B Cell Receptor Signaling in Human Systemic Lupus Erythematosus

Aimee E. Pugh-Bernard; John C. Cambier


Curr Opin Rheumatol. 2006;18(5):451-455. 

In This Article

Abstract and Introduction


Purpose of Review: The purpose of this review is to inform the scientific community of the most recent findings surrounding B cell receptor signaling function in human systemic lupus erythematosus and how altered B cell signaling may explain the characteristic hyperactivity of B cells in active disease and contribute to its pathogenesis.
Recent Findings: B cell receptor signaling is abnormal in patients with active systemic lupus erythematosus as demonstrated by increased calcium flux and global B cell hyperactivity. Altered signaling has been explained by a variety of factors such as defective FcγRIIB signaling, decreased expression of the protein tyrosine kinase Lyn, and increased serum levels of B lymphocyte stimulator.
Summary: The studies reviewed suggest that B cells from systemic lupus erythematosus patients display molecular signaling defects that most likely contribute to pathogenesis of the disease and explain the characteristic hyperactivity of B cells in active disease.


Systemic lupus erythematosus (SLE) is a complicated autoimmune disease diagnosed on presentation of a variable subset of a wide array of clinical symptoms. The feature common to all SLE patients, however, is the presence of autoantibodies. Although self-reactive B cells produce the autoantibodies essential to the diagnosis of disease, B cells have proven in recent years to be active participants in the development of disease irrespective of autoantibody production. In light of this advancement, a central question surrounding the pathogenesis of the disease is whether intrinsic defects in SLE B cells play a role in triggering the immunological events that result in the onset of clinical disease. Although other immune cells play a role in SLE, B cells from SLE patients display signaling defects that may underlie pathogenesis and explain the characteristic hyperactivity of B cells in active disease. This article will present the current understanding of how B cell signaling plays a role in the pathogenesis and exacerbation of disease in human SLE.


Comments on Medscape are moderated and should be professional in tone and on topic. You must declare any conflicts of interest related to your comments and responses. Please see our Commenting Guide for further information. We reserve the right to remove posts at our sole discretion.