While macrophages or myeloid cells have been the target in IPF, including Galecto’s galectin-3 inhibitor GB0139 and Hoffmann-La Roche’s zinpentraxin (RPM-151; a recombinant human pentraxin-2), neither of these targeting strategies have affected the primary endpoint of slowing the forced vital capacity decline in clinical trials. Here, APCS is linked to idiopathic pulmonary fibrosis.