title: Clinical features and long-term outcomes of systemic lupus erythematosus: comparative data of childhood, adult and late-onset disease in a national register.
abstract: Systemic lupus erythematosus (SLE) affects predominantly women at reproductive age but may present at any age. Age at disease onset has a modulating effect on presentation and course of disease, but controversies persist regarding its impact on long-term outcome. Our aims were to characterize clinical features, co-morbidities and cumulative damage in childhood-onset, adult-onset and late-onset SLE. Patients with childhood-onset SLE fulfilling ACR 1997 criteria were identified in a nationwide register-Reuma.pt/SLE (N = 89) and compared with adult-onset and late-onset counterparts matched 1:1:1 for disease duration. 267 SLE patients with mean disease duration of 11.9 ± 9.3 years were analyzed. Skin (62 %), kidney (58 %), neurological (11 %) and hematologic involvement (76 %) were significantly more common in childhood-onset SLE and disease activity was higher in this subset than in adult- and late-onset disease (SLEDAI-2K 3.4 ± 3.8 vs. 2.2 ± 2.7 vs. 1.6 ± 2.8, respectively; p = 0.004). Also, more childhood-onset patients received cyclophosphamide (10 %) and mycophenolate mofetil (34 %). A greater proportion of women (96 %), prevalence of arthritis (89 %) and anti-SSA antibodies (34 %) were noted in the adult-onset group. There was a significant delay in the diagnosis of SLE in older ages. Co-morbidities such as hypertension, diabetes and thyroid disease were significantly more frequent in late-onset SLE, as well as the presence of irreversible damage evaluated by the SLICC/ACR damage index (20 vs. 26 vs. 40 %; p < 0.001). Greater organ involvement as well as the frequent need for immunosuppressants supports the concept of childhood-onset being a more severe disease. In contrast, disease onset is more indolent but co-morbidity burden and irreversible damage are greater in late-onset SLE, which may have implications for patients' management.
authors.affiliation: Hospital Garcia de Orta, Almada, Portugal. sandrainsousa@gmail.com.
authors.forename: S
authors.initials: S
authors.lastname: Sousa
authors.affiliation: Hospital de Santa Maria, Lisbon, Portugal.|Rheumatology Research Unit, Instituto de Medicina Molecular, Lisbon, Portugal.
authors.forename: M J
authors.initials: MJ
authors.lastname: Gonçalves
authors.affiliation: Hospitais da Universidade de Coimbra, Coimbra, Portugal.|School of Health Sciences, University of Beira Interior, Covilhã, Portugal.
authors.forename: L S
authors.initials: LS
authors.lastname: Inês
authors.affiliation: Hospitais da Universidade de Coimbra, Coimbra, Portugal.
authors.forename: G
authors.initials: G
authors.lastname: Eugénio
authors.affiliation: Hospitais da Universidade de Coimbra, Coimbra, Portugal.
authors.forename: D
authors.initials: D
authors.lastname: Jesus
authors.affiliation: Instituto Português de Reumatologia, Lisbon, Portugal.
authors.forename: S
authors.initials: S
authors.lastname: Fernandes
authors.affiliation: Centro Hospitalar de São João, Porto, Portugal.
authors.forename: G
authors.initials: G
authors.lastname: Terroso
authors.affiliation: Hospital de Santa Maria, Lisbon, Portugal.|Rheumatology Research Unit, Instituto de Medicina Molecular, Lisbon, Portugal.
authors.forename: V C
authors.initials: VC
authors.lastname: Romão
authors.affiliation: Hospital Conde de Bertiandos, Ponte de Lima, Portugal.
authors.forename: M
authors.initials: M
authors.lastname: Cerqueira
authors.affiliation: Hospital Conde de Bertiandos, Ponte de Lima, Portugal.
authors.forename: A
authors.initials: A
authors.lastname: Raposo
authors.affiliation: Hospital de Viseu, Viseu, Portugal.
authors.forename: M
authors.initials: M
authors.lastname: Couto
authors.affiliation: Hospital Egas Moniz, Lisbon, Portugal.
authors.forename: P
authors.initials: P
authors.lastname: Nero
authors.affiliation: Centro Hospitalar de Faro, Faro, Portugal.
authors.forename: G
authors.initials: G
authors.lastname: Sequeira
authors.affiliation: Hospital Divino Espírito Santo, Ponta Delgada, Portugal.
authors.forename: T
authors.initials: T
authors.lastname: Nóvoa
authors.affiliation: Instituto Português de Reumatologia, Lisbon, Portugal.
authors.forename: J A
authors.initials: JA
authors.lastname: Melo Gomes
authors.affiliation: Hospital Garcia de Orta, Almada, Portugal.
authors.forename: J Canas
authors.initials: JC
authors.lastname: da Silva
authors.affiliation: Centro Hospitalar de São João, Porto, Portugal.
authors.forename: L
authors.initials: L
authors.lastname: Costa
authors.affiliation: Hospital de Santa Maria, Lisbon, Portugal.
authors.forename: C
authors.initials: C
authors.lastname: Macieira
authors.affiliation: Instituto Português de Reumatologia, Lisbon, Portugal.
authors.forename: C
authors.initials: C
authors.lastname: Silva
authors.affiliation: Hospitais da Universidade de Coimbra, Coimbra, Portugal.
authors.forename: J A P
authors.initials: JA
authors.lastname: Silva
authors.affiliation: Hospital de Santa Maria, Lisbon, Portugal.|Rheumatology Research Unit, Instituto de Medicina Molecular, Lisbon, Portugal.
authors.forename: H
authors.initials: H
authors.lastname: Canhão
authors.affiliation: Hospital Garcia de Orta, Almada, Portugal.|Rheumatology Research Unit, Instituto de Medicina Molecular, Lisbon, Portugal.
authors.forename: M J
authors.initials: MJ
authors.lastname: Santos
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D002648:Child; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011174:Portugal / Q000453:epidemiology; D015995:Prevalence; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D012737:Sex Factors; D013997:Time Factors; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: Portugal; Register; Reuma.pt; Systemic lupus erythematosus