Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosus
Abstract Background This study aimed to describe the prevalence of the various clinical features and severity of juvenile systemic lupus erythematosus (jSLE) and to assess predictors of AQP4-Ab positivity in jSLE. In addition, we assessed the relationship of AQP4-Abs with neuropsychiatric disorders...
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| Format: | Article |
| Language: | English |
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BMC
2023-05-01
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| Series: | Pediatric Rheumatology Online Journal |
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| Online Access: | https://doi.org/10.1186/s12969-023-00827-6 |
| _version_ | 1797822969921142784 |
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| author | Yasmeen Shaaban Hala EL-Marsafawy Reham M El-Farahaty Sherine El-Ziny Ahmed M EL-Refaey |
| author_facet | Yasmeen Shaaban Hala EL-Marsafawy Reham M El-Farahaty Sherine El-Ziny Ahmed M EL-Refaey |
| author_sort | Yasmeen Shaaban |
| collection | DOAJ |
| description | Abstract Background This study aimed to describe the prevalence of the various clinical features and severity of juvenile systemic lupus erythematosus (jSLE) and to assess predictors of AQP4-Ab positivity in jSLE. In addition, we assessed the relationship of AQP4-Abs with neuropsychiatric disorders and white matter lesions in jSLE. Method For 90 patients with jSLE, demographic data, clinical manifestations, and treatments received were recorded, and all of the patients were underwent clinical examinations, including assessments for the neurological manifestations of jSLE and neuropsychiatric disorders; Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score evaluations; laboratory investigations, including serum AQP4-Ab assays; and 1.5 Tesla brain MRI. Echocardiography and renal biopsy were performed for the indicated patients. Result Fifty-six patients (62.2%) tested positive for AQP4-Abs. These patients were more likely to have higher disease activity scores (p < 0.001); discoid lesions (p = 0.039); neurological disorders (p = 0.001), mainly psychosis and seizures (p = 0.009 and p = 0.032, respectively); renal and cardiac involvement (p = 0.004 and p = 0.013, respectively); lower C3 levels (p = 0.006); white matter hyperintensities (p = 0.008); and white matter atrophy (p = 0.03) than patients who were negative for AQP4-Abs. Furthermore, AQP4-Ab-positive patients were more likely to have received cyclophosphamide (p = 0.028), antiepileptic drugs (p = 0.032) and plasma exchange therapy (p = 0.049). Conclusion jSLE patients with higher severity scores, neurological disorders, or white matter lesions could develop antibodies against AQP4. We recommend more studies for systematic screening of AQP4-Ab positivity in jSLE patients to confirm its relationship with neurological disorders. |
| first_indexed | 2024-03-13T10:17:07Z |
| format | Article |
| id | doaj.art-c49abf316121438bb64823d18391acd8 |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-03-13T10:17:07Z |
| publishDate | 2023-05-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-c49abf316121438bb64823d18391acd82023-05-21T11:10:58ZengBMCPediatric Rheumatology Online Journal1546-00962023-05-012111910.1186/s12969-023-00827-6Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosusYasmeen Shaaban0Hala EL-Marsafawy1Reham M El-Farahaty2Sherine El-Ziny3Ahmed M EL-Refaey4Department of Paediatrics, Mansoura University Children’s HospitalDepartment of Paediatrics, Mansoura University Children’s HospitalDepartment of Clinical Pathology, Faculty of Medicine, Mansoura UniversityFaculty of Medicine, Mansoura UniversityDepartment of Paediatrics, Mansoura University Children’s HospitalAbstract Background This study aimed to describe the prevalence of the various clinical features and severity of juvenile systemic lupus erythematosus (jSLE) and to assess predictors of AQP4-Ab positivity in jSLE. In addition, we assessed the relationship of AQP4-Abs with neuropsychiatric disorders and white matter lesions in jSLE. Method For 90 patients with jSLE, demographic data, clinical manifestations, and treatments received were recorded, and all of the patients were underwent clinical examinations, including assessments for the neurological manifestations of jSLE and neuropsychiatric disorders; Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score evaluations; laboratory investigations, including serum AQP4-Ab assays; and 1.5 Tesla brain MRI. Echocardiography and renal biopsy were performed for the indicated patients. Result Fifty-six patients (62.2%) tested positive for AQP4-Abs. These patients were more likely to have higher disease activity scores (p < 0.001); discoid lesions (p = 0.039); neurological disorders (p = 0.001), mainly psychosis and seizures (p = 0.009 and p = 0.032, respectively); renal and cardiac involvement (p = 0.004 and p = 0.013, respectively); lower C3 levels (p = 0.006); white matter hyperintensities (p = 0.008); and white matter atrophy (p = 0.03) than patients who were negative for AQP4-Abs. Furthermore, AQP4-Ab-positive patients were more likely to have received cyclophosphamide (p = 0.028), antiepileptic drugs (p = 0.032) and plasma exchange therapy (p = 0.049). Conclusion jSLE patients with higher severity scores, neurological disorders, or white matter lesions could develop antibodies against AQP4. We recommend more studies for systematic screening of AQP4-Ab positivity in jSLE patients to confirm its relationship with neurological disorders.https://doi.org/10.1186/s12969-023-00827-6AQP4-AbAquaporinjSLENeuromyelitis optica spectrum disorders (NMOSDs)White matter lesions |
| spellingShingle | Yasmeen Shaaban Hala EL-Marsafawy Reham M El-Farahaty Sherine El-Ziny Ahmed M EL-Refaey Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosus Pediatric Rheumatology Online Journal AQP4-Ab Aquaporin jSLE Neuromyelitis optica spectrum disorders (NMOSDs) White matter lesions |
| title | Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosus |
| title_full | Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosus |
| title_fullStr | Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosus |
| title_full_unstemmed | Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosus |
| title_short | Aquaporin-4 IgG antibodies: predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in Juvenile systemic lupus erythematosus |
| title_sort | aquaporin 4 igg antibodies predictors of positivity and their relationship with neuropsychiatric disorders and white matter lesions in juvenile systemic lupus erythematosus |
| topic | AQP4-Ab Aquaporin jSLE Neuromyelitis optica spectrum disorders (NMOSDs) White matter lesions |
| url | https://doi.org/10.1186/s12969-023-00827-6 |
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