Abstract
Background
Systemic lupus erythematosus (SLE) is associated with a numerical and functional reduction
of peripheral blood (PB) invariant natural killer T (iNKT) cells. Limited information
exists on the role of iNKT cells in the pathogenesis of lupus erythematosus.
Objective
To investigate the frequency and phenotype of iNKT cells in PB and dermal infiltrates
from patients with SLE, subacute-cutaneous lupus erythematosus (SCLE) and discoid
lupus erythematosus (DLE).
Methods
PB was obtained from 23 SLE, 6 SCLE, and 11 DLE patients, and from 30 healthy controls.
iNKT cell frequency and CCR4/CCR6 surface expression were assessed by flow cytometry.
The frequency and phenotype of skin infiltrating Vα24+Vβ11+ iNKT cells were investigated by immunofluorescence in lesional biopsies from 20 patients,
unaffected skin from 3 patients, and from 6 healthy controls.
Results
Lupus erythematosus patients displayed significantly lower percentages of circulating
CD3+6B11+ iNKT cells compared to healthy controls. Whereas CCR6 expression on iNKT cells was
enhanced in active SLE patients regardless of cutaneous involvement compared to healthy
controls, CCR4 was exclusively increased in patients with active cutaneous lesions.
Furthermore, iNKT cells were significantly enriched in lesional skin of SLE and DLE
patients, but not in unaffected skin of lupus patients. The majority of lesional iNKT
cells expressed IFN-γ and CCR4.
Conclusion
The deficiency in circulating iNKT cells in cutaneous lupus erythematosus is associated
with an increase of iNKT cells at the site of cutaneous inflammation. These data underscore
the importance of analyzing iNKT cells not only in PB, but also in the target tissues.
Keywords
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Article info
Publication history
Published online: May 09, 2013
Accepted:
April 4,
2013
Received in revised form:
March 3,
2013
Received:
September 15,
2012
Identification
Copyright
© 2013 Japanese Society for Investigative Dermatology. Published by Elsevier Inc. All rights reserved.
