Virgil Dalm, MD, PhD, internist and clinical immunologist at Erasmus MC, Rotterdam, Netherlands, discusses autoinflammatory mechanisms in patients with primary immunodeficiency (PID). The mechanisms are not well understood, but some genetic variants in patients with common variable immune deficiency (CVID) lead to a clear disturbance in B-cell phenotypes. Treatment is very difficult due to the increased risk of infections with primary immunodeficiencies when the general treatment for autoimmune complications includes immunosuppressants. Patients with primary immunodeficiency and autoinflammatory complications also share their experiences.