Urticarial vasculitis in pediatric systemic lupus erythematosus

Pediatr Dermatol. 2020 Jul;37(4):651-655. doi: 10.1111/pde.14184. Epub 2020 May 5.

Abstract

Background/objectives: To determine the prevalence of urticarial vasculitis (UV) in a pediatric systemic lupus erythematosus (pSLE) cohort of Arab ethnicity from Oman and to describe their demographic, clinical, and laboratory features.

Methods: We conducted a retrospective multicenter study among pediatric rheumatology centers in Oman over a 10-year period from 2008 to 2018. Analyses were performed using univariate statistics.

Results: A total of 148 pSLE under the age of 13 years were included of which 30% (n = 44) were men. The overall mean age at SLE diagnosis was 7.6 ± 3.5 years while the mean disease duration was 10.1 ± 5.4 years. UV was diagnosed in 36% (n = 53) of pSLE patients. Patients with pSLE and UV were more likely to be male (57% vs 15%; P < .001), diagnosed at a younger age (5.9 vs 8.5 years; P < .001), have a family history of SLE (53% vs 36%; P = .044), and have associated conjunctivitis, (32% vs 5.3%; P < .001) but less likely to have CNS involvement (7.6% vs 20%; P = .045) or hematological manifestations such as leukopenia (9.4% vs 24%; P = .028) and thrombocytopenia (5.7% vs 18%; P = .045). This subgroup was also more likely to be associated with low C3 complement count (94% vs 66%; P < .001) and positive cytoplasmic ANCA (11% vs 0%; P = .022).

Conclusion: We report a high occurrence of UV in a pSLE cohort associated with unique demographic, clinical, and laboratory features.

Keywords: Arab; Oman; children; epidemiology; systemic lupus erythematosus; vasculitis.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Child
  • Female
  • Humans
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Lupus Erythematosus, Systemic* / epidemiology
  • Male
  • Retrospective Studies
  • Severity of Illness Index
  • Urticaria* / diagnosis
  • Urticaria* / epidemiology
  • Urticaria* / etiology
  • Vasculitis* / diagnosis
  • Vasculitis* / epidemiology