Chronic Spontaneous Urticaria in Patients with Common Variable Immunodeficiency

Abstract

Common variable immunodeficiency (CVID) is a predominantly antibody deficiency and is one of the most common primary immunodeficiencies in adulthood. Replacement therapy with Ig has significantly reduced infectious complications; however, malignant, autoimmune, and inflammatory diseases are still current major causes of morbidity and mortality. In recent years, interest has increased regarding allergic manifestations that may be associated with primary immunodeficiencies; however, no data are currently available on chronic spontaneous urticaria (CSU). In this report, the authors describe CSU in patients with CVID attending their centre. Three CVID patients were affected by CSU and were unresponsive to antihistamines. Patients were screened for the presence of serum autoreactivity by an autologous serum skin test; only one patient was positive for serum autoreactivity. The serum of this patient was found to induce CD63 upregulation on basophils and degranulation of LAD2 mast cells. All patients were treated with omalizumab therapy at the standard dose of 300 mg every 4 weeks. The patient with autoreactive serum was the best responder to omalizumab therapy, whereas the other two patients experienced urticaria flares related to intercurrent infections. In this article, the authors describe the presence of CSU in patients with CVID for the first time. Although autoimmunity is a feature of CVID, autoreactivity was documented in one patient only, thus showing that CSU in patients with CVID reflects the heterogeneity of this immune defect.