Patients present with heterogeneous phenotypes ranging from severe and fatal neonatal respiratory distress (surfactant protein (SP)-B and the intracellular transporter ABCA3) to chILD (SP-C, ABCA3) or adult forms of FPF (SP-C, ABCA3) with or without adenocarcinoma of the lung (SP-A1, SP-A2) (Fig. 1)16, 17, 18, 19 This Position Paper will not specifically detail the genetic causes of ILD that have notably been the subject of a recent ERS statement,4 but rather highlight and discuss what could be the future direction of in vitro studies and clinical trials for patients and their relatives. This evidence concerns the gene SFTPC and interstitial lung disease.