title: Differences in Clinical Features and Mortality between Childhood-onset and Adult-onset Systemic Lupus Erythematosus: A Prospective Single-center Study.
abstract: To compare clinical features and mortality between childhood-onset systemic lupus erythematosus (cSLE) and adult-onset SLE (aSLE) in a prospective single-center cohort.
  
  
  
  A total of 1112 patients with SLE (133 cSLE and 979 aSLE) were enrolled and followed from 1998 to 2012. The 2 groups were compared regarding American College of Rheumatology (ACR) classification criteria for SLE, autoantibodies, disease activity measured by the Adjusted Mean SLE Disease Activity Index (AMS), damage measured by the Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI), and medication. The standardized mortality ratio (SMR) was calculated. Predictors of mortality in SLE were evaluated using Cox proportional hazard models.
  
  
  
  After a mean followup of 7.6 years, patients with cSLE had a higher number of cumulative ACR criteria and a higher AMS (p < 0.001 each), but there was no difference in SDI (p = 0.797). Immunosuppressants were used more frequently by patients with cSLE (p < 0.001). The SMR of cSLE was 18.8 (95% CI 8.6-35.6), significantly higher than that of aSLE (2.9, 95% CI 2.1-3.9). We found cSLE to be an independent predictor of mortality (HR 3.6, p = 0.008). Moreover, presence of hemolytic anemia (7.2, p = 0.034) and antiphospholipid antibody (aPL; 3.8, p = 0.041) increased the magnitude of risk of early mortality more in the patients with cSLE than in those with aSLE.
  
  
  
  The clinical course of cSLE as measured by number of clinical manifestations and disease activity is worse than that of aSLE. Also, cSLE patients with hemolytic anemia and aPL are at greater risk of death than patients with aSLE who have those features.
authors.affiliation: From the Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul; Department of Rheumatology, Myoungji Hospital, Goyang-si; Clinical Research Center for Rheumatoid Arthritis (CRCRA), Seoul, Republic of Korea.Y.B. Joo*, MD, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases; S.Y. Park*, MD, PhD, Department of Rheumatology, Myoungji Hospital; S. Won, PhD, CRCRA; S.C. Bae, MD, PhD, MPH, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, and CRCRA.
authors.forename: Young Bin
authors.initials: YB
authors.lastname: Joo
authors.affiliation: From the Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul; Department of Rheumatology, Myoungji Hospital, Goyang-si; Clinical Research Center for Rheumatoid Arthritis (CRCRA), Seoul, Republic of Korea.Y.B. Joo*, MD, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases; S.Y. Park*, MD, PhD, Department of Rheumatology, Myoungji Hospital; S. Won, PhD, CRCRA; S.C. Bae, MD, PhD, MPH, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, and CRCRA.
authors.forename: So-Yeon
authors.initials: SY
authors.lastname: Park
authors.affiliation: From the Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul; Department of Rheumatology, Myoungji Hospital, Goyang-si; Clinical Research Center for Rheumatoid Arthritis (CRCRA), Seoul, Republic of Korea.Y.B. Joo*, MD, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases; S.Y. Park*, MD, PhD, Department of Rheumatology, Myoungji Hospital; S. Won, PhD, CRCRA; S.C. Bae, MD, PhD, MPH, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, and CRCRA.
authors.forename: Soyoung
authors.initials: S
authors.lastname: Won
authors.affiliation: From the Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul; Department of Rheumatology, Myoungji Hospital, Goyang-si; Clinical Research Center for Rheumatoid Arthritis (CRCRA), Seoul, Republic of Korea.Y.B. Joo*, MD, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases; S.Y. Park*, MD, PhD, Department of Rheumatology, Myoungji Hospital; S. Won, PhD, CRCRA; S.C. Bae, MD, PhD, MPH, Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, and CRCRA. scbae@hanyang.ac.kr.
authors.forename: Sang-Cheol
authors.initials: SC
authors.lastname: Bae
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D063189:Symptom Assessment; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: CHILDHOOD ONSET; CLINICAL FEATURE; MORTALITY; SYSTEMIC LUPUS ERYTHEMATOSUS