Impact of autoimmune cytopenias on severity of childhood-onset systemic lupus erythematosus: A single-center retrospective cohort study

Abstract

Objective To assess whether children with autoimmune cytopenias prior to or at diagnosis of systemic lupus erythematosus (cSLE), differ phenotypically from other cSLE patients; and have a lower risk and severity of lupus nephritis (LN) as observed in prior adult studies. To assess the effect of prior immune therapy for autoimmune cytopenias on 2-year risk of LN. Methods This was a retrospective cohort study of incident cSLE cases. We included patients aged less than 17 years at diagnosis. We excluded patients with LN at cSLE diagnosis. Our follow-up period was 2 years. We defined autoimmune cytopenias as either autoimmune hemolytic anemia, immune thrombocytopenia or Evan’s syndrome. Results Forty-three (33%) of the 130 patients had autoimmune cytopenias before or at cSLE diagnosis. Those with autoimmune cytopenias had significantly more neuropsychiatric symptoms and higher mean ESR but less arthritis, malar rash and myositis versus those without autoimmune cytopenias. They had lower 2-year incidence proportion of LN compared to other cSLE patients (7% vs 15%). Of the 16 patients who developed LN, those with autoimmune cytopenias had mostly class V (2 of 3 patients) versus mostly class III and IV in those without autoimmune cytopenias (6 of 12 patients). None of the 13 patients pre-treated for autoimmune cytopenias developed LN. Conclusion Patients with autoimmune cytopenias before or at cSLE diagnosis have intriguing differences from other cSLE patients. They may represent a unique sub-type of cSLE patients and should be further explored.

Three Clinical Clusters Identified through Hierarchical Cluster Analysis Using Initial Laboratory Findings in Korean Patients with Systemic Lupus Erythematosus

“…SLE patients with lymphopenia have reduced surface expression of complement regulatory proteins and endogenous production of type 1 interferon [34]. An analysis of autoimmune cytopenia before or at childhood-onset SLE showed that patients with autoimmune cytopenia had a lower incidence of arthritis and a lower 2-year incidence of LN than those without autoimmune cytopenia [35]. Herein, cytopenia is not a typical finding of a particular cluster and might occur through the different etiologies of arthritis and renal involvement in SLE.…”

Early add-on use of belimumab as induction therapy for patients with childhood-onset systemic lupus erythematosus

“…[1][2][3][4] However, little data is available regarding the implications of the use of belimumab in a "real-world" setting for the treatment of childhood-onset SLE (cSLE). 6,7 In this context, we read with great interest a paper by Roberts et al 7 in this journal. For the past 5 years, we have also used belimumab add-on treatment in nine patients with cSLE.…”

Updates in the care and management of children and adolescents with systemic lupus erythematosus

“…Real-world use and outcomes of biologics were recently reported in cSLE. A case series of 21 cSLE patients under belimumab ≥12 months showed that the median dose of prednisone was significantly reduced throughout 1 year (12.5 mg/day at baseline vs. 9 at 6 months vs. 5 at 12 months), however no difference was evidenced in SLEDAI-2K scores [47]. A multivariate analysis of a prospective multicenter Chinese study with 193 active cSLE under belimumab treatment showed that SELENA-SLEDAI score <10 at baseline and low glucocorticoid dose predicted achievement of LLDAS, and a low glucocorticoid dose also predicted achievement of clinical remission [48].…”