Abstract
ObjectivesSignificant differences have been reported in disease phenotype and severity of systemic lupus erythematosus (SLE) presenting in different age groups. Most indicate a more severe phenotype in juvenile-onset SLE (JSLE). There have been limited studies in older patients and no large studies looking at SLE across all age groups.MethodsWe assessed the effect of age of onset of SLE on the clinical phenotype by analysing data from two large UK cohorts (the UK JSLE Cohort and the UCLH SLE cohort).ResultsA total of 924 individuals were compared (413 JSLE, 511 adult-onset SLE). A female preponderance was present, but less pronounced at either end of the age spectrum. Arthritis was more common with advancing age (93% vs 72%, p < 0.001), whereas renal disease (44% vs 33%, p = 0.001), alopecia (47% vs 23%, p < 0.001) and aphthous ulcerations (39% vs 26%, p = 0.001) were more common in the young. Neuropsychiatric lupus was less common in mature-onset SLE (p < 0.01). JSLE was associated more commonly with thrombocytopenia (21% vs 15%, p = 0.01), haemolytic anaemia (20% vs 3%, p < 0.001), high anti-dsDNA (71% vs 63%, p = 0.009), Sm (22% vs 16%, p = 0.02) and RNP (36% vs 29%, p < 0.04) auto-antibodies. Leucopenia increased with advancing age (p < 0.001). Mortality has been declining over recent decades. However, death rates were substantially higher than the general population. The standardized mortality ratio was 18.3 in JSLE and 3.1 in adult-onset SLE.ConclusionThese data from the largest-ever direct comparison of JSLE with adult-onset SLE suggest an aggressive phenotype of disease with a worse outcome in patients with JSLE and emphasizes the importance of careful follow-up in this population.
Highlights
Systemic lupus erythematosus (SLE) is a severe, complex, multi-system autoimmune rheumatic disease
In this report we describe the effect of age at onset of SLE on the phenotypic manifestations by assessing two large cohorts: the United Kingdom (UK) juvenile-onset SLE (JSLE) Cohort Study and the University College London Hospital (UCLH) cohort
A total of 924 individuals diagnosed with SLE and meeting 4 American College of Rheumatology (ACR) criteria were included once duplicates had been removed
Summary
Systemic lupus erythematosus (SLE) is a severe, complex, multi-system autoimmune rheumatic disease. It is most prevalent among women of childbearing age.[1,2,3] Approximately 20% of cases begin during childhood, usually after puberty,[4,5,6] and published data suggest a further 10–20% present after the age of 50 years.[7,8,9]. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav reported by several groups including our own,[10] with most reports finding a severe phenotype with a greater burden of steroids and immunosuppressive treatment.[10,11,12,13,14,15] previous comparison studies generally assessed fewer than 100 JSLE cases. Few, if any, studies have looked at SLE across the broader age groups within the same study
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