AB0548 A COMPARATIVE STUDY BETWEEN THE NEUROPSYCHIATRIC INVOLVEMENTS IN ADULT-ONSET AND CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS

Abstract

BackgroundNeuropsychiatric(NP) manifestations in systemic lupus erythematosus(SLE) encompass a wide spectrum of neurologic and psychiatric features.The nervous system is frequently affected by adult-onset and childhood-onset SLE patients,but disease course and clinical features may differ between both groups.ObjectivesThe aim of this study is to evaluate and compare NP manifestations in adult-onset and childhood-onset SLE patients.MethodsThe study included a retrospective analysis of medical records of adult-onset SLE patients and childhood-onset SLE patients following at Hacettepe University from Jan 2015 to Jan 2021.NP events were identified using the ACR established case definitions seen in SLE.Additionally, posterior reversible encephalopathy syndrome, cerebral venous thrombosis, small fiber neuropathy and pseudotumor cerebri were evaluated as having NPSLE in this study.NP were also divided into three categories:neurologic syndromes of the central nervous system, neurologic syndromes of the peripheral nervous system,and diffuse neuropsychological syndromes.Results1062 adult-onset and 161 childhood-onset SLE patients were followed-up median 150 (84-227) months.At least one neuropsychiatric event occurred in 101 (9.51%) adult-onset and 29 (18.01%) childhood-onset SLE patients (p=0.002). NP events were present before or around the time of SLE diagnosis 64.6% of the adult-onset patients and 72.4% of the childhood-onset patients.One-third of the patients in both groups were diagnosed with NPSLE during the follow-up.At the time of NPSLE diagnosis, 60.4% of adult-onset and 86.2% of childhood-onset patients had additional involvement other than neurologic manifestations and overall disease activity(SLEDAI-2k)was higher in childhood-onset patients (17vs.14p=0.036).Almost all patients in the pediatric group and half of the adult patients were given pulse corticosteroids.Cyclophosphamide was the most preferred regimen and was given to 36.6% of the adult patients and 75.9% of the pediatric patients.Rituximab (11.9% and 27.6%) and mycophenolate mofetil (13.9% and 3.4%) were other induction treatments.The adult-onset and childhood-onset NPSLE patients were followed-up median 105(54-165) and 80(40-132) months, respectively.On the last follow-up visit, disease damage scores (SLICC) were similar in both groups,with a median score of 2.ConclusionThe nervous system was more commonly involved in childhood-onset patients, and disease presentations vary significantly between adult-onset and childhood-onset NPSLE patients.Disease activity appeared to be more severe in children at diagnosis, but permanent organ damage was similar in both groups on follow-up.Table 1.Demographics, clinical features of adult-onset and childhood-onset NPSLE patientsAdult-onset NPSLE (n=101)Childhood-onset NPSLE (n=29)P valueCerebrovascular disease63 (62.4)8 (27.6)0.001Arterial cerebrovascular events44 (43.6)5 (17.2)0.007Central nervous system vasculitis25 (24.8)10 (34.5)0.209Parenchymal involvement18 (17.8)3 (10.3)0.256Demyelinating syndrome14 (13.9)2 (6.9)0.221Transient ischemic attack5 (5.0)-0.277Cerebral venous sinus thrombosis4 (4.0)6 (20.7)0.008Meningeal involvement5 (5.0)-0.277Posterior reversible encephalopathy syndrome3 (3.0)2 (6.9)0.310Pseudotumor cerebri7 (6.9)3 (10.3)0.393Idiopathic intracranial hypertension2 (2.0)3 (10.3)0.073Lupus headache38 (37.6)18 (62.1)0.017Acute confusional state13 (12.9)4 (13.8)0.555Cognitive dysfunction9 (8.9)1 (3.4)0.299Seizure disorders31 (30.7)11 (37.9)0.302Movement disorder (chorea)4 (4.0)2 (6.9)0.402Myasthenia gravis3 (3.0)-0.466Cranial neuropathy7 (6.9)3 (10.3)0.393Optic neuritis4 (4.0)1 (3.4)0.690Peripheral polyneuropathy9 (8.9)5 (17.2)0.173Mononeuritis multiplex1 (1.0)-0.777Myelopathy5 (5.0)2 (6.9)0.489Autonomic dysfunction---Small fiber neuropathy1 (1.0)-0.777Figure 1.Distribution of adult-onset and childhood-onset NPSLE patientsDisclosure of InterestsNone declared