CO:11:2 | Systemic lupus erythematosus with major neuropsychiatric involvement treated with anifrolumab: literature review and a case report

Giulia Rizzo¹, Fabio Congiu¹, Elisabetta Chessa², Marta Paola Pireddu¹, Elena Ragusa¹, Alessandra Oliva¹, Marianna Salis¹, Alberto Cauli^1|2, Matteo Piga^1|2 | ¹SC Reumatologia, Dipartimento di Scienze Mediche e Salute Pubblica, Università di Cagliari Italy; ²SC Reumatologia, AOU Cagliari Italy

Background. Anifrolumab is a biologic agent approved for the treatment of Systemic Lupus Erythematosus (SLE). Clinical trials evaluating its efficacy (TULIP-1 and TULIP-2) excluded patients with active neuropsychiatric manifestations (NPSLE). The aim of this study was to review the published cases of NPSLE treated with Anifrolumab (ANI) and to describe a clinical case from our cohort. Materials and Methods. A literature search was conducted on Medline/PubMed and Embase for reports of NPSLE patients treated with ANI. In addition, we describe a case from our local cohort. Results. The literature review identified seven published cases of NPSLE successfully treated with ANI, including two with confusional states, one with acute psychosis, one with headache, one with cerebral vasculitis, one with aseptic meningitis, and one with demyelinating polyneuropathy. The data are summarized in Table 1. We also report the case of a 63-year-old woman with a 26-year history of SLE and antiphospholipid syndrome (APS), who initially presented with polyarthritis, lymphopenia, an ischemic stroke, and positivity for ANA, anti-dsDNA, anti-Ro/SSA, lupus anticoagulant, anti-cardiolipin IgG, and hypocomplementemia. Over the years, she experienced a relapsing-remitting course characterized by cutaneous vasculitis of the hands and feet, thrombocytopenia, leukopenia, and epileptic seizures with spatiotemporal disorientation. Electroencephalography revealed left-hemispheric irritative electrical abnormalities, interpreted as secondary to ischemic damage. The patient’s treatment history included several immunosuppressants—cyclophosphamide, azathioprine, mycophenolate mofetil, belimumab, and hydroxychloroquine (discontinued due to gastrointestinal intolerance, INR abnormalities, and arrhythmia). Levetiracetam was escalated to 2500 mg/day due to worsening seizures, without achieving clinical remission (DORIS). In 2022, following prednisone tapering to 5 mg/day, disease flare occurred with hand vasculitis, malar rash, alopecia, leukopenia (1700/µl), and thrombocytopenia (95,000/µl). Anifrolumab was initiated, and prednisone was increased to 10 mg/day. At the 3-month follow-up, mucocutaneous and hematologic manifestations had resolved, and seizure frequency had decreased (from 2/week to 1 every 8 weeks). After 6 months of ANI treatment, the patient achieved complete clinical remission (CLASI = 0, cSLEDAI-2K = 0) with prednisone tapered to 2.85 mg/day. Conclusions. The results from both the literature review and our clinical case suggest that Anifrolumab may be effective in managing neuropsychiatric manifestations of SLE. In our experience, a remarkable improvement was also observed in the hematologic domain. Although these findings are encouraging, they warrant confirmation in dedicated clinical trials, emphasizing the need for personalized therapeutic strategies in SLE management.