Rheumatology International

, Volume 35, Issue 7, pp 1225–1232 | Cite as

Dissecting the damage in Northern Greek patients with childhood-onset systemic lupus erythematosus: a retrospective cohort study

  • Artemis KoutsonikoliEmail author
  • Maria Trachana
  • Anna-Bettina Heidich
  • Vasiliki Galanopoulou
  • Polyxeni Pratsidou-Gertsi
  • Alexandros Garyphallos
Original Article - Observational Research


The improved survival of childhood-onset systemic lupus erythematosus (cSLE) has resulted in longer patients’ exposure to disease inflammation, medications and/or comorbid diseases, which can all contribute to the development of organ damage. The aim of this study was to assess the evolution of damage accrual in cSLE patients overtime and investigate for predisposing factors. Disease characteristics and treatment in 47 Northern Greek Caucasian cSLE patients were retrospectively reviewed. The Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index (SDI) was used for damage assessment and the European Consensus Lupus Activity Measurement (ECLAM) to monitor cSLE activity. After a median disease duration of 7.4 years, 17/47 patients (36 %) had developed damage (SDI > 0). The most frequent domains damaged were the ocular (41 %), neuropsychiatric (35 %) and peripheral vascular (35 %) one. Peripheral vascular and neuropsychiatric damage was seen more frequently during the first 5 years of the disease. Longer exposure to azathioprine was associated with higher SDI at the end of follow-up (β = 0.008 for every additional month of use, p = 0.041). The mean annual flare frequency was associated with a shorter time interval until the development of the first damage (hazard’s ratio, HR 2.38 for each unit of increase, p = 0.018), while hydroxychloroquine use was associated with longer time interval (HR 0.19, p = 0.007). The lower rates of damage accrual in this study compared to other cohorts might be due to milder disease phenotype in Greek Caucasian cSLE patients, prompt diagnosis and effective disease control. Damage was noticed early in the disease course, and one-third of patients had an SDI > 0 at study completion. Disease flares and a severe disease course leading to prolonged use of immunosuppressives were significant risk factors, while hydroxychloroquine use was protective against cSLE damage accrual.


Childhood Lupus Damage SDI 



The authors wish to thank Dr G. Bertsias for his critical review of the manuscript.

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Artemis Koutsonikoli
    • 1
    Email author
  • Maria Trachana
    • 1
  • Anna-Bettina Heidich
    • 2
  • Vasiliki Galanopoulou
    • 3
  • Polyxeni Pratsidou-Gertsi
    • 1
  • Alexandros Garyphallos
    • 4
  1. 1.1st Department of Pediatrics, Pediatric Immunology and Rheumatology Referral Center, Hippokration HospitalAristotle UniversityThessalonikiGreece
  2. 2.Department of Hygiene and EpidemiologyAristotle UniversityThessalonikiGreece
  3. 3.Rheumatology DepartmentPapageorgiou HospitalThessalonikiGreece
  4. 4.4th Department of Internal Medicine, Hippokration HospitalAristotle UniversityThessalonikiGreece

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