A Brazilian single-centre series comparision of childhood, adult and late onset Systemic lupus Erythematosus

Abstract

BackgroundSystemic Lupus Erythematosus (SLE) is a heterogeneous multisystem autoimmune disease, with variable severity, autoantibody profile, response to treatment, relapsing course and damage accrual. The age at disease onset may influence disease trajectory and prognosis, with remarkable differences of major organ involvement, disease activity, and prognosis. SLE clinical profile, activity indices, remission, and damage comparison were carried out in childhood-onset (cSLE), adult-onset (aSLE) and late-onset (lSLE) patients from a single-centre series.MethodsA cross-sectional analysis reviewing the clinical profile of SLE cases seen between 2012 and 2022 and classified in 3 age-groups according to disease onset: cSLE (<18 years), aSLE (18 to 49 years) and lSLE (>=50 years), was performed. Disease activity status was assessed by PGA (0-3), SLEDAI-2K, SLE-DAS, LLDAS and DORIS criteria, and damage using SDI at the same visit were compared in those 3 age-groups.ResultsFour hundred and fifteen patients were included in the analysis: 289 (68%) aSLE, 79 (19%) cSLE, and 47 (11.3%) lSLE. Most common clinical manifestations were articular (75.2%), hematological (70.1%), cutaneous (67.9%), photosensitivity (59.3%), and renal (41.7%). The clinical profile was similar among the subgroups of SLE patients, except for lSLE with lower frequency of nephritis and serositis, lower frequency of autoantibodies and hypocomplementenemia, lower SLEDAI-2K and SLE-DAS scores, and a higher frequency of LLDAS and DORIS remission; and a higher damage scores (SDI). Patients with cSLE had a significantly higher frequency of renal and neurological involvement, and a longer disease duration, but the disease activity, damage scores and remission rates were comparable to adult-onset group.ConclusionThe remarkable differences among the 3-age-groups were higher frequency of renal and neuropsychiatric domains in youngsters and disease activity scores compared to other age-groups. The lSLE group had less nephritis and serositis, lower disease activity and higher damage scores.