Disease duration, hypertension and medication requirements are associated with organ damage in childhood-onset systemic lupus erythematosus.
OBJECTIVE
To investigate the frequency of organ damage in childhood-onset systemic lupus erythematosus (SLE) and to identify disease variables and patient characteristics related to organ damage.


METHODS
A cohort of 71 patients was examined in a cross-sectional study after a mean disease duration of 10.8+/-8.2 years (mean age 26.4+/-9.8 years). The occurrence of organ damage was measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Factors analysed as possible explanatory variables of organ damage were the following: demographic variables, clinical variables at diagnosis and during disease course, as well as medication use. Growth and self-reported health status were also measured.


RESULTS
The most frequent areas of organ damage were in the neuropsychiatric (28%), renal (13%) and musculoskeletal (13%) organ systems. Forty-three patients (61%) had evidence of damage. The mean SDI score was 1.3 for the whole study population. Hypertension, longer disease duration and use of cyclophosphamide were factors significantly related to an increasing SDI score in multiple linear regression analyses. Furthermore, patients with damage (SDI > or =1) compared to those without damage (SDI = 0) had a significantly higher cumulative corticosteroid dose (24.7 g versus 10.6 g) and more frequently required high-dose prednisolone at diagnosis (68% versus 43%).


CONCLUSIONS
Evidence of organ damage was found in 61% of all patients. Long disease duration, known hypertension and use of cylophosphamide were significantly associated with an increasing SDI score. Furthermore high-dose prednisolone at diagnosis and cumulative prednisolone dose were significantly related to the presence of organ damage.
Lilleby
V
V
Department of Rheumatology, Rikshospitalet University Hospital, Oslo, Norway. vibke.lilleby@riskshospitalet.no
Flatø
B
B
Førre
O
O
D000293:Adolescent; D017668:Age of Onset; D003430:Cross-Sectional Studies; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006304:Health Status; D006801:Humans; D006973:Hypertension / Q000209:etiology / Q000473:pathology*; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008181:Lupus Nephritis / Q000453:epidemiology / Q000209:etiology / Q000473:pathology*; D020945:Lupus Vasculitis, Central Nervous System / Q000453:epidemiology / Q000209:etiology / Q000473:pathology*; D008297:Male; D009140:Musculoskeletal Diseases / Q000453:epidemiology / Q000209:etiology / Q000473:pathology*; D009664:Norway / Q000453:epidemiology; D012720:Severity of Illness Index; D013997:Time Factors
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide