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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BMJ Publishing Group
2023-01-01
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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. |
first_indexed | 2024-03-12T21:45:31Z |
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id | doaj.art-aa2053b0c76b4624a8f355b4cd3d2c16 |
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issn | 2053-8790 |
language | English |
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publishDate | 2023-01-01 |
publisher | BMJ Publishing Group |
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series | Lupus Science and Medicine |
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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