title: Anti-RO/SSA and anti-La/SSB antibodies: Association with mild lupus manifestations in 645 childhood-onset systemic lupus erythematosus.
abstract: BACKGROUND
  To our knowledge there are no studies assessing anti-Ro/SSA and anti-La/SSB autoantibodies in a large population of childhood-systemic lupus erythematosus (cSLE) patients.
  
  
  METHODS
  This was a retrospective multicenter cohort study performed in 10 Pediatric Rheumatology services, São Paulo state, Brazil. Anti-Ro/SSA and anti-La/SSB antibodies were measured by enzyme linked immunosorbent assay (ELISA) in 645 cSLE patients.
  
  
  RESULTS
  Anti-Ro/SSA and anti-La/SSB antibodies were evidenced in 209/645 (32%) and 102/645 (16%) of cSLE patients, respectively. Analysis of cSLE patients with and without anti-Ro/SSA antibodies revealed higher frequencies of malar rash (79% vs. 71%, p=0.032), photosensitivity (73% vs. 65%, p=0.035), cutaneous vasculitis (43% vs. 35%, p=0.046) and musculoskeletal involvement (82% vs. 75%, p=0.046) in spite of long and comparable disease duration in both groups (4.25 vs. 4.58years, p=0.973). Secondary Sjögren syndrome was observed in only five patients with this antibody (2.5% vs. 0%, p=0.0035), two of them with concomitant anti-La/SSB. The presence of associated autoantibodies: anti-Sm (50% vs. 30%, p<0.0001), anti-RNP (39% vs. 21%, p<0.0001) and anti-ribossomal P protein (46% vs. 21%, p=0.002) was also significantly higher in patients with anti-Ro/SAA antibodies. Further evaluation of cSLE patients with the presence of anti-La/SSB antibodies compared to those without these autoantibodies showed that the frequency of alopecia (70% vs. 51%, p=0.0005), anti-Sm (59% vs. 31%, p<0.0001) and anti-RNP (42% vs. 23%, p<0.0001) were significantly higher in the former group.
  
  
  CONCLUSIONS
  Our large multicenter cohort study provided novel evidence in cSLE that anti-Ro/SSA and/or anti-La/SSB antibodies were associated with mild manifestations, particularly cutaneous and musculoskeletal. Secondary Sjögren syndrome was rarely observed in these patients, in spite of comparable frequencies of anti-Ro/SSA and/or anti-La/SSB reported for adult SLE.
authors.affiliation: Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Glaucia V
authors.initials: GV
authors.lastname: Novak
authors.affiliation: Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Mariana
authors.initials: M
authors.lastname: Marques
authors.affiliation: Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Verena
authors.initials: V
authors.lastname: Balbi
authors.affiliation: Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil; Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Natali W S
authors.initials: NW
authors.lastname: Gormezano
authors.affiliation: Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Kátia
authors.initials: K
authors.lastname: Kozu
authors.affiliation: Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Brazil.
authors.forename: Ana P
authors.initials: AP
authors.lastname: Sakamoto
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Rosa M R
authors.initials: RM
authors.lastname: Pereira
authors.affiliation: Pediatric Rheumatology Unit, Universidade Federal de São Paulo, Brazil.
authors.forename: Maria T
authors.initials: MT
authors.lastname: Terreri
authors.affiliation: Pediatric Rheumatology Division, São Paulo State University (UNESP) - Faculdade de Medicina de Botucatu, Brazil.
authors.forename: Claudia S
authors.initials: CS
authors.lastname: Magalhães
authors.affiliation: Pediatric Rheumatology Unit, Irmandade da Santa Casa de Misericórdia de São Paulo, Brazil.
authors.forename: Andressa
authors.initials: A
authors.lastname: Guariento
authors.affiliation: Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Adriana M E
authors.initials: AM
authors.lastname: Sallum
authors.affiliation: Pediatric Rheumatology Unit, State University of Campinas (UNICAMP), Brazil.
authors.forename: Roberto
authors.initials: R
authors.lastname: Marini
authors.affiliation: Pediatric Rheumatology Unit, Ribeirão Preto Medical School - University of São Paulo, Brazil.
authors.forename: Virginia Paes Leme
authors.initials: VP
authors.lastname: Ferriani
authors.affiliation: Pediatric Rheumatology Unit, Hospital Infantil Darcy Vargas, Brazil.
authors.forename: Cássia Maria
authors.initials: CM
authors.lastname: Barbosa
authors.affiliation: Pediatric Rheumatology Unit, Hospital Menino Jesus, Brazil.
authors.forename: Tânia Caroline Monteiro
authors.initials: TC
authors.lastname: de Castro
authors.affiliation: Pontifical Catholic University of Sorocaba, Brazil.
authors.forename: Valéria C
authors.initials: VC
authors.lastname: Ramos
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Bonfá
authors.affiliation: Pediatric Rheumatology Unit, Children's Institute, Faculdade de Medicina da Universidade de São Paulo, Brazil; Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Brazil. Electronic address: clovisaasilva@gmail.com.
authors.forename: Clovis A
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D000328:Adult; D000974:Antibodies, Antinuclear / Q000378:metabolism*; D001324:Autoantigens / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology* / Q000473:pathology; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000974:Antibodies, Antinuclear; D001324:Autoantigens; C035356:SS-A antibodies; C035357:SS-B antibodies
keywords: Anti-La/SSB autoantibodies; Anti-Ro/SSA autoantibodies; Multicenter cohort; Sjögren syndrome and childhood-systemic lupus erythematosus