Common variable immunodeficiency in systemic lupus erythematosus.
OBJECTIVE
The development of common variable immunodeficiency (CVID) or hypogammaglobulinemia in systemic lupus erythematosus (SLE) is rare. The purpose of this article is to provide a detailed review of lupus-associated CVID and to identify clinical characteristics and laboratory features in patients with SLE-associated CVID.


METHODS
We describe 2 patients with SLE and CVID and review the cases published in the English literature highlighting both the demographic and the clinical characteristics and the laboratory and therapeutic aspects of this disorder.


RESULTS
Detailed descriptions of 18 patients were available; 89% were females with a mean age at the onset of SLE of 23.8 years. In 50% of patients CVID developed within the first 5 years after the diagnosis of SLE. All patients had been treated with corticosteroids and 72% had also received immunosuppressive therapy. Sinopulmonary infections were the most frequent symptom. SLE disease activity decreased after the development of CVID in 67% of patients. Most patients (89%) were treated with gammaglobulin therapy. The most notable immunological feature was a reduced number or percentage of B-cells in 60% of patients.


CONCLUSIONS
CVID should be suspected in any SLE patient with recurrent sinopulmonary infections in the absence of SLE activity and/or immunosuppressive treatment.
Fernández-Castro
Mónica
M
Department of Rheumatology, Hospital Universitario Puerta de Hierro, Universidad Autónoma de Madrid, Madrid, Spain.
Mellor-Pita
Susana
S
Citores
María Jesús
MJ
Muñoz
Pilar
P
Tutor-Ureta
Pablo
P
Silva
Lucia
L
Vargas
Juan Antonio
JA
Yebra-Bango
Miguel
M
Andreu
José Luis
JL
D000293:Adolescent; D000328:Adult; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D016131:Lymphocyte Subsets / Q000145:classification*
D016756:Immunoglobulins, Intravenous