Clinical characteristics of chronic spontaneous urticaria in patients with Common variable immune deficiency and hypogammaglobulinemia

Abstract

Chronic spontaneous urticaria is a prevalent disease that is associated with various autoimmune, infectious (viral, bacterial, parasitic), and non-infectious inflammatory pathologies, and could occur in the symptom complex of other diseases, such as primary immunodeficiencies. Nowadays, data on the features of chronic spontaneous urticaria in patients with primary immunodeficiencies are accumulating. Common variable immune deficiency takes a special place in clinical practice among primary immunodeficiencies with impaired antibody synthesis and is associated with late-onset in adulthood, as well as the heterogeneity of its clinical manifestations, such as infectious and non-infectious complications, which can serve as a background for chronic urticaria development. Intravenous Immunoglobulin (IVIg) replacement therapy is the mainstay of treatment and prevention of infectious complications in these patients; however, immunomodulatory and anti-inflammatory effects can occur in high doses. Herein, presented two clinical cases of patients with common variable immune deficiency and one with hypogammaglobulinemia who suffer from chronic spontaneous urticaria. The peculiarities of IVIg replacement therapy at urticaria were noted in these patients. The discussion presents the review of world literature and proposes key features for further investigations.