Biological Therapies in Patients with Primary Immunodeficiency Diseases

Tuğba ÖNALANa , Şevket ARSLANa
aNecmettin Erbakan University Faculty of Medicine, Department of Internal Medicine, Division of Immunology and Allergy Diseases, Konya, Türkiye

Önalan T, Arslan Ş. Biological therapies in patients with primary immunodeficiency diseases. Çölkesen F, ed. Primary Immunodeficiency Diseases in Adults. 1st ed. Ankara: Türkiye Klinikleri; 2024. p.115-24.

ABSTRACT
Primary immunodeficiencies are a group of disorders resulting from inborn errors of immunity. In addition to recurrent infections, immundysregulation, lymphoproliferation, and autoimmunity can be a part of their clinical spectrum; and management of these accompanying processes are as important as treating and controlling infections themselves. Targeted therapies are planned to eventually achieve a result in which either an aberrantly active pathway caused by a gain of function variant is silenced, or a deficient molecular process resulted from a loss of function is reinstituted. Biological drugs play a leading role in this personalized treatment modality. This article discusses the pathophysiology of primary immunodeficiencies (PID) accompanied by autoimmunity, genetic changes associated with them, and commonly used biological agents in the treatment.

Keywords: Immunodeficiency; autoimmunity; biological therapy; molecular targeted therapy

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