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Rheumatology Advance Access originally published online on July 14, 2008
Rheumatology 2008 47(9):1400-1405; doi:10.1093/rheumatology/ken255
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Management of hypogammaglobulinaemia occurring in patients with systemic lupus erythematosus

P. F. K. Yong1,2, L. Aslam2, M. Y. Karim2,3 and M. A. Khamashta2

1Department of Clinical Immunology, Kings College Hospital, 2Louise Coote Lupus Unit, St Thomas’ Hospital, London and 3Department of Immunology, Frimley Park Hospital, Frimley, UK.

Correspondence to: M. Y. Karim, Louise Coote Lupus Unit, St Thomas’ Hospital, Lambeth Palace Road, London SE1 7EH, UK. E-mail: yousuf.karim{at}gstt.nhs.uk


  Abstract

Objectives. Systemic lupus erythematosus (SLE) is typically associated with hypergammaglobulinaemia but has been described in the setting of hypogammaglobulinaemia as well. The purpose of this article is to describe various cases of SLE and hypogammaglobulinaemia, review the literature and present management strategies for hypogammaglobulinaemia in SLE.

Methods. We describe five patients with SLE and antibody deficiency, and review the literature exploring the relationship between the two.

Results. Various types of antibody deficiency syndromes, including common variable immunodeficiency (CVID), IgA deficiency, IgM deficiency, drug-induced hypogammaglobulinaemia and hypogammaglobulinaemia secondary to nephrotic syndrome can occur in SLE. Antibody deficiency states can be treated with antibiotics and replacement immunoglobulin therapy (particularly CVID) but sometimes close monitoring is all that is required.

Conclusion. Measurement of immunoglobulin levels is useful in SLE to identify coexisting antibody deficiency and the later development of hypogammaglobulinaemia. This allows monitoring and appropriate treatment to be instituted.

KEY WORDS: Systemic lupus erythematosus, Primary antibody deficiency, Immunodeficiency, Hypogammaglobulinaemia, Immunoglobulin G

Submitted 21 September 2007; revised version accepted 13 June 2008.
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