Pulmonary Fibrosis has been appreciated in HPS-1 and HPS-4 patients, whose genetic defects are in biogenesis of lysosome-related organelle complex 3 (BLOC-3), which includes HPS1 and HPS4 proteins, and, less commonly, HPS-2 patients (Brantly et al., 2000; Anderson et al., 2003; Chiang et al., 2003; Li et al., 2004; Carmona-Rivera et al., 2013). This evidence concerns the gene HPS4 and pulmonary fibrosis.