Therefore, we examined the expression and distribution pattern of MIF and its two receptors, CD74 and CXCR4, in the lungs of patients with IPF-PH, and tested the effects of ISO-1 and compound 31, a new small molecule targeting MIF, on BLM-induced collagen deposition, PH, and vascular remodeling in mouse lungs. Here, CD74 is linked to idiopathic pulmonary fibrosis.