title: Disease activity patterns in juvenile systemic lupus erythematosus and its relation to early aggressive treatment.
abstract: This study aimed to determine disease activity patterns in juvenile systemic lupus erythematosus (jSLE) and its relation to early treatment. All jSLE patients who visited the outpatient departments of three Dutch university hospitals for at least 6 months were included. Data were retrospectively collected from each patient visit and hospitalization. Patient characteristics, clinical and laboratory findings categorized in organ systems, flare rate, medication use and disease course were analysed. Included were 35 patients (female 77%; White 47%) with a total follow-up of 142 years. Median age at diagnosis was 12.8 years. Flare rate was 0.45/ patient-year. An organ system not earlier involved was affected in 34% of flares. Identifiable disease activity patterns were: chronic active (49%), relapse remitting (14%) and long quiescence (37%), with no significant difference in organ involvement at diagnosis. Positive anti-Sm and non-White ethnicity were significantly associated with a chronic active pattern. In 14 patients with severe symptoms at diagnosis, treatment with intravenous cyclophosphamide and/or biologics and/or intravenous methylprednisone in the first 6 months resulted in a long quiescence pattern in seven patients. In conclusion, distinct disease activity patterns are identifiable in children. Suppression of disease with early aggressive treatment may decrease the rate of progression.
authors.affiliation: Department of Pediatrics/Pediatric Rheumatology, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands. m.otten@erasmusmc.nl
authors.forename: M H
authors.initials: MH
authors.lastname: Otten
authors.forename: K
authors.initials: K
authors.lastname: Cransberg
authors.forename: M A J
authors.initials: MA
authors.lastname: van Rossum
authors.forename: J W
authors.initials: JW
authors.lastname: Groothoff
authors.forename: J E
authors.initials: JE
authors.lastname: Kist-van Holthe
authors.forename: R
authors.initials: R
authors.lastname: Ten Cate
authors.forename: L W A
authors.initials: LW
authors.lastname: Van Suijlekom-Smit
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006785:Hospitals, University; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D008775:Methylprednisolone / Q000009:adverse effects / Q000627:therapeutic use; D009426:Netherlands; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors
chemical_list: D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone