Agency for Healthcare Research and Quality.

Agency for Healthcare Research and Quality.

significantly more common in PXE patients in the intracranial internal carotid artery (75% vs 44%), the arteries of the arms (20% vs 3%), the femoral-popliteal arteries (74% vs 44%), and the infrapopliteal arteries (84% vs 38%). In these arteries, calcification scores also indicated more severe calcification. Severe calcification was significantly more prevalent in PXE patients compared to controls in the internal intracranial carotid artery (36% vs 19%; P < .001), femoral-popliteal arteries (51% vs 31%; P < .001), and infrapopliteal arteries (61% vs 24%; P < .001). In the hospital controls, significant more severe calcification was observed for the thoracic aorta, the mesenteric arteries, and the external iliac arteries. No significant differences in prevalence of arterial calcification were observed in other arterial beds, such as the coronary arteries (45% vs 43%; P ¼ .776), the carotid arteries (52% vs 46%; P ¼ .476), and the abdominal aorta (71% vs 63%; P ¼ .287). Subgroup analyses of patients younger than 55 years only showed similar differences in prevalence of arterial calcifications between PXE patients and controls, with most pronounced calcifications in the arteries of the lower legs (67% vs 8%). Similar patterns were observed in those without concomitant diabetes or renal dysfunction. Comments: This study adds to our information regarding the location of arterial disease in patients with PXE, confirming that leg arteries are commonly involved and more so than a control population of similar demographics. The involvement of the intracranial internal carotid artery might explain the suspected brain involvement in PXE, which has not be clearly investigated in the past. The involvement of arm arteries with calcification is extremely rare without diabetes and/or renal failure and may suggest PXE in patients so discovered at an early age. The major limitation of this study is the use of hospital controls that have disease states, which might confound the interpretation, but the overall effect is likely minor.

Agency for Healthcare Research and Quality.