Pediatric Rheumatology Online Journal

Pediatric Rheumatology Online Journal → July 2003 → Childhood Lupus→ Clinical Presentation, course and Outcomes → Abstract #97

SLICC/ACR-DI IN CHILDREN WITH JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS AND ITS RELATIONSHIP WITH DISEASE DURATION

S. K. F. Oliveira,¹ B. Scofono, A. F. Melo, V. C. Costa, M. E. C. Munhoz, T. D. Gava

¹Pediatric Rheumatology, IPPMG _ Federal University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil

Background: SLICC/ACR-DI (Systemic Lupus International Collaborating Clinics/American College of Rheumatology- Damage Index) is a validated tool to measure present damage in patients with SLE. It includes items to evaluate irreversible damage in organs and systems affected by the disease itself as well as by its therapy or intercurrent
diseases. Our aim was to evaluate the presence of damage in our children and adolescents with juvenile systemic lupus (JSLE).
Method: SLICC-DI was calculated in a cohort of JSLE patients at intervals of 0-2 years, 2-5 years and 5-10 years of the disease. Damage was defined as any score [ge> 1.
Results: We analyzed 63 patients (male=8, female=55). Average age at diagnosis was 9y 2mo (3y 2mo to 15y 4mo). The median time of follow-up was 6y9m (3y 1mo to 10 years) and the median time for a patient to have a positive SLICC was 2y9m. SLICC-DI [ge> 1 was present in 8 patients (average: 1.8) in the first 2 years of disease, in 15 patients (average: 1.8) until the 5th year of disease and in 21 patients (average: 1.5) until the 10^th year of disease. Later on, we added two more domains as indication of damage in children: delayed puberty and growth failure. Positive SLICC started to occur from 1 mo to 8y 7mo (average: 2y 9mo) in different domains: neurological, ocular, musculoskeletal, growth failure and puberty delayed. Among the domains that were responsible for a positive SLICC after 5 years of disease we found cardiomyopathy, pulmonary hypertension, pulmonary fibrosis and venous thrombosis.
Conclusion: We concluded that evaluation based on SLICC-DI is useful in children. Our data showed a low score in the 5 first years of the disease and the affected domains were different from the ones frequently reported in adults. We confirm that growth failure and delayed puberty are important domains to be included in the study of children and, in the future, we also suggest striae and obesity be included as damage.