On lung histology, ABCA3 deficiency is characterized by AEC II hyperplasia, variable degrees of interstitial thickening, prominent macrophages, and proteinaceous material in the alveolar spaces, which is an aspect that is frequently described as chronic or desquamative or non-specific interstitial pneumonitis or alveolar lung proteinosis; lung fibrosis is associated in fatal cases; however, these changes are frequently seen in other surfactant metabolism conditions, including SFTPB and SFTPC deficiency [11]. This evidence concerns the gene ABCA3 and pulmonary fibrosis.