Background Many patients with primary immunodeficiencies develop immune-mediated diseases that require biological treatment. For some time it has been denied the use of biological treatment to patients with immunodeficiencies, because of the theoretical risk of serious infections.
Objectives To analyze our experience in the use of biological agents in patients with primary immunodeficiency.
Methods Retrospective analysis of 8 patients over 18 years, diagnosed with primary imunodeficiency, by WHO classification, that have received biological treatment. Demographic data, type of immune-mediated disease, biological treatment used, response to treatment and rate of infection were record.
Results We present 8 patients that have received biological drugs. The mean age was 23 years, all of whom were male,except one woman. The time evolution of the priamry immunodeficiency was on average 10.2 years. Five patients have a common variable immunodeficiency, two chronic granulomatous disease and one of them agammaglobulinemia-X. Patients with chronic granulomatous disease and agammaglobulinemia received anti-TNF, for the treatment of inflammatory bowel disease. While patients with common variable immunodeficiency received rituximab; two of them for immune-mediated thrombocytopenia, two for lymphoid interstitial pneumonitis and another by transverse myelitis. Agammaglobulinemia-X and one patient with chronic granulomatous disease precised the use of various anti-TNF (infliximab, adalimumab, certolizumab). All patients had disease improvement in which biological treatment was indicated. The rate of infection was lower along the use of biological drugs (median follow up 1.2 years) than along the treatment with other immunosuppressants. There was only one episode of serious infection that required hospitalization, a patient with agammaglobulinemia-X who presented a herpes zoster, while being trated with steroids and certolizumab for inflammatory bowel disease. The perceived quality of life was better when using biological agents than previously to it.
Conclusions Our experience could open the prudent use of biological drugs for patients with primary immunodeficiencies, always under close monitoring and appropriate prescription.
Disclosure of Interest None declared
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