Defining Common Variable Immunodeficiency Disorders in 2020

Common variable immunodeficiency disorders (CVID) are the most frequent symptomatic primary immune deficiency in adults. Because there is no known cause for these conditions, there is no single clinical feature or laboratory test that can confirm the diagnosis with certainty. If a causative mutation is identified, patients are deemed to have a CVID-like disorder caused by a specific primary immunodeficiency/inborn error of immunity. In the remaining patients, the explanation for these disorders remains unclear. The understanding of CVID continues to evolve and the authors review recent studies, which have addressed some of these uncertainties.