and 12 case reports were identified. The incidence of ICH was low (0.4%–1.5%), but the associated mortality risk was high (exceeding 20%). Compared with the non-SLE population, patients with SLE face a 2- to 3-fold higher risk of ICH. In case reports, all patients were female, 83.3% were aged ≤ 35 years, and 58.3% presented with ICH at the time of SLE diagnosis. Active lupus nephritis coexisted in 41.7% of cases. Common risk factors associated with the disease include young age, high disease activity, concomitant antiphospholipid syndrome, thrombocytopenia, and the use of antiplatelet agents. NPSLE should be considered in young female patients presenting with spontaneous ICH accompanied by multisystem involvement, particularly when conventional vascular risk factors are absent. The triad of active lupus nephritis, hypocomplementemia, and LA positivity may be associated with a high-risk phenotype for SLE-associated ICH. Timely recognition and appropriate immunomodulation may be important for improving outcomes.