Clinical manifestations, immunological features and prognosis of Chinese pediatric systemic lupus erythematosus: A single-center study

Int J Rheum Dis. 2019 Jun;22(6):1070-1076. doi: 10.1111/1756-185X.13547. Epub 2019 Apr 8.

Abstract

Aim: Since there are only a few reports on pediatric systemic lupus erythematosus (pSLE) in Chinese populations, therefore we retrospectively report the clinical and immunological features as well as renal outcome in Chinese pSLE.

Methods: Patients diagnosed with pSLE at Shanghai Children's Medical Center between 2001 and 2016 were evaluated and clinical data were retrospectively collected.

Results: A total of 102 pSLE patients were analyzed. Renal disorder including proteinuria (81.37%) and hematuria (65.69%) were most commonly identified. Class IV was the most common finding on renal biopsy. In lupus nephritis (LN), 67.21%, 78.0%, 86.0% and 94.55% achieved complete remission within 6, 12, 18 and 24 months, respectively. Furthermore, 16.67% of LN patients suffered at least one renal flare. Antinuclear antibodies were detected in nearly all patients (97.62%), followed by anti-double-stranded DNA (anti-dsDNA) antibodies (70.0%) and anti-Sjögren's syndrome A (anti-SSA) antibodies (60.64%). Oral corticosteroid (93.14%) and mycophenolate mofetil (64.71%) was used in the majority of patients. Infection (32.35%) was the main side effect caused by the medications.

Conclusions: Our population-based pSLE cohort indicated that compared to other international cohorts, there was a higher prevalence of LN in Chinese pSLE. Proteinuria was the most frequent manifestation both at disease onset and during the entire clinical course. Class IV LN was the dominant renal pathological type. Nevertheless, there was a favorable renal remission rate and relatively low incidence of renal flare in our cohort. Apart from antinuclear antibodies and anti-dsDNA antibodies, anti-SSA antibodies were most frequently detected. Infection was the leading complication caused by the medications.

Keywords: infection; lupus nephritis; pediatric systemic lupus erythematosus; renal outcome.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / administration & dosage
  • Adrenal Cortex Hormones / adverse effects
  • Age of Onset
  • Antibodies, Antinuclear / blood*
  • Biomarkers / blood
  • Child
  • China / epidemiology
  • Female
  • Hematuria / diagnosis
  • Humans
  • Immunocompromised Host
  • Incidence
  • Lupus Erythematosus, Systemic / diagnosis*
  • Lupus Erythematosus, Systemic / drug therapy
  • Lupus Erythematosus, Systemic / epidemiology
  • Lupus Erythematosus, Systemic / immunology
  • Lupus Nephritis / diagnosis*
  • Lupus Nephritis / drug therapy
  • Lupus Nephritis / epidemiology
  • Lupus Nephritis / immunology
  • Male
  • Mycophenolic Acid / administration & dosage
  • Mycophenolic Acid / adverse effects
  • Opportunistic Infections / epidemiology
  • Opportunistic Infections / immunology
  • Prevalence
  • Proteinuria / diagnosis
  • Remission Induction
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Antibodies, Antinuclear
  • Biomarkers
  • SS-A antibodies
  • Mycophenolic Acid