Case of Spontaneous Renal Artery Dissection in EDS Patient Highlighted in Report

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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spontaneous renal artery dissection

A diagnosis of spontaneous renal artery dissection (SRAD) should be considered in Ehlers-Danlos syndrome (EDS) patients who experience sudden abdominal pain, a case report highlights.

The report, “Spontaneous Renal Artery dissection in Ehler-Danlos Syndrome,” was published in the journal Kidney International Reports.

Renal infarction is the term used for when the blood flow in the kidneys is blocked. Typically, this happens in people who are also dealing with other conditions, such as heart disease, cancer, or traumatic damage to the kidneys. About a third of cases of renal infarction, however, are considered idiopathic, or with no clear cause.

SRAD is an emerging diagnosis that may account for some, if not most, of these idiopathic cases. It is associated with conditions that affect the vascular tunica media (part of the wall of blood vessels), including EDS, and may be triggered by strenuous exercise.

The case report details the story of a 48-year-old white male who experienced sudden, severe back pain following sexual intercourse.

Initial screening showed normal blood pressure, lack of fever, and normal heart measurements. Overall, the patient appeared in good health, and ultrasounds ruled out kidney stones.

Laboratory tests, however, revealed a mild reduction in kidney renal function, and a computed tomography (CT) scan confirmed renal infarction in the left kidney.

Further examination in the months following the acute episode suggested a diagnosis of SRAD. The condition eventually resolved itself with only symptomatic management, which included medications to manage pain.

Interestingly, the patient had not previously been diagnosed with EDS, but after questioning him, the team learned  that one of his children had the disease.

This led to genetic testing, which revealed a mutation in the COL5A1 gene, although it was not clear whether this variant would be the cause of the disease.

Additional analysis of skin biopsies from both the patient and the child with confirmed EDS showed that both had changes in connective tissue that would be indicative of the syndrome, confirming the diagnosis.

“This case shows the need for attention to SRAD, a rare, possibly under-diagnosed condition, and its association with genetic collagen disorders,” the researchers concluded.

According to them, this case report draws attention to SRAD “as a cause of kidney infarction, and suggest considering it in the case of symptoms suddenly appearing after strenuous efforts or sexual intercourse.”

Th team believes this report is also “a reminder of the importance of obtaining a detailed clinical history, so that time-consuming, expensive, and stressful evaluations can be avoided.”