2 research outputs found
Nailfold capillary abnormalities in childhood-onset systemic lupus erythematosus: a cross-sectional study compared with healthy controls
Objectives: For selection of high-risk systemic lupus erythematosus (SLE) patients it is necessary to obtain indicators of disease severity that predict disease damage. As in systemic sclerosis, nailfold capillary abnormalities could be such a biomarker in SLE. The primary objective of this cross-sectional study is to describe capillary abnormalities in childhood-onset SLE (cSLE) cohort (onset < 18 years) and compare them with matched healthy controls. The secondary objective is to correlate the observed capillary abnormalities with demographical variables in both cohorts and with disease-specific variables in cSLE patients. Methods: Healthy controls were matched for ethnic background, age and gender. Videocapillaroscopy was performed in eight fingers with 2-4 images per finger. Quantitative and qualitative assessments of nailfold capillaroscopy images were performed according to the definitions of the EULAR study group on microcirculation in Rheumatic Diseases. Results: Both groups (n = 41 cSLE-patients and n = 41 healthy controls) were comparable for ethnic background (p = 0.317). Counted per mm, cSLE-patients showed significantly more âgiantsâ (p = 0.032), âabnormal capillary shapesâ (p = 0.003), âlarge capillary hemorrhagesâ (p < 0.001) and âpericapillary extravasationsâ (p < 0.001). Combined âabnormal capillary shapes and pericapillary extravasationsâ (in the same finger) were detected in 78% (32/41 patients). By qualitative analysis, âmicroangiopathyâ was detected in 68.3% (28/41) and a âscleroderma patternâ in 17.1% (7/41) of the cSLE-patients (without scleroderma symptoms). The difference of percentage positive anti-RNP antibodies in the group with or without a scleroderma pattern was not significant (p = 0.089). The number of âabnormal capillary shapes per mmâ was significantly correlated with treatment-naivety. The number of âlarge pathological hemorrhages per mmâ was significantly correlated with SLEDAI score and presence of nephritis. Compared to healthy controls, âpericapillary extravasationsâ were found in significantly higher numbers per mm (p < 0.001) as well as in percentage of patients (p < 0.001). Conclusions: Our observations confirm that giants, abnormal capillary morphology and capillary hemorrhages are also observed in cSLE, as was already known for adults with SLE. Number of capillary hemorrhages in cSLE was significantly correlated with disease activity. A high frequency and total amount of âpericapillary extravasationsâ was observed in cSLE patients, possibly revealing a new subtype of capillary hemorrhage that might reflect endothelial damage in these pediatric patients
An amino acid motif in HLA-DRÎČ1 distinguishes patients with uveitis in juvenile idiopathic arthritis
Uveitis is a visually-debilitating disorder that affects up to 30% of children with the most common forms of juvenile idiopathic arthritis (JIA). The disease mechanisms predisposing only a subgroup of children to uveitis are unknown. To identify genetic susceptibility loci for uveitis in JIA, we conducted a genome-wide association study totalling 522 JIA cases.status: accepte