B cell loss leading to remission in severe systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) pathogenesis is mediated in part by autoantibodies. We describe a patient with central nervous system lupus who developed a loss of B cells with associated hypogammaglobulinemia and sinopulmonary infections requiring intravenous immunoglobulin. The SLE went into complete remission. Of 18 reported patients with SLE developing persistent hypogammaglobulinemia, only 5 patients including ours had a nearly complete loss of circulating B cells. Of those whose SLE and B cell status was reported, 5/5 with B cell loss and 1/10 without B cell loss experienced a durable response of SLE (p = 0.002). These cases illustrate that B cell ablative therapies may have efficacy for SLE.
Tarrant
Teresa K
TK
Department of Medicine, Duke University Medical Center, Durham, North Carolina 27710, USA. tarra002@mc.duke.edu
Frazer
D Hugh
DH
Aya-Ay
James P
JP
Patel
Dhavalkumar D
DD
D000328:Adult; D000361:Agammaglobulinemia / Q000209:etiology*; D001402:B-Lymphocytes / Q000166:cytology* / Q000276:immunology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000276:immunology* / Q000628:therapy*; D018655:Lymphocyte Count; D012074:Remission Induction; D012720:Severity of Illness Index
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents