Clinical characterization and outcomes of 85 patients with neurosarcoidosis
Abstract To analyze the frequency and clinical phenotype of neurosarcoidosis (NS) in one of the largest nationwide cohorts of patients with sarcoidosis reported from southern Europe. NS was evaluated according to the Diagnostic Criteria for Central Nervous System and Peripheral Nervous System Sarcoi...
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| Format: | Article |
| Language: | English |
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Nature Portfolio
2021-07-01
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| Series: | Scientific Reports |
| Online Access: | https://doi.org/10.1038/s41598-021-92967-6 |
| _version_ | 1819038985129820160 |
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| author | Manuel Ramos-Casals Roberto Pérez-Alvarez Belchin Kostov Ricardo Gómez-de-la-Torre Carlos Feijoo-Massó Joel Chara-Cervantes Blanca Pinilla Andrés González-García José-Salvador Garcia-Morillo Miguel López-Dupla Begoña De-Escalante Javier Rascón Patricia Perez-Guerrero Mariona Bonet Gracia Cruz-Caparrós Ana Alguacil José-Luis Callejas Eva Calvo Cristina Soler Angel Robles Borja de Miguel-Campo Pedro Oliva-Nacarino Jorge Estela-Herrero Lucio Pallarés Pilar Brito-Zerón Yolanda Blanco the SarcoGEAS-SEMI Registry |
| author_facet | Manuel Ramos-Casals Roberto Pérez-Alvarez Belchin Kostov Ricardo Gómez-de-la-Torre Carlos Feijoo-Massó Joel Chara-Cervantes Blanca Pinilla Andrés González-García José-Salvador Garcia-Morillo Miguel López-Dupla Begoña De-Escalante Javier Rascón Patricia Perez-Guerrero Mariona Bonet Gracia Cruz-Caparrós Ana Alguacil José-Luis Callejas Eva Calvo Cristina Soler Angel Robles Borja de Miguel-Campo Pedro Oliva-Nacarino Jorge Estela-Herrero Lucio Pallarés Pilar Brito-Zerón Yolanda Blanco the SarcoGEAS-SEMI Registry |
| author_sort | Manuel Ramos-Casals |
| collection | DOAJ |
| description | Abstract To analyze the frequency and clinical phenotype of neurosarcoidosis (NS) in one of the largest nationwide cohorts of patients with sarcoidosis reported from southern Europe. NS was evaluated according to the Diagnostic Criteria for Central Nervous System and Peripheral Nervous System Sarcoidosis recently proposed by Stern et al. Pathologic confirmation of granulomatous disease was used to subclassify NS into definite (confirmation in neurological tissue), probable (confirmation in extraneurological tissue) and possible (no histopathological confirmation of the disease). Of the 1532 patients included in the cohort, 85 (5.5%) fulfilled the Stern criteria for NS (49 women, mean age at diagnosis of NS of 47.6 years, 91% White). These patients developed 103 neurological conditions involving the brain (38%), cranial nerves (36%), the meninges (3%), the spinal cord (10%) and the peripheral nerves (14%); no patient had concomitant central and peripheral nerve involvements. In 59 (69%) patients, neurological involvement preceded or was present at the time of diagnosis of the disease. According to the classification proposed by Stern et al., 11 (13%) were classified as a definite NS, 61 (72%) as a probable NS and the remaining 13 (15%) as a possible NS. In comparison with the systemic phenotype of patients without NS, patients with CNS involvement presented a lower frequency of thoracic involvement (82% vs 93%, q = 0.018), a higher frequency of ocular (27% vs 10%, q < 0.001) and salivary gland (15% vs 4%, q = 0.002) WASOG involvements. In contrast, patients with PNS involvement showed a higher frequency of liver involvement (36% vs 12%, p = 0.02) in comparison with patients without NS. Neurosarcoidosis was identified in 5.5% of patients. CNS involvement prevails significantly over PNS involvement, and both conditions do not overlap in any patient. The systemic phenotype associated to each involvement was clearly differentiated, and can be helpful not only in the early identification of neurological involvement, but also in the systemic evaluation of patients diagnosed with neurosarcoidosis. |
| first_indexed | 2024-12-21T08:46:00Z |
| format | Article |
| id | doaj.art-1e44c96df86b41beb3c7a917cfef4cc5 |
| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-12-21T08:46:00Z |
| publishDate | 2021-07-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-1e44c96df86b41beb3c7a917cfef4cc52022-12-21T19:09:49ZengNature PortfolioScientific Reports2045-23222021-07-0111111410.1038/s41598-021-92967-6Clinical characterization and outcomes of 85 patients with neurosarcoidosisManuel Ramos-Casals0Roberto Pérez-Alvarez1Belchin Kostov2Ricardo Gómez-de-la-Torre3Carlos Feijoo-Massó4Joel Chara-Cervantes5Blanca Pinilla6Andrés González-García7José-Salvador Garcia-Morillo8Miguel López-Dupla9Begoña De-Escalante10Javier Rascón11Patricia Perez-Guerrero12Mariona Bonet13Gracia Cruz-Caparrós14Ana Alguacil15José-Luis Callejas16Eva Calvo17Cristina Soler18Angel Robles19Borja de Miguel-Campo20Pedro Oliva-Nacarino21Jorge Estela-Herrero22Lucio Pallarés23Pilar Brito-Zerón24Yolanda Blanco25the SarcoGEAS-SEMI RegistryDepartment of Autoimmune Diseases, ICMiD, Hospital ClínicDepartment of Internal Medicine, Hospital Alvaro CunqueiroPrimary Healthcare Transversal Research Group, IDIBAPS, Primary Care Center Les Corts, CAPSBEDepartment of Internal Medicine, Hospital Universitario Central de Asturias (HUCA)Department of Internal Medicine, Hospital Parc TauliDepartment of Internal Medicine, Hospital Josep TruetaDepartment of Internal Medicine, Hospital Gregorio MarañónDepartment of Internal Medicine, Hospital Ramon y CajalDepartment of Internal Medicine, Hospital Virgen del RocioDepartment of Internal Medicine, Hospital Joan XXIIIDepartment of Internal Medicine, Hospital ClínicoDepartment of Internal Medicine, Hospital Son EspasesDepartment of Internal Medicine, Hospital Puerta del MarDepartment of Internal Medicine, Althaia, Xarxa Assistencial de ManresaDepartment of Internal Medicine, Hospital de Poniente de El EjidoDepartment of Internal Medicine, Hospital Virgen de la SaludDepartment of Internal Medicine, Hospital San CecilioDepartment of Internal Medicine, Hospital San JorgeDepartment of Internal Medicine, Hospital Santa CaterinaDepartment of Internal Medicine, Hospital La PazDepartment of Internal Medicine, Hospital 12 de OctubreDepartment of Neurology. Hospital, Universitario Central de Asturias (HUCA)Department of Neurology, Hospital Parc TauliDepartment of Internal Medicine, Hospital Son EspasesSystemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital CIMA-SanitasDepartment of Neurology, Hospital ClínicAbstract To analyze the frequency and clinical phenotype of neurosarcoidosis (NS) in one of the largest nationwide cohorts of patients with sarcoidosis reported from southern Europe. NS was evaluated according to the Diagnostic Criteria for Central Nervous System and Peripheral Nervous System Sarcoidosis recently proposed by Stern et al. Pathologic confirmation of granulomatous disease was used to subclassify NS into definite (confirmation in neurological tissue), probable (confirmation in extraneurological tissue) and possible (no histopathological confirmation of the disease). Of the 1532 patients included in the cohort, 85 (5.5%) fulfilled the Stern criteria for NS (49 women, mean age at diagnosis of NS of 47.6 years, 91% White). These patients developed 103 neurological conditions involving the brain (38%), cranial nerves (36%), the meninges (3%), the spinal cord (10%) and the peripheral nerves (14%); no patient had concomitant central and peripheral nerve involvements. In 59 (69%) patients, neurological involvement preceded or was present at the time of diagnosis of the disease. According to the classification proposed by Stern et al., 11 (13%) were classified as a definite NS, 61 (72%) as a probable NS and the remaining 13 (15%) as a possible NS. In comparison with the systemic phenotype of patients without NS, patients with CNS involvement presented a lower frequency of thoracic involvement (82% vs 93%, q = 0.018), a higher frequency of ocular (27% vs 10%, q < 0.001) and salivary gland (15% vs 4%, q = 0.002) WASOG involvements. In contrast, patients with PNS involvement showed a higher frequency of liver involvement (36% vs 12%, p = 0.02) in comparison with patients without NS. Neurosarcoidosis was identified in 5.5% of patients. CNS involvement prevails significantly over PNS involvement, and both conditions do not overlap in any patient. The systemic phenotype associated to each involvement was clearly differentiated, and can be helpful not only in the early identification of neurological involvement, but also in the systemic evaluation of patients diagnosed with neurosarcoidosis.https://doi.org/10.1038/s41598-021-92967-6 |
| spellingShingle | Manuel Ramos-Casals Roberto Pérez-Alvarez Belchin Kostov Ricardo Gómez-de-la-Torre Carlos Feijoo-Massó Joel Chara-Cervantes Blanca Pinilla Andrés González-García José-Salvador Garcia-Morillo Miguel López-Dupla Begoña De-Escalante Javier Rascón Patricia Perez-Guerrero Mariona Bonet Gracia Cruz-Caparrós Ana Alguacil José-Luis Callejas Eva Calvo Cristina Soler Angel Robles Borja de Miguel-Campo Pedro Oliva-Nacarino Jorge Estela-Herrero Lucio Pallarés Pilar Brito-Zerón Yolanda Blanco the SarcoGEAS-SEMI Registry Clinical characterization and outcomes of 85 patients with neurosarcoidosis Scientific Reports |
| title | Clinical characterization and outcomes of 85 patients with neurosarcoidosis |
| title_full | Clinical characterization and outcomes of 85 patients with neurosarcoidosis |
| title_fullStr | Clinical characterization and outcomes of 85 patients with neurosarcoidosis |
| title_full_unstemmed | Clinical characterization and outcomes of 85 patients with neurosarcoidosis |
| title_short | Clinical characterization and outcomes of 85 patients with neurosarcoidosis |
| title_sort | clinical characterization and outcomes of 85 patients with neurosarcoidosis |
| url | https://doi.org/10.1038/s41598-021-92967-6 |
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