Background: Systemic lupus erythematosus (SLE) is a heterogenous disease with complex pathogenesis. Aim: In this review, we summarise recent progress in the understanding of SLE pathogenesis and discuss implications for the treatment of SLE patients using standard and experimental medications. Conclusions: The discovery that Toll-like receptor signalling and interferon-α abundance are central elements of the disease process has led to a new appreciation for hydroxychloroquine as an essential baseline medication. Although much needs to be learned, modulation of the immune system via B-cell depletion is entering clinical practice. Mycophenolate mofetil is an effective and safer alternative to cyclophosphamide for many patients with lupus nephritis. Several other therapeutic approaches are at various stages of preclinical and clinical development. These include anticytokine therapies, co-stimulatory blockade, antigen-specific immune modulation and haematopoietic stem cell transplantation.
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