Autoimmunity in common variable immunodeficiency.
BACKGROUND
Autoimmunity has been increasingly recognized as a major issue in patients with common variable immunodeficiency (CVID), the most common symptomatic primary immunodeficiency in adulthood. Different authors report high prevalences of autoimmune diseases in CVID, and several mechanisms have been proposed to explain this apparent paradox. Genetic predisposition, under current surveillance, innate and adaptive immunity deficiencies leading to persistent/recurrent infections, variable degrees of immune dysregulation, and possible failure in central and peripheral mechanisms of tolerance induction or maintenance may all contribute to increased autoimmunity.


CONCLUSIONS
Data on the clinical/immunological profile of affected patients and treatment are available mostly concerning autoimmune cytopenias, the most common autoimmune diseases in CVID. Treatment is based on conventional alternatives, in association with short experience with new agents, including rituximab and infliximab. Benefits of early immunoglobulin substitutive treatment and hypothetical premature predictors of autoimmunity are discussed as potential improvements to CVID patients' follow-up.
Lopes-da-Silva
Susana
S
Serviço de Imunoalergologia, Hospital de Santa Maria, Unidade de Imunologia Clínica, Instituto de Medicina Molecular, Faculdade de Medicina de Lisboa, Lisbon, Portugal. slopessilva@hsm.min-saude.pt
Rizzo
Luiz Vicente
LV
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001327:Autoimmune Diseases / Q000150:complications / Q000235:genetics / Q000276:immunology / Q000628:therapy; D015551:Autoimmunity / Q000235:genetics; D001938:Brazil; D017074:Common Variable Immunodeficiency / Q000235:genetics / Q000276:immunology* / Q000628:therapy; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D016867:Immunocompromised Host; D000069285:Infliximab; D008297:Male; D009894:Opportunistic Infections / Q000150:complications / Q000276:immunology / Q000628:therapy; D011110:Polymorphism, Genetic; D015995:Prevalence; D000069283:Rituximab; D017634:Self Tolerance / Q000276:immunology*; D012737:Sex Factors
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D000069285:Infliximab