From the Guidelines
Hypermobile Ehlers-Danlos Syndrome (hEDS) can potentially cause hematuria due to the fragility of blood vessel walls and connective tissues in the urinary tract. The connection between hEDS and hematuria exists because hEDS affects collagen production throughout the body, including tissues in the urinary system 1. Patients with hEDS who experience hematuria should seek medical evaluation, as it could be related to their underlying connective tissue disorder or might indicate a separate urological issue requiring treatment. The management typically involves identifying the specific cause of bleeding, ruling out other conditions, and addressing any contributing factors. According to the latest guidelines on adult neurogenic lower urinary tract dysfunction, patients with neurogenic lower urinary tract dysfunction (NLUTD) should be educated on the signs and symptoms that would warrant additional assessment, including hematuria 1. No specific medication regimen exists specifically for hEDS-related hematuria, but treatment is individualized based on severity and underlying mechanisms. Patients should maintain good hydration and follow up with both their EDS specialist and a urologist if hematuria persists or recurs, as distinguishing between hEDS-related bleeding and other urological conditions is important for proper management. Some key points to consider in the management of hEDS patients with hematuria include:
- Identifying the specific cause of bleeding
- Ruling out other conditions
- Addressing any contributing factors
- Maintaining good hydration
- Following up with both an EDS specialist and a urologist if hematuria persists or recurs. It is essential to note that the latest study on the management of adult patients with severe chronic small intestinal dysmotility highlights the importance of considering hEDS in patients with gastrointestinal associations, as it can contribute to dysmotility and other complications 1. However, the study on the AUA/SUFU guideline on adult neurogenic lower urinary tract dysfunction provides more relevant guidance on the management of hematuria in patients with NLUTD, emphasizing the need for individualized assessment and treatment 1.
From the Research
Hypermobile Ehlers-Danlos Syndrome (hEDS) and Hematuria
- hEDS is a genetic disorder characterized by hyperflexible joints, hyperextensible skin, and a tendency for easy bruising and tissue injuries 2.
- A case report of a 13-year-old girl with hEDS presented with microscopic hematuria, among other symptoms, highlighting the complexity of diagnosing and managing hEDS with multisystem involvement 2.
- While the study does not explicitly state that hEDS causes hematuria, it suggests that hEDS can be associated with various symptoms, including hematuria, due to the fragility of connective tissues and blood vessels 2, 3.
- Another study reported a case of hemarthrosis complicated by severe anemia related to Ehlers-Danlos syndrome, highlighting the risk of bleeding and hematuria in patients with EDS 4.
- A study on hemostatic abnormalities in patients with EDS found that nearly half of patients with EDS have an abnormal bleeding assessment score, which may contribute to the risk of bleeding, including hematuria 5.
Mechanisms and Associations
- The vascular aspects of EDS, including vascular fragility and bleeding tendency, are attributed to defects in collagen and other constituents of the extracellular matrix 3.
- Easy bruising and bleeding tendency are common in all subtypes of EDS, including hEDS, and may manifest as gum bleeding, menometrorrhagia, or postnatal or peri-operative hemorrhage 3.
- The risk of bleeding, including hematuria, may be increased in patients with hEDS due to platelet function abnormalities and other hemostatic abnormalities 5.