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.