Purpose Neuropsychiatric manifestations in patients with systemic lupus erythematosus (SLE) can have many underlying causes, including inflammation (inflammatory NPSLE). Inflammatory NPSLE is rare and many aspects of inflammatory NPSLE, including treatment and treatment outcomes, remain to be elucidated. Therefore, we used clinical data from our tertiary referral centre for NPSLE to investigate inflammatory NPSLE manifestations, type of immunosuppressive treatment and clinical outcomes.
Methods All patients with the clinical diagnosis of SLE visiting the LUMC NPSLE clinic between 2007–2021 that received any type of immunosuppressive therapy for NP symptoms were included. Clinical data was collected in a standardized way during a multidisciplinary evaluation at the NPSLE clinic and this information was later retrieved from patient records Patients were followed-up prospectively and clinical outcomes (short-term, 0–6 months and long-term outcomes, 7–24 months) were assessed by two independent readers and scored on a 7-point Likert scale, ranging from death to resolved.
Results In a total of 101 NPSLE events in 95/398 patients (24%) visiting the NPSLE clinic, immunosuppressive treatment was initiated. The most common inflammatory NP manifestation was (severe) cognitive dysfunction (50%), often present in combination with other NPSLE manifestations (such as myelopathy and neuropathy). Interestingly, 24 patients (24%) had clear NP manifestations that are not part of the 1999 ACR case definitions for NPSLE (e.g. cerebral vasculitis and organic brain syndrome). The most common treatment modality was a combination of induction and maintenance therapy (73%), followed by solely induction therapy (24%) and other types of therapy. Nearly all patients were treated with prednisone (97%), often in combination with methylprednisolone (53%), azathioprine (49%) and cyclophosphamide (42%). Short-term outcome showed improvement on the Likert scale in 73% of patient (improved: 22%, much improved: 29%, resolved: 22%). No change was observed in 21% of patients and worsening in 6% of patients (see also Figure 1). Long-term outcome was available for 78/101 events and an improvement of the NP manifestations was seen in 70% of patients (improved:14%, much improved: 28%, resolved: 28%). No change was seen in 17% of patients, worsening in 10% and death in 3% of patients. None of the deaths were directly related to NPSLE, whereas one was potentially related to immunosuppressive treatment.
Conclusion Although NPSLE is considered as one of the major organ involvements of SLE, the outcome of inflammatory NPSLE after immunosuppressive treatment is generally good, with improvement in approximately 70% of events.
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