Background Systemic Lupus Erhytematosus (SLE) is a multiorgan disease in which pattern of evolution is linked to activity and damage. When the disease starts at an early age, all the outcomes might be influenced by this.
Objectives The main focus of this study is to describe the differences between juvenile and adult onset SLE in a Romanian cohort.
Methods 101 SLE patients were evaluated between March 2015 and December 2015. Patients were diagnosed with SLE according to ACR classification criteria and splited in 2 groups, taking into account age at disease onset: before and after 18. All patients agree to participate in this study. Data about demographic, clinical or serological characteristics, activity (SLEDAI) or damage (SLICC damage index SDI), treatment were collected. Statistical analisys was performed with SPSS 20.0.
Results Our cohort was made from 18 patients with juvenile onset SLE and 83 with adult onset SLE. Mean age at diagnosis was 14.05 versus 37.89. The juvenile onset SLE group had significant more autoimmune disease as family background, mainly SLE and rheumatoid arthritis (33.3% versus 12.04%, p 0.025), significant more severe organ involvement during the disease evolution: SLE related neurologic involvement 33.3% versus 12% (p 0.025) and renal involvement 50% versus 16.8% (p 0.023), more frequent anti dsDNA Ab positives 88.8% versus 60.24% (p 0.021). This more severe pattern of evolution is also reflected by immunosuppressant therapy, Cyclophosphamide being used in juvenile subgroup in 66% cases versus 35.3% in adults (p 0.014). Interesting, there were no differences between cumulative damage between groups, as measured by SDI (p 0.24), with the mention that in juvenile onset patients, SDI>1 was more frequent SLE related. This is in line with the fact that side effects of the corthicotherapie were identified more in adult onset SLE 61.44% versus 33.3% in juvenile onset (p 0.029).
Conclusions Our data clearly show that juvenile onset SLE has more genetic background and it is more prone to a severe disease, renal and neurologic involvement being more frequent than in adult onset form. Severity seems to be related both to the activity, but also to fast damage accrual over time. Controlling the disease activity must be our goal, since cumulative irreversible damage is mainly SLE related for our patients.
Watson, L., Leone, V., Pilkington, C., et all, on behalf of the UK Juvenile-Onset Systemic Lupus Erythematosus Study Group, “Disease activity, severity, and damage in the UK juvenile-onset systemic lupus erythematosus cohort”. Arthritis & Rheumatism, 2012, 64: 2356–2365. doi: 10.1002/art.34410.
Disclosure of Interest None declared