RT Journal Article
SR Electronic
T1 Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study
JF Lupus Science & Medicine
JO Lupus Sci Med
FD Lupus Foundation of America
SP e000850
DO 10.1136/lupus-2022-000850
VO 10
IS 1
A1 Rory C Monahan
A1 Liesbeth J J Beaart-van de Voorde
A1 Rolf Fronczek
A1 Jeroen de Bresser
A1 Jeroen Eikenboom
A1 Margreet Kloppenburg
A1 Huub A M Middelkoop
A1 Gisela M Terwindt
A1 Nic J A van der Wee
A1 Tom W J Huizinga
A1 Gerda M Steup-Beekman
YR 2023
UL http://lupus.bmj.com/content/10/1/e000850.abstract
AB Background The short-term and long-term outcome of inflammatory neuropsychiatric SLE (NPSLE) with immunosuppressive treatment is largely unknown. We used clinical data from our tertiary referral centre for NPSLE to investigate the type of inflammatory NPSLE manifestations, type of immunosuppressive treatment prescribed for these manifestations and clinical outcomes.Methods All patients with SLE visiting the Leiden University Medical Centre NPSLE clinic between 2007 and 2021 receiving immunosuppressive therapy for neuropsychiatric symptoms were included. Clinical, immunological and radiological information was collected in as standardised way during a 1-day multidisciplinary assessment. In a multidisciplinary consensus meeting, the presence of NPSLE and the type of NPSLE manifestations and treatment were determined. For this study, short-term (0–6 months) and long-term outcomes (7–24 months) of the NP symptoms were assessed by two independent readers and scored on a 7-point Likert scale, ranging from death to resolved.Results In total, 95 out of 398 (24%) patients visiting the NPSLE clinic between 2007 and 2021 received any form of immunosuppressive treatment for 101 separate NPSLE events. The most common NP manifestation was cognitive dysfunction (50%) as identified by formal cognitive assessment, often present in combination with other NPSLE manifestations. Treatment modalities were induction (24%), induction and maintenance (73%) and other therapy (3%). The treatments mostly consisted of (combinations of) prednisone (97%), methylprednisolone (53%), azathioprine (generally 2 mg/kg daily) (49%) and cyclophosphamide (generally induction 750 mg/m2 every 4 weeks for 24 weeks or 500mg biweekly for 12 weeks) (42%). Short-term outcome showed improvement on the Likert scale in 73% (improved: 22%, much improved: 29%, resolved: 22%), no change in 21% and worsening in 6% of patients. Long-term outcome was available for 78 out of 101 events and showed improvement in 70% (improved: 14%, much improved: 28%, resolved: 28%), no change in 17%, worsening in 10% and death in 3% of patients (none directly NPSLE-related).Conclusion The outcome of inflammatory NPSLE after immunosuppressive treatment is generally good, with improvement of neuropsychiatric symptoms occuring in approximately 70% of events.Data are available on reasonable request. The data underlying this article will be shared on reasonable request to the corresponding author (r.c.monahan@lumc.nl).