Additionally, the RNF213 p.R4810K variant is associated with peripheral pulmonary artery stenosis.[5] While such stenosis is also observed in conditions such as chronic thromboembolic pulmonary hypertension and pulmonary tumor thrombotic microangiopathy, the constriction in these instances stems from inflammatory reactions spurred by blood clots and malignant cells.[18,19] Differentiating these conditions using computed tomography, lung ventilator/perfusion scans, and other techniques is complicated. Here, RNF213 is linked to thrombotic microangiopathy.