In addition to being a vital organ for gas exchange, the lung is a crucial immune organ
continuously exposed to the external environment. Genetic defects that impair immune …

ILD is a frequent complication found in many IEI. This article provides a guide to identifying
manifestations of ILD in IEI. We review a broad spectrum of IEI that develop ILD, including …

In recent years, a growing number of monogenic disorders have been described that are
characterized by immune dysregulation. A subset of these “primary immune regulatory …

Background Inborn errors of immunity (IEIs) are a group of congenital diseases caused by
genetic defects in the development and function of the immune system. The involvement of …

Inborn errors of immunity (IEIs) are a group of conditions whereby parts of the immune
system are missing or dysfunctional. Once thought to primarily be a pediatric disorder, it is …

Inborn errors of immunity (IEIs) are generally considered to be rare monogenic disorders of
the immune system that cause immunodeficiency, autoinflammation, autoimmunity, allergy …

Background Inborn errors of immunity (IEIs) are a group of conditions affecting immune
system development and function. Due to their clinical heterogeneity and lack of provider …

Recurrent respiratory infections (RRI s) are frequent in children and are characterized by
more than 6 airway infections in 1 year or more than 1 upper airway infection per month in …

Background Inborn errors of immunity (IEI) comprise more than 400 rare diseases with
potential life‐threatening conditions. Clinical manifestations and genetic defects are …

The field of immunology has a rich and diverse history, and the study of inborn errors of
immunity (IEIs) represents both the “cake” and the “icing on top of the cake,” as it has …