Dissecting the damage in Northern Greek patients with childhood-onset systemic lupus erythematosus: a retrospective cohort study
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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.
KeywordsChildhood 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.
- 1.Ravelli A, Duarte-Salazar C, Buratti S, Reiff A, Bernstein B, Maldonado-Velazquez MR, Beristain-Manterola R, Maeno N, Takei S, Gerloni V, Spencer CH, Pratsidou-Gertsi P, Ruperto N, Pistorio A, Martini A (2003) Assessment of damage in juvenile-onset systemic lupus erythematosus: a multicenter cohort study. Arthritis Rheum 49:501–507CrossRefPubMedGoogle Scholar
- 5.Gladman D, Ginzler E, Goldsmith C, Fortin P, Liang M, Urowitz M, Bacon P, Bombardieri S, Hanly J, Hay E, Isenberg D, Jones J, Kalunian K, Maddison P, Nived O, Petri M, Richter M, Sanchez-Guerrero J, Snaith M, Sturfelt G, Symmons D, Zoma A (1996) The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum 39:363–369CrossRefPubMedGoogle Scholar
- 10.Bandeira M, Buratti S, Bartoli M, Gasparini C, Breda L, Pistorio A, Grassi S, Alpigiani MG, Barbano G, Janz-Junior LL, Martini A, Ravelli A (2006) Relationship between damage accrual, disease flares and cumulative drug therapies in juvenile-onset systemic lupus erythematosus. Lupus 15:515–520CrossRefPubMedGoogle Scholar
- 13.Gutiérrez-Suárez R, Ruperto N, Gastaldi R, Pistorio A, Felici E, Burgos-Vargas R, Martini A, Ravelli A (2006) A proposal for a pediatric version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index based on the analysis of 1,015 patients with juvenile-onset systemic lupus erythematosus. Arthritis Rheum 54:2989–2996CrossRefPubMedGoogle Scholar
- 16.Uziel Y, Gorodnitski N, Mukamel M, Padeh S, Brik R, Barash J, Mevorach D, Berkun Y, Tauber T, Press J, Harel L, Navon P, Rubenstein M, Naparstek Y, Hashkes PJ, Pediatric Rheumatology Study Group Of Israel @ SLERI (2007) Outcome of a national Israeli cohort of pediatric systemic lupus erythematosus. Lupus 16:142–146CrossRefPubMedGoogle Scholar
- 18.Taddio A, Rossetto E, Rosé CD, Brescia AM, Bracaglia C, Cortis E, Rigante D, Stabile A, Montico M, Ronfani L, Ventura A, Lepore L (2010) Prognostic impact of atypical presentation in pediatric systemic lupus erythematosus: results from a multicenter study. J Pediatr 156:972–977CrossRefPubMedGoogle Scholar
- 20.Watson L, Leone V, Pilkington C, Tullus K, Rangaraj S, McDonagh JE, Gardner-Medwin J, Wilkinson N, Riley P, Tizard J, Armon K, Sinha MD, Ioannou Y, Archer N, Bailey K, Davidson J, Baildam EM, Cleary G, McCann LJ, Beresford MW, UK Juvenile-Onset Systemic Lupus Erythematosus Study Group (2012) Disease activity, severity, and damage in the UK Juvenile-Onset Systemic Lupus Erythematosus Cohort. Arthritis Rheum 64:2356–2365CrossRefPubMedGoogle Scholar
- 25.Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G, Ramsey-Goldman R, Bae SC, Hanly JG, Sánchez-Guerrero J, Clarke A, Aranow C, Manzi S, Urowitz M, Gladman D, Kalunian K, Costner M, Werth VP, Zoma A, Bernatsky S, Ruiz-Irastorza G, Khamashta MA, Jacobsen S, Buyon JP, Maddison P, Dooley MA, van Vollenhoven RF, Ginzler E, Stoll T, Peschken C, Jorizzo JL, Callen JP, Lim SS, Fessler BJ, Inanc M, Kamen DL, Rahman A, Steinsson K, Franks AG Jr, Sigler L, Hameed S, Fang H, Pham N, Brey R, Weisman MH, McGwin G Jr, Magder LS (2012) Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 64:2677–2686CrossRefPubMedCentralPubMedGoogle Scholar
- 27.Vitali C, Bencivelli W, Isenberg DA, Smolen JS, Snaith ML, Sciuto M, Neri R, Bombardieri S (1992) Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. II. Identification of the variables indicative of disease activity and their use in the development of an activity score. The European Consensus Study Group for Disease Activity in SLE. Clin Exp Rheumatol 10:541–547PubMedGoogle Scholar
- 32.Pons-Estel GJ, Alarcón GS, McGwin G Jr, Danila MI, Zhang J, Bastian HM, Reveille JD, Vilá LM, Lumina Study Group (2009) Protective effect of hydroxychloroquine on renal damage in patients with lupus nephritis: LXV, data from a multiethnic US cohort. Arthritis Rheum 61:830–839CrossRefPubMedCentralPubMedGoogle Scholar
- 33.Rygg M, Pistorio A, Ravelli A, Maghnie M, Di Iorgi N, Bader-Meunier B, Da Silva C, Roldan-Molina R, Barash J, Dracou C, Laloum SG, Jarosova K, Deslandre CJ, Koné-Paut I, Garofalo F, Press J, Sengler C, Tauber T, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (PRINTO) (2012) A longitudinal PRINTO study on growth and puberty in juvenile systemic lupus erythematosus. Ann Rheum Dis 71:511–517CrossRefPubMedGoogle Scholar
- 34.Andrianakos A, Trontzas P, Christoyannis F, Dantis P, Voudouris C, Georgountzos A, Kaziolas G, Vafiadou E, Pantelidou K, Karamitsos D, Kontelis L, Krachtis P, Nikolia Z, Kaskani E, Tavaniotou E, Antoniades C, Karanikolas G, Kontoyanni A, ESORDIG Study (2003) Prevalence of rheumatic diseases in Greece: a cross-sectional population based epidemiological study. J Rheumatol 30:1589–1601PubMedGoogle Scholar