Pediatric rheumatology

Phenotype and disease course differences in monogenic and sporadic childhood lupus

LupusPublished Cross-sectional study

Authors' conclusion

Patients with monogenic lupus are likely to have an early disease onset and a strong family history of lupus, as well as a guarded prognosis, which is likely due to the disease's severity and frequent infections.

Study context

Population
Children with lupus seen at a single tertiary childhood lupus clinic between June 1997 and July 2022
Participants
54 children
Exposure
Monogenic lupus vs sporadic childhood lupus
  • Monogenic lupusn = 27
  • Sporadic childhood lupus (SC-lupus)n = 27reference
Outcome
Age at disease onset (years), monogenic vs sporadic childhood lupus
Methodological detailsdescriptive, observational, retrospective data collection, single tertiary centre, systematic sampling of SC-lupus
  • Descriptive
  • Observational
  • Retrospective data collection
  • Single tertiary centre
  • Systematic sampling of SC-lupus

Main finding

As reported in the abstract

Monogenic lupus

3.5

median · IQR 1.0–6.0

SC-lupus

9.5

median · IQR 7.0–11.8

P < 0.05

Observational data: associations, not effects.

Original publication

Phenotype and disease course differences in monogenic and sporadic childhood lupus. Lupus. Published 25 October 2023. doi:10.1177/09612033231211065

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