Midaortic syndrome and celiac disease: a case of local vasculitis

Abstract

Midaortic syndrome is a variety of aortic coarctation, located in the distal thoracic aorta, the abdominal aorta or both, involving the intestinal and renal vessels, usually presenting with renovascular arterial hypertension. Underlying conditions are thought to be Takayasu’s arteritis, von Recklinghausen’s disease, and connate hypoplasia. Celiac disease is an inflammation in the small intestine, triggered by an allergic reaction to gluten. It is known to be associated with a variety of other autoimmune disorders, e.g., dermatitis herpetiformis (Duhring’s disease), insulin-dependent diabetes mellitus, and IgA nephropathy. We describe the case of a young woman who presented with claudication of the lower limbs, therapy-refractory arterial hypertension, and untreated celiac disease. We found a midaortic syndrome, characterized by severe stenosis of the infrarenal aorta, of both renal arteries (more pronounced on the right side) and of the inferior mesenteric artery. We assume that—after having excluded other possible pathogeneses—the underlying condition is a local vasculitis in the abdominal aorta and the renal and mesenteric arteries due to the chronic inflammation of untreated celiac disease. We performed a percutaneous transluminal angioplasty together with implantation of two stents into the infrarenal aorta and the right renal artery and started treating the celiac disease by dietary intervention. The patient is now under regular medical control and observation.