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Systemic Immune-Based Rheumatic Diseases: Blissless States of BLySfulness

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BLyS Ligands and Receptors

Part of the book series: Contemporary Immunology ((CONTIM))

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Abstract

B lymphocyte stimulator (BLyS) is a vital B-cell survival and differentiation factor. Overexpression of BLyS in mice can lead to clinical and serological features of systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SS). Treatment with BLyS antagonists of mice with established SLE ameliorates disease progression and enhances survival. Moreover, similar treatment of mice with inflammatory arthritis ameliorates the ongoing inflammation and subsequent joint destruction. In humans, BLyS overexpression is common in patients with several systemic immune-based rheumatic diseases (SIRDs), including SLE, rheumatoid arthritis (RA), SS, scleroderma, dermatomyositis, ANCA-associated vasculitis, and mixed cryoglobulinemia. Phase-I and phase-II clinical trials with BLyS antagonists in SLE and RA have documented in vivo biological activities along with favorable safety profiles for these agents and have pointed to beneficial effects in subsets of patients. These features collectively point to BLyS as an attractive therapeutic target in human SIRDs.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, University of Southern California Keck School of Medicine, 90033, Los Angeles, CA, USA

    William Stohl

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  1. William Stohl
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  1. School of Medicine, University of Pennsylvania, 36th and Hamilton Walk, Philadelphia, 19104-6082, U.S.A.

    Michael P. Cancro

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© 2009 Humana Press, a part of Springer Science+Business Media, LLC

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Stohl, W. (2009). Systemic Immune-Based Rheumatic Diseases: Blissless States of BLySfulness. In: Cancro, M. (eds) BLyS Ligands and Receptors. Contemporary Immunology. Humana Press. https://doi.org/10.1007/978-1-60327-013-7_8

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