Background Children and adolescents with juvenile-onset systemic lupus erythematosus (jSLE) face considerable morbidity due to sequelae of disease activity, comorbidities and adverse effects of treatment. In order to improve the management of these patients, it is essential to better understand the risk factors associated with the development of permanent damage. Objectives The main goals of this study were to determine the prevalence of organ damage among children with jSLE and to identify risk factors for damage. Methods This was a retrospective chart review of the clinical courses of jSLE patients followed at MSCHONY/CUMC, from the time of diagnosis or first encounter until December 2009 or loss to follow-up. All patients were diagnosed before the age of 18, had a disease duration ≥6 months and were followed during the period 2007-2009. Disease activity (SLEDAI) at diagnosis and Systemic Lupus International Collaborating Clinics (SLICC) Damage Index (SDI) at the conclusion of the study were evaluated. Major infections were identified, defined as those that required treatment with parenteral antimicrobial agents or necessitated a course of antibiotics for more than one week. Chi-square/Fisher’s exact test and t-test were used to analyze categorical and continuous data, respectively. Results There were 120 children and adolescents with SLE: 93 female/27 male (F/M: 3.4/1), 61 Hispanic (51%) and 34 African-American (28%). The majority (53%) were diagnosed between the ages of 10-15 years. Mean duration of follow-up was 4.7±3.2 years. Mean SLEDAI at diagnosis was 9.6±6.5. Lupus nephritis affected 59 patients (49%). Neuropsychiatric manifestations (NPSLE) occurred in 15 children (12%). Mycophenolate mofetil (MMF) was used in 56% of the patients, cyclophosphamide in 37% and rituximab in 6%. The mean cumulative prednisone dose was 12g. There were 101 major infections affecting 44 patients (37%), the most common being skin infections (19), pneumonias (17), urinary tract infections with fever (14), sepsis (9), and herpes zoster (8). During the study 2 patients died due to disease-associated comorbidities. Damage (SDI ≥1) was present in 33% (39/118) of the patients after a mean disease duration of 5.0±3.3 years. The median SDI score was 1 (range 0-6). Damage to the musculoskeletal system was observed most frequently (9%), followed by neuropsychiatric (7%), ocular (7%), skin (7%), pulmonary (5%) and renal (5%) systems. No malignancies were encountered. Damage was significantly more likely in patients who had experienced neuropsychiatric manifestations during the first year of disease, had major infections or were treated with cyclophosphamide, MMF or rituximab (p<0.05). There was no association of damage with gender, renal involvement or cumulative dose of prednisone. Conclusions In a large cohort of children with jSLE, damage was common, but less frequent than previously reported. Neuropsychiatric involvement during the first year of disease, major infections and immunosuppressive therapy with cyclophosphamide, MMF and rituximab were identified in this cohort as risk factors for damage. References Gutiérrez-Suárez R et al. Arthritis Rheum 2006. 54:2989-96. Ravelli A et al. Arthritis Rheum 2003. 49:501-7. Disclosure of Interest None Declared

Results There were 120 children with SLE followed during the study period: 93 female (77.5%, M:F 3.4:1); 61 Hispanic (50.8%) and 34 African-American (28.3%). NPSLE affected 15 children (12.5%). Cognitive dysfunction was the most common manifestation, followed by significant headache, seizures, psychosis, and focal neurologic signs. NPSLE was diagnosed in the first year in 10/15 patients (67%). Abnormalities of brain magnetic resonance imaging were encountered in 10 patients (67%). Children with NPSLE had a significantly greater number of co-morbidities (end-stage renal disease, thrombocytopenia, and pancreatitis) than unaffected children with SLE (p<0.05). NPSLE was associated with a higher SLEDAI at diagnosis and the presence of anti-Smith antibodies (p<0.05). All patients received high-dose corticosteroid and cyclophosphamide. NPSLE was associated with the occurrence of major infections, as well as a higher SLICC damage index, and mortality (p<0.05). Conclusions In this cohort NPSLE was associated with co-morbidities, major infections, and higher damage scores.

Case report A 3 year old boy presented with 3 days of fever, rash, pharyngeal and gingival erythema, and swollen extremities. Laboratory investigations revealed leukocytosis, C– reactive protein 25.8 mg/dl, and erythrocyte sedimentation rate 100 mm/hr. Echocardiography disclosed diffuse dilatation of all proximal coronary arteries. The child received IVIG (2g/kg) and aspirin (100 mg/kg/d) with no response. IVIG was repeated, followed by methylprednisolone 30 mg/kg for 3 days, but the child remained febrile. Infliximab (5 mg/kg) was thereupon employed with prompt defervescence. Low-dose aspirin was continued, as well as clopidogrel. Echocardiographic findings remained stable. Six months after the initial episode, the child again presented with fever, irritability, sore throat and nuchal rigidity. Physical examination revealed cracked, swollen lips, oropharyngeal erythema, posterior cervical lymphadenopathy, and rash. Desquamation of the distal extremities was observed some days later. Aneurysms were detected, involving the left and right main coronary arteries, as well as the left anterior descending coronary. Magnetic resonance angiography of the chest and abdomen revealed no other involved vessels. The child again received IVIG, pulse methylprednisolone, and infliximab, but remained febrile and developed significant arthritis, requiring daily prednisolone. He is now asymptomatic.