Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study

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...

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Main Authors: Margreet Kloppenburg, Tom W J Huizinga, Jeroen Eikenboom, Gisela M Terwindt, Liesbeth J J Beaart-van de Voorde, Gerda M Steup-Beekman, Jeroen de Bresser, Rory C Monahan, Huub A M Middelkoop, Nic J A van der Wee, Rolf Fronczek
Format: Article
Language:English
Published: BMJ Publishing Group 2023-01-01
Series:Lupus Science and Medicine
Online Access:https://lupus.bmj.com/content/10/1/e000850.full
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author Margreet Kloppenburg
Tom W J Huizinga
Jeroen Eikenboom
Gisela M Terwindt
Liesbeth J J Beaart-van de Voorde
Gerda M Steup-Beekman
Jeroen de Bresser
Rory C Monahan
Huub A M Middelkoop
Nic J A van der Wee
Rolf Fronczek
author_facet Margreet Kloppenburg
Tom W J Huizinga
Jeroen Eikenboom
Gisela M Terwindt
Liesbeth J J Beaart-van de Voorde
Gerda M Steup-Beekman
Jeroen de Bresser
Rory C Monahan
Huub A M Middelkoop
Nic J A van der Wee
Rolf Fronczek
author_sort Margreet Kloppenburg
collection DOAJ
description 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.
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spelling doaj.art-aa2053b0c76b4624a8f355b4cd3d2c162023-07-26T12:35:07ZengBMJ Publishing GroupLupus Science and Medicine2053-87902023-01-0110110.1136/lupus-2022-000850Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort studyMargreet Kloppenburg0Tom W J Huizinga1Jeroen Eikenboom2Gisela M Terwindt3Liesbeth J J Beaart-van de Voorde4Gerda M Steup-Beekman5Jeroen de Bresser6Rory C Monahan7Huub A M Middelkoop8Nic J A van der Wee9Rolf Fronczek10Department of Rheumatology, Leiden University Medical Centre (LUMC), Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, NetherlandsDepartment of Internal Medicine, Division of Thrombosis and Hemostasis, LUMC, Leiden, The NetherlandsDepartment of Neurology, LUMC, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Center, Leiden, The NetherlandsDepartment of Rheumatology, Haaglanden Medical Centre, the Hague, The NetherlandsDepartment of Radiology, LUMC, Leiden, The NetherlandsDepartment of Rheumatology, Leiden University Medical Centre (LUMC), Leiden, The NetherlandsDepartment of Neurology, LUMC, Leiden, The NetherlandsDepartment of Psychiatry, LUMC, Leiden, The NetherlandsDepartment of Neurology, LUMC, Leiden, The NetherlandsBackground 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.https://lupus.bmj.com/content/10/1/e000850.full
spellingShingle Margreet Kloppenburg
Tom W J Huizinga
Jeroen Eikenboom
Gisela M Terwindt
Liesbeth J J Beaart-van de Voorde
Gerda M Steup-Beekman
Jeroen de Bresser
Rory C Monahan
Huub A M Middelkoop
Nic J A van der Wee
Rolf Fronczek
Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study
Lupus Science and Medicine
title Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study
title_full Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study
title_fullStr Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study
title_full_unstemmed Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study
title_short Clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression: an observational cohort study
title_sort clinical outcome in patients with suspected inflammatory neuropsychiatric lupus treated with immunosuppression an observational cohort study
url https://lupus.bmj.com/content/10/1/e000850.full
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