Idioma:

Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children

Ramos-Casals, Manuel ; Acar-Denizli, Nihan ; Vissink, Arjan ; Brito-Zerón, Pilar ; Li, Xiaomei ; Carubbi, Francesco ; Priori, Roberta ; Toplak, Nataša ; Baldini, Chiara ; Faugier-Fuentes, Enrique ; Kruize, Aike A ; Mandl, Thomas ; Tomiita, Minako ; Gandolfo, Saviana ; Hashimoto, Kunio ; Hernandez-Molina, Gabriela ; Hofauer, Benedikt ; Mendieta-Zerón, Samara ; Rasmussen, Astrid ; Sandhya, Pulukool ; Sene, Damien ; Trevisani, Virginia Fernandes Moça ; Isenberg, David ; Sundberg, Erik ; Pasoto, Sandra G ; Sebastian, Agata ; Suzuki, Yasunori ; Retamozo, Soledad ; Xu, Bei ; Giacomelli, Roberto ; Gattamelata, Angelica ; Bizjak, Masa ; Bombardieri, Stefano ; Loor-Chavez, Richard-Eduardo ; Hinrichs, Anneline ; Olsson, Peter ; Bootsma, Hendrika ; Lieberman, Scott M

Rheumatology (Oxford, England), 2021-10, Vol.60 (10), p.4558-4567 [Periódico revisado por pares]

England: Oxford University Press

Texto completo disponível

Citações Citado por

Enviar para

Título:
Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children
Autor: Ramos-Casals, Manuel ; Acar-Denizli, Nihan ; Vissink, Arjan ; Brito-Zerón, Pilar ; Li, Xiaomei ; Carubbi, Francesco ; Priori, Roberta ; Toplak, Nataša ; Baldini, Chiara ; Faugier-Fuentes, Enrique ; Kruize, Aike A ; Mandl, Thomas ; Tomiita, Minako ; Gandolfo, Saviana ; Hashimoto, Kunio ; Hernandez-Molina, Gabriela ; Hofauer, Benedikt ; Mendieta-Zerón, Samara ; Rasmussen, Astrid ; Sandhya, Pulukool ; Sene, Damien ; Trevisani, Virginia Fernandes Moça ; Isenberg, David ; Sundberg, Erik ; Pasoto, Sandra G ; Sebastian, Agata ; Suzuki, Yasunori ; Retamozo, Soledad ; Xu, Bei ; Giacomelli, Roberto ; Gattamelata, Angelica ; Bizjak, Masa ; Bombardieri, Stefano ; Loor-Chavez, Richard-Eduardo ; Hinrichs, Anneline ; Olsson, Peter ; Bootsma, Hendrika ; Lieberman, Scott M
Assuntos: Adolescent ; Age of Onset ; autoimmune diseases ; childhood ; Clinical Medicine ; epidemiology ; Female ; Humans ; Klinisk medicin ; Male ; Medical and Health Sciences ; Medicin och hälsovetenskap ; paediatrics ; Parotid Gland - pathology ; Phenotype ; Registries ; Reumatologi och inflammation ; Rheumatology and Autoimmunity ; Severity of Illness Index ; Sjogren's syndrome ; Sjogren's Syndrome - diagnosis ; Sjogren's Syndrome - pathology
É parte de: Rheumatology (Oxford, England), 2021-10, Vol.60 (10), p.4558-4567
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
Descrição: Abstract Objectives To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren’s syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. Conclusions Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.
Editor: England: Oxford University Press
Idioma: Inglês

Links

Voltar para lista de resultados

Realização: Logos de Redes Sociais:

Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children

England: Oxford University Press

Buscando em bases de dados remotas. Favor aguardar.