Introduction
Childhood onset interstitial lung disease (chILD) due to connective
tissue or immune-mediated disorders comprises 16.5% of patients in the
U.S. Children’s Interstitial Lung Disease Research Network Registry
Cohort [1]—the second largest diagnostic category. This is likely
an underestimate, as some patients may have secondary immune-mediated
ILD in the “alveolar hemorrhage”, “other specific multisystemic
disorders”, and “environmental/toxic/drug related” categories (9.2%,
7%, and 2.2% of the registry cohort, respectively).
The goal of this review is to familiarize the reader with the distinct
patterns of ILD associated with monogenic inborn errors of immunity and
systemic autoimmune and autoinflammatory diseases, and to highlight
current and emerging therapies. Through advances in our understanding of
cellular and genetic pathophysiology and improvements in
multidisciplinary management, the care of children with immune mediated
chILD is rapidly advancing.