Representative images of the lung specimens from patients with the RNF213 p.Arg4810Lys variant demonstrated microscopically progressive stenosis, concentric remodeling, and subsequent occlusion of the pulmonary capillaries and veins as well as pulmonary arteries, which is not typical for patients with severe idiopathic PAH with BMPR2 mutations. Here, RNF213 is linked to pulmonary arterial hypertension.