Mechanisms for PH development in ILD include fibrotic vascular ablation, alveolar–septal remodeling, chronic inflammation, aberrant angiogenesis, and potentially genetic factors (e.g., Bone Morphogenetic Protein Receptor type 2 (BMPR2) and TGF-β pathway) [56,57,58,59,60]. Here, BMPR2 is linked to interstitial lung disease.