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Review
. 2001 Dec 17;194(12):F59-63.
doi: 10.1084/jem.194.12.f59.

A pivotal role for the natural interferon alpha-producing cells (plasmacytoid dendritic cells) in the pathogenesis of lupus

L Rönnblom  1 , G V Alm
Affiliations
Review

A pivotal role for the natural interferon alpha-producing cells (plasmacytoid dendritic cells) in the pathogenesis of lupus

L Rönnblom et al. J Exp Med. .
No abstract available

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Figures

Figure 1.
Figure 1.
Hypothetical role of NIPCs, also termed PDCs, in SLE. The NIPCs/PDCs become activated naturally by infectious microorganisms, but in SLE also by DNA or RNA molecules in complex with autoantibodies (endogenous IFN-α inducers) in an FcγRII-dependent manner. Such nucleic acids, possibly bound to proteins, may be derived from apoptotic cells. The TLR9 is expressed on the NIPCs/PDCs and mediates responses to unmethylated CpG-DNA. The CD40 on NIPCs/PDCs deliver costimulatory signals when interacting with CD40-ligands, resulting in IL-12 production and enhanced IFN-α synthesis in response to at least CpG-DNA. The C-type lectin BDCA-2 is also depicted (natural ligand unknown) and ligation of such molecules inhibits the IFN-α production. The IFN-α finally acts on many cells, one immediate effect being costimulation (priming) of NIPCs/PDCs that is essential for efficient IFN-α production. Furthermore, the IFN-α acts on DCs and T- and B lymphocytes to counteract self-tolerance and promote autoimmunity. This results in the immune-mediated inflammation seen in SLE, as well as generation of more endogenous IFN-α inducers.
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