Lymphocyte alterations in patients with Common Variable Immunodeficiency (CVID) and autoimmune manifestations

Autoimmunity is a common feature in CVID patients. To date the mechanisms leading to the development of such complications are not fully elucidated. Data from 122 CVID patients subdivided in three groups based on the absence of autoimmunity (n-AI) or the presence of hematologic autoimmune phenomena (Cy-AI) or non-hematologic autoimmune phenomena (n-Cy-AI) were evaluated. We identified a total of 128 autoimmune manifestations in 55/122 patients (45.1%). 30/122 (24.6%) patients presented hematologic autoimmune phenomena while 29/122 (23.8%) presented gastrointestinal autoimmune involvement. Immune thrombocytopenia was the most common manifestation (27/122; 22.1%), followed by autoimmune hemolytic anemia (18/122; 14.8%) and autoimmune enteropathy (17/122; 13.9%). Cy-AI patients displayed higher CD4+ effector memory and terminally differentiated CD8+ cells with lower percentages of naïve and recent thymic emigrants (RTEs) CD4+ cells and a significant expansion of the CD19hiCD21low population. CVID patients developing autoimmune cytopenias display characteristic immune phenotypic features. •Autoimmune phaenomena frequently complicate the clinical course of patients with CVID.•Hematological and gastrointestinal autoimmune manifestations are the most prevalent ones.•Patients with CVID and autoimmunity present distinct lymphocyte patterns.