Risk factors for damage in childhood-onset systemic lupus erythematosus in Asians: a case control study

Abstract

BackgroundAccumulated damage is an important prognostic factor in systemic lupus erythematous. However, the pattern of disease damage and its risk factors have not been well studied in childhood-onset systemic lupus erythematosus (cSLE) in Asia. The objectives are to evaluate the pattern of damage and to identify the risk factors for accumulated damage in an Asian group of cSLE.MethodsA retrospective chart review was conducted on a group of 59 patients with cSLE. Patient demographics and clinical variables were first collected at diagnosis. Over the course of their disease, clinical variables considered as risk factors for damage were also collected. Damage was measured using the Systemic Lupus International Collaborating Clinics/ American College of Rheumatology Damage Index (SDI) for each patient at their last encounter. Based on their SDI scores, patients were then dichotomized to two groups: a group with presence of disease damage (SDI ≥1) and a group with absence of disease damage (SDI score = 0). Clinical variables including age at diagnosis, gender, ethnicity, disease duration, disease manifestations, laboratory values at diagnosis, disease activity at diagnosis and last encounter, major organ involvement, number of lupus flares, major infection, and intensity of immunosuppressive medications were compared between the two groups. Growth failure and estimated glomerular filtration rate (eGFR) were also analysed as secondary outcomes.ResultsAfter a median disease duration and follow up of 7.8 years, 39 patients (66.1%) had no disease damage while 20 patients (33.9%) had acquired disease damage. Disease damage most frequently occurred in the ocular (15.3%), neuropsychiatric (11.9%) and musculoskeletal (11.9%) domains. The most frequent forms of damage were cataracts (11.9%), and avascular necrosis (unilateral and bilateral combined 10.2%). After controlling for other variables, presence of neuropsychiatric manifestations remained the only statistically significant risk factor for damage. The rate of growth failure in our group of patients was 16%. Patients who experienced growth failure were significantly younger at disease diagnosis. The median age of diagnosis was 10 for those who experienced growth failure, whereas the median age of diagnosis was 13 for those who did not experience growth failure. Despite a high rate of renal involvement in the group (79.7%), renal damage was only seen in 3.2% of the patients. 91.5% of the studied group had normal eGFR of ≥90 ml/min/1.73m2 at their last follow up.ConclusionThis group of patients had a low rate of damage accrual, with one of the lowest rates in renal damage when compared to other cohorts reported. The presence of neuropsychiatric manifestations was identified as the most significant risk factor for disease damage, while the most frequent forms of damage were cataracts and avascular necrosis, which were both related to prolonged steroid use. Despite the limitations of this study, it highlights the need for larger prospective studies to understand the relatio...

Differential Parameters between Activity Flare and Acute Infection in Pediatric Patients with Systemic Lupus Erythematosus

“…One study demonstrated that there is a positive correlation between SDI score and the number of recurrent major infections in pediatric-onset SLE [47]. Sit et al also reported that disease damage was signi cantly associated with a greater number of episodes of major infection [48]. These nding are in consistent with our study the associated between SDI with infection (P < 0.0001) by using multivariate GEE.…”

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

“…Our cohort exhibited a higher female to male ratio (7.3:1) in comparison with other childhood lupus cohorts with a typical female to male ratio of 5.6:1 and had a younger median age of diagnosis at 10.3 years with the majority (88.6%) having disease onset before the age of 12 years. 2,16,19,20 However, the age-related gender differences normally seen in other paediatric cohorts was similarly seen in our cohort with a female to male ratio of 2.3:1 in the under 5s and 15:1 in those with pubertal or post-pubertal onset. 3,20,21 Compared with other reported data, our male patients were not significantly younger at diagnosis.…”

Epidemiology and risk factors for avascular necrosis in childhood systemic lupus erythematosus in a Taiwanese population

“…To the best of our knowledge, this is the largest pediatric study to date on the association between SLE and AVN. Overall, 2.6% of our patients demonstrated evidence of symptomatic AVN, which is lower than in previous studies (ranging from 5.4 to 40%) [15][16][17][18][19][20][21] but similar to the 3.1% reported by Bogmat et al 22 Affected individuals can be asymptomatic in the early stages of disease, and are detected by magnetic resonance imaging. Therefore, the true prevalence rate in our cohort is likely to be higher as only symptomatic cases who sought medical care were identified.…”