Elsevier

Journal of Autoimmunity

Volume 11, Issue 5, October 1998, Pages 465-470
Journal of Autoimmunity

Regular Article
Patients with Systemic Lupus Erythematosus have Reduced Numbers of Circulating Natural Interferon-α- Producing Cells

https://doi.org/10.1006/jaut.1998.0215Get rights and content

Abstract

Systemic lupus erythematosus (SLE) patients often have continuous production of interferon-α (IFN-α), but production of in vitro IFN-α by peripheral blood mononuclear cells (PBMC) may be varyingly reduced. We here report that IFN-α production induced by Herpes simplex virus (HSV) in PBMC resembling immature dendritic cells and designated natural IFN-α producing cells (NIPC), was much more affected than that induced by sendai virus (SV) in monocytes. At the cell level, the frequency of HSV-activated NIPC was reduced 70-fold, but residual NIPC produced normal amounts of IFN-α (1–2 U/cell). The NIPC frequency increased 10-fold in SLE-PBMC, but not in control PBMC, when co-stimulated by the combination IFN-α-γ and GM- CSF. No spontaneous IFN-α production by PBMCs was detected in SLE patients. While no SLE serum factor inhibiting IFN-α production was seen, sera of four out of 11 SLE patients induced IFN-α production in healthy control PBMC. We propose that the number of NIPC in SLE are reduced in blood because of recruitment to tissues and activation by an endogenous IFN-α inducer, as well as because of lack of co-stimulatory cytokines. IFN-α produced in SLE could be of pathogenic significance, because autoimmune diseases develop in patients with infections or tumours during IFN-α therapy.

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Correspondence to: Lars Rönnblom, Department of Internal Medicine, University Hospital, S-751 85 Uppsala, Sweden. Fax: +46 18 510133. E- mail:[email protected]

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