Spontaneous Renal Artery Dissection in Ehler–Danlos Syndrome

Renal infarction is a relatively infrequent condition, most commonly occurring in the presence of predisposing factors, including atherosclerosis, valvular or ischemic heart disease, atrial fibrillation, endocarditis, hypercoagulability, malignancy, vasculitis, and kidney trauma. About 30% of renal infarctions, however, occur in the absence of these classic risk factors and are usually called “idiopathic.”1 In this noncategorized group, an emerging diagnosis is spontaneous renal artery dissection (SRAD), which may account for several, if not the majority of cases. The largest series so far published reports on 17 cases, mostly in males in their fourth or fifth decade of life, usually without any underlying disease. Strenuous exercise may be a facilitating factor, and SRAD is associated with diseases and conditions affecting the vascular tunica media, including fibromuscular dysplasia, Marfan syndrome, Ehlers-Danlos syndrome, polyarteritis nodosa, and cystic medial necrosis.5 The clinical and laboratory presentation is shared with all other types of kidney infarction, with sudden-onset flank pain, which radiates to the epigastrium or groin, and high lactate dehydrogenase. Retroperitoneal hemorrhage may be a rare presentation of SRAD, whereas new onset, occasionally severe hypertension may suggest SRAD in the context of kidney infarction. The case reported here highlights this emerging diagnosis as a cause of kidney infarction and suggests that it should be considered in the case of symptoms that appear suddenly after physically strenuous activities or sexual intercourse