Is there a relationship between Ehlers-Danlos syndrome (EDS) and the development of vasculitis or kidney disease?

From the Guidelines

There is a potential relationship between Ehlers-Danlos syndrome (EDS) and vascular complications, including vasculitis and kidney disease, though the connection varies by EDS subtype, with Vascular EDS (vEDS) having the strongest association with blood vessel and organ complications.

Key Considerations

• Vascular EDS (vEDS) is associated with potential kidney involvement due to arterial ruptures affecting renal blood vessels, as noted in the 2024 European Heart Journal guidelines 1.
• The connection between EDS and kidney disease is indirect, with kidney issues often arising from vascular complications or comorbid conditions rather than direct kidney pathology.
• Patients with EDS, particularly vEDS, should undergo regular cardiovascular monitoring, including blood pressure checks and vascular imaging when appropriate, as recommended by the 2022 Circulation guidelines 1.
• Kidney function tests (creatinine, BUN, urinalysis) may be recommended, especially if symptoms develop, to monitor for potential kidney involvement.

Management and Prevention

• Management focuses on preventing complications through blood pressure control (often with medications like beta-blockers), avoiding activities that increase vascular strain, and prompt evaluation of symptoms like flank pain, blood in urine, or unexplained fever.
• The use of celiprolol, a beta blocker with vasodilatory properties, has been suggested to be beneficial in patients with vascular Ehlers-Danlos syndrome, although data are limited and more research is needed, as noted in the 2022 Circulation guidelines 1.
• Surgical repair in vascular Ehlers-Danlos syndrome carries an increased risk due to vascular fragility and associated bleeding complications, and should be approached with caution, as discussed in the 2024 European Heart Journal guidelines 1.

Underlying Mechanism

• The connection between EDS and vascular or kidney complications exists because EDS affects collagen production and structure, which is crucial for blood vessel integrity and kidney tissue architecture, potentially leading to vascular fragility and subsequent organ damage in certain EDS subtypes, as explained in the 2010 Circulation guidelines 1.

From the Research

Relationship between Ehlers-Danlos Syndrome and Vasculitis or Kidney Disease

• There is evidence to suggest that Ehlers-Danlos syndrome (EDS) is associated with vascular complications, including aneurysms, dissections, and ruptures of arteries 2, 3, 4, 5, 6.
• Vascular EDS is characterized by the fragility of medium and large arteries due to type III procollagen deficiency, leading to a high incidence of vascular lesions 3, 6.
• The syndrome can result in life-threatening complications, including rupture of arterial vessels, intestinal organs, and the uterus 2.
• Patients with EDS are predisposed to premature vascular complications, and the manifestation of vascular EDS frequently involves multiple vascular segments 4.
• Imaging findings in patients with vascular EDS include aneurysms, dissections, ectasias, and occlusions, as well as parenchymal infarcts involving the brain, kidney, and spleen 6.
• There is no direct evidence to suggest a relationship between EDS and vasculitis, but the syndrome can lead to vascular complications that may increase the risk of kidney disease 5, 6.
• Surgical management of patients with EDS is hazardous and often unrewarding due to the extreme fragility of arteries, and operative mortality remains high 3, 5.

Vascular Complications and Kidney Disease

• Vascular EDS can lead to kidney disease due to the formation of aneurysms and dissections in the renal arteries, as well as parenchymal infarcts involving the kidney 5, 6.
• The syndrome can also increase the risk of hemorrhagic events, including those related to spontaneous vascular rupture and interventional or surgical procedures 6.
• The key to favorable outcomes lies in identification of the syndrome preoperatively, surgical intervention only in life- or limb-threatening situations, and appropriate modification of surgical technique 5.