Rheumatic manifestations of primary immunodeficiencies in children

Primary immunodeficiency disorders (PIDs) usually present with a variety of infections due to a defect in the body's defence mechanisms which can affect both the adaptive and the innate immune systems. The infections can involve bones and joints. Beside infections, autoimmune diseases are the second most common feature of PID. Arthritis is the most common rheumatological manifestation of PIDs. Consider PID in a child who presents with recurrent septic arthritis, arthritis associated with increased susceptibility to infection, autoimmune disease involving multiple organs or systemic lupus erythematosus with negative antinuclear antibody (ANA).