Background/Purpose:  Systemic lupus erythematosus (SLE) is a persistent or remitting-relapsing autoimmune disease that handled in the context of connective tissue diseases and also vasculitides. Although there are commonly used biomarkers of active kidney disease and central nervous system disease, there is not a specific marker for vasculitic involvement. Pentraxin-3 (PTX3) is derived primarily from vascular endothelium and innate immunity cells in response to local inflammation and plays an important role locally at the site of inflammation. Thus PTX3 seems to be a useful biomarker directly reflecting local inflammation and local vasculitis. To the best of our knowledge PTX-3 was not studied in children with SLE. In the present study, we aimed to compare the concentrations of plasma PTX-3 among childhood-onset SLE (c-SLE) patients and control groups and to assess the association of PTX-3 levels with SLEDAI-2K, clinical manifestations and laboratory results.

Methods: From October 2015 to May 2016, 76 c-SLE patients without active infection sign and symptom were eligible for this cross-sectional single center study. We have also measured pentraxin-3 level in 41 healthy and age-matched controls. Both the cumulative and current organ involvement and manifestations were recorded from patient records and from the last examination at that moment, respectively. All of the laboratory analyses were studied concurrently with PTX-3 levels. PedSDI and SLEDAI-2K scores at disease onset, at the most severe flare and at the last examination were calculated. Serum pentraxin-3 levels were measured by a commercially available enzyme-linked immunosorbent assay kit.

Results: Plasma PTX3 concentrations were measured in 76 patients with c-SLE and 41 control subjects. Plasma PTX3 concentration of the SLE patients was significantly higher than that of the healthy controls (mean 10.6 ± 8.2 vs. 2.7 ± 1.3 ng/mL, p<0.001). The ratio of females to males with c-SLE was 5.3:1. The mean SLEDAI scores decreased from 10.3±4.8 (at disease onset) to 5.2± 5.3 (at last examination). Additionally, only 10.5% (n=8) and 3.9% (n=3) of the cohort were displaying the signs of active nephritis and active neuropsychiatric disease at last examination. In patients with SLE, PTX3 concentrations were correlated with SLEDAI-2K (p<0.001), active vasculitis (p<0.001), Raynaud’s phenomenon (p = 0.006) and active mucocutaneous involvement (p<0.001). PTX3 level was not associated with disease duration, anti-ds DNA antibody, decreased complement levels, PedSDI, active nephritis, active neuropsychiatric involvement, musculoskeletal involvement, hematological involvement, ESR,CRP, procalcitonin levels.

Conclusion: In brief, PTX3 levels were significantly correlated with SLEDAI scores. Additionally, its levels are found to be substantially increased in the presence of Raynaud’s phenomenon and vasculitic manifestations. Thus predicting the vascular involvement early and quantitatively in a c-SLE patient with presumed clinically inactive, will provide a better management of the disease. In conclusion, as in other vasculitides, PTX3 may represent a potential biomarker for vascular involvement in c-SLE.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pentraxin-3-level-predicts-vasculitis-and-mucocutaneous-involvement-in-childhood-onset-systemic-lupus-erythematosus/