Autoimmunity and immune dysregulation may lead to cytopenia and represent key features
of many primary immunodeficiencies (PIDs). Especially when cytopenia is the initial …

Autoimmune manifestations are increasingly being recognized as a component of several
forms of primary immunodeficiencies (PID). Defects in purging of self-reactive T and B cells …

Primary immune deficiencies have an estimated overall incidence of 1 in 10,000 individuals.
These disorders are diverse, depending on the specific immune functions involved, and lead …

Primary immune deficiencies are often associated with autoimmune disease due to the
dysregulation of the immune system as a whole. In many immune deficiencies, lymphocytes …

Background Immune dysregulation is as important as susceptibility to infection in defining
primary immunodeficiencies (PIDs). Because of the variability and nonspecificity of the …

A broad spectrum of autoimmunity is now well described in patients with primary
immunodeficiencies (PIDs). Management of autoimmune disease in the background of PID …

Primary immunodeficiencies (PIDs) are a widely heterogeneous group of inherited defects of
the immune system consisting of many clinical phenotypes with at least 300 underlying …

Autoimmune manifestations have long been perceived as paradoxical in patients with
primary immune deficiencies (PID). However, a defect in the mechanisms of control of self …

Primary immune deficiencies (PIDs) are a growing group of over 230 different disorders
caused by ineffective, absent or an increasing number of gain of function mutations in …

Human primary immunodeficiencies (PIDs) are often thought to be confined to a few rare,
familial, monogenic, recessive traits impairing the development or function of one or several …