학술논문
Autoinflammatory diseases: a Latin American multicenter study according to age and sex
Document Type
article
Author
Daniela Gerent Petry Piotto; Katia Kozu; Nádia Emi Aikawa; Pedro Lopes Carneiro; María Martha Katsicas; Sheila Knupp Feitosa de Oliveira; Taciana de Albuquerque Pedrosa Fernandes; Claudia Saad Magalhães; Ana Luiza Garcia Cunha; Blanca Elena Rios Gomes Bica; Carlos Nobre Rabelo Júnior; Cristina Battagliotti; Erica Naomi Naka Matos; Flavia Patrícia Sena Teixeira Santos; Flavio Roberto Sztajnbok; Liliana Bezrodnik; Marcia Bandeira; Marta Cristine Felix Rodrigues; Pablo García Munittis; Simone Appenzeller; Teresa Cristina Martins Robazzi; Gleice Clemente; Clovis Artur Silva; Maria Teresa Terreri
Source
Revista Paulista de Pediatria, Vol 42 (2023)
Subject
Language
English
Spanish; Castilian
Portuguese
Spanish; Castilian
Portuguese
ISSN
1984-0462
Abstract
ABSTRACT Objective: To evaluate autoinflammatory diseases (AID) according to age at diagnosis and sex, and response to therapy in a large population. Methods: This is a cross-sectional observational study of a Latin American registry using a designed web system for data storage, collected between 2015 and 2018. Any altered findings during follow-up were recorded. The forms were translated into Portuguese and Spanish, including demographic, clinical, laboratory, genetic and treatment characteristics. Results: We included 152 patients, 51.3% male and 75% Caucasian. The median age at disease onset was 2.1 years (0–15.6 years) and median age at diagnosis 6.9 years (0–21.9 years); 111 (73%) were children (0–9 years old), and 41 (27%) were adolescents and young adults (AYA) (10–21 years old). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) occurred in 46/152 (30%), chronic non-bacterial osteomyelitis (CNO) in 32/152 (21%), and familial Mediterranean fever (FMF) in 24/152 (15.7%). PFAPA was significantly higher in young children than in AYA (38.7% vs. 7.3%, p