Features of 847 Childhood‐Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study

Abstract

Our large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.

Childhood-onset systemic lupus erythematosus: early disease manifestations that the paediatrician must know

“…Weight loss, photosensitivity, and leukopenia/lymphopenia were more frequently observed in adolescence at cSLE diagnosis. Early-onset cSLE patients were associated with complement (C1q, C4, and C2) deficiencies in this study [10]. Furthermore, C4 is encoded by C4A and C4B genes.…”

“…Constitutional signs and symptoms are the most first disease manifestations, such as fever, weight loss, fatigue, anorexia, and diffuse lymphadenopathy. Reticuloendothelial involvement at diagnosis with mild-to-moderate hepatomegaly (liver edge up to 7 cm below the right costal margin) and splenomegaly (spleen edge up to 7 cm below the left costal margin) was recently reported in 22% and 9% of 847 cSLE patients, respectively [10].…”

“…Mucocutaneous (60-85%), musculoskeletal (64-68%), hematologic (44-72%), nephritis (50-51%), serositis (28%), and neuropsychiatric (17%-25%) are the most common manifestations at cSLE onset [2,10,14].…”

“…The most common acute skin and oral lesions in cSLE patients are malar rash, photosensitivity, cutaneous vasculitis, mucosal ulcerations, and non-scaring alopecia [10,14]. Discoid lupus and lupus erythematosus panniculitis (or lupus erythematosus profundus) were rare forms of chronic cutaneous manifestations reported in 4% and 0.7% at cSLE onset, respectively [10,15]. A diagnosis of bullous cSLE should also be considered in patients with recurrent tense vesiculobullous lesions and usually not associated with classic SLE lesions [16].…”

What are the benefits of two-dimensional speckle tracking echocardiography for diagnosis and treatment follow-up of childhood-onset systemic lupus erythematosus myocarditis?

“…10 Treatment options for severe lupus manifestations, including myocarditis, FIGURE 2 Longitudinal and circumferential peak systolic strain. Prospective multicenter studies using 2DST myocardial deformation analysis should be conducted in order to establish the ideal moment to address specific treatment for lupus myocarditis, given that myocardial compromise contributes considerably for morbidity and mortality among c-SLE patients.…”