COPA (coatomer protein complex subunit α) syndrome is a rare inborn error of immunity associated with constitutive activation of type I interferon signaling. Organ inflammation and damage (i.e. lungs, kidneys, joints) usually develops early in life. Kidney involvement occurs in a subset of COPA syndrome patients, with the potential for rapid progression to kidney failure. Through an international collaboration, we identified COPA syndrome patients who developed kidney failure and underwent a kidney transplantation.