The Impact of Serum Anti-neutrophil Cytoplasmic Antibody on Clinical Characteristics and Outcomes in Pediatric-Onset Systemic Lupus Erythematosus Patients

Abstract

Background: Systemic lupus erythematosus (SLE), an autoimmune disease, is characterized by the overproduction of autoantibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) have been recognized in SLE for decades. To date, their association with SLE disease activity, especially in pediatric-onset SLE (pSLE) patients, is limited.Methods: We conducted a retrospective case-control study of pSLE patients with ANCAs from 2010 to 2020. Clinical characteristics, laboratory data, renal histological features, treatment and outcomes were analyzed.Results: A total of 70 pediatric-onset SLE patients (9 ANCA-positive vs. 61 ANCA-negative) with a median age of 12.23 years (age ranging from 4 years to 18 years) at diagnosis were enrolled. Among patients with ANCAs, MPO-ANCA was found in seven and PR3-ANCA in two of those cases. Patients with ANCAs had a tendency to have hematuria compared with those without ANCAs (66 vs. 24.6%, respectively; p = 0.026). Of the 70 SLE patients, 8 with ANCAs and 44 without ANCAs underwent renal biopsies. Patients with ANCAs (25%, 2/8) were more likely to lack the typical full-house pattern in their renal immunofluorescence (IF) staining.Conclusion: pSLE patients with ANCAs tend to have hematuria and an absence of typical IF histology. However, patients with and without ANCAs showed no difference in their clinical presentations and treatment outcomes.

Clinicopathologic features in childhood-onset lupus nephritis with antineutrophil cytoplasmic antibody positivity——a multi-center retrospective study

“…Hematuria was more prominent in the ANCA-positive LN group, which similar finding has been reported by both pediatric and adult cohorts. 21,22 Typical signs of SLE including fever and cutaneous presentation were less frequently displayed in ANCA-positive children, suggesting a notion should be noticed that lupus children with ANCAs may have atypical clinical presentation. Previous adult LN cohort has proposed that positive ANCA serology is associated with a more active lupus in terms of serology, 6,12 including higher anti-dsDNA antibody titers and lower serum complement concentrations.…”

“…Pauci-immune glomerulonephritis was not reported in another pediatric cohort or adult cohorts of LN with ANCA positivity either. 6,12,21 These inconsistent results emphasized the need of further prospective studies to clarify the relationship between ANCAs and the degree of renal immune deposition in context of LN.…”

“…To our knowledge, this is currently the largest pediatric LN cohort to analyze the impact of ANCA positivity on disease clinical and histopathologic characteristics. [19][20][21] Regardless of ANCA serology, LN children in this cohort had highly active diseases at their onset. However, ANCA-positive children presented with different renal and extrarenal manifestations.…”