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.

Colour-Doppler US evaluation of patients with hypertension and nephropathy

“…CDUS consistently revealed haemodynamically significant stenosis of the stent site with parvus-tardus pulse below the stenosis, in most cases due to the presence of fibrointimal celiaco e malattie autoimmuni (dermatite erpetiforme, diabete insulino dipendente); spesso ad esordio clinico con ipertensione nefrovascolare e claudicatio [22][23][24].…”

“…In the patient with middle aortic syndrome, CDUS revealed the alteration in renal haemodynamics, thus justifying the use of a more panoramic modality, such as CT angiography, which shed light on the patient's complex anatomical situation [22][23][24].…”

“…Rarely reported in the literature, the syndrome has been described in young subjects (6-28 years old) as a rare variant of aortic coarctation (0.2%-2% of aortic coarctations) and to be associated with AAA and RAS. Multiple causes have been recognised, including congenital and secondary to Takayasu's syndrome, von Recklinghausen's neurofibromatosis, coeliac disease and autoimmune disorders (dermatitis herpetiformis, insulindependent diabetes), and clinical onset is usually renovascular hypertension and claudication [22][23][24].…”

“…Questi sono pazienti a prognosi infausta con mortalità ad 1 anno variabile dal 64% all'87% in cui il monitoraggio strumentale può essere certamente di ausilio al clinico nell'affrontare una così complessa patologia, dal momento che in tali pazienti è fortemente controindicata l'angiografia, sia perché manovra interventistica sia per la somministrazione di eparina [31][32][33]34]. Nella paziente con la middle aortic syndrome l'ECD ha svelato l'alterazione emodinamica a livello renale, giustificando il ricorso a metodiche più panoramiche come l'angio-TC che ha rilevato la complessa situazione anatomica da cui era affetta [22][23][24].…”

Endovascular abdominal aortic stenosis treatment with the optimed self‐expandable nitinol stent

“…Coarctation of the abdominal aorta is an uncommon disease in which different etiologies and treatment methods have been reported. There are a few life threatening sequelae of untreated disease, such as uncontrolled hypertension from chronic renin‐angiotensin activation, gastro‐intestinal tract ischemia, lower limb ischemia or emboli, renal failure, aneurysm rupture, cardiac hypertrophy, and arrhythmia [1–11].…”