Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity

Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the ‘Attikon’ cohort

“…1 In contrast, the prevalence of multiple autoantibodies is almost double in large cohort consisting of Hispanics, Afro-Americans and Asians. 2,25–27 Approximately 6% of our patients were ANA negative at the time of diagnosis, a finding identical to the multi-centre SLICC lupus cohort. 35…”

“…18,19 The incidence of distinct and relatively common neuropsychiatric manifestations, such as seizures, cerebrovascular events, neuropathies and psychosis, are also consistent with currently published large studies, 31–34 with more than half of primary neuropsychiatric manifestations occurring at the time of diagnosis. Notably, our data indicate that neuropsychiatric involvement is more frequent at the time of diagnosis in Caucasians compared to Asians and Hispanics, 25,26 while cSLE seems to have comparable prevalence of primary NPSLE at disease onset compared to our results 27 (Table 3). Thus, neuropsychiatric disease represents an increasingly recognized phenotype in lupus patients.…”

“…In this group, we included only lupus subjects with comprehensive medical records and adequate information from their medical history, reflecting the true course of the disease of each patient. The low incidence of some severe manifestations captured in our cohort, such as LN in 21.3%, may be attributed to the skewed distribution of disease duration, since the median disease duration in our cohort is Table 3 Comparison of clinical features of SLE patients at the time of diagnosis from large SLE cohorts around the world Items 'Attikon' cohort Mosca et al 3 Pons-Estel et al 25 Joo et al 26 Fiorot et al 27 only two years. Yet, lower rates of LN have also been reported in the 'Leto' lupus cohort in Crete.…”

“…A comparison of clinical manifestations at first presentation among our cohort and other cohorts around the world is summarized in Table 3. 3,[25][26][27] In Caucasian populations, musculoskeletal and skin involvement is common at disease onset, while the incidence of LN and positivity for anti-dsDNA or other lupus-specific autoantibodies are lower compared to non-Caucasian races. Neuropsychiatric disease represents an emerging phenotype among Caucasians.…”

Improving musculoskeletal health for children and young people – A ‘call to action’

“…Diagnosis can be challenging where infections (such as TB) are often mimics of rheumatic diseases and diagnostic capabilities (including availability of autoantibody tests) are often limited in low resource settings [ 5 , 95 ]. Delayed diagnosis and access to specialist care in JSLE with worse clinical outcomes is widely reported across the world [ 79 , 88 , [96] , [97] , [98] ], often associated with more severe disease activity and organ damage at presentation [ 75 , 99 , 100 ]. Many of the innovations that have improved survival in HRIC remain unavailable in most of the world [ 77 ] and access to renal replacement therapy may be severely limited or non-existent [ 101 ].…”