MyLupusTeam members have asked questions about whether there is a connection between lupus and Ehler-Danlos syndrome (EDS). “Does someone else here have Ehler-Danlos or similar connective tissue disease?” one team member asked. “Hi all, any other lupus/EDS zebras out there?” another member wrote.

Lupus (also called systemic lupus erythematosus, or SLE) and EDS are two types of connective tissue disorders that can affect skin, muscles, joints, and organs. Some people with lupus also have EDS, and sometimes symptoms can be difficult to differentiate between the two conditions.

What Is Ehlers-Danlos Syndrome?

EDS is an inherited condition — or genetic disorder — that disrupts the formation and function of collagen, which makes up 30 percent of the body’s protein. Collagen is needed to support and strengthen the body’s musculoskeletal structure, including muscles, bones, ligaments, and tendons, as well as other connective tissues that are needed for the vascular system (blood vessels), organs, intestines, and skin to function properly.

There are several subtypes of EDS. The most common type of EDS — hypermobile EDS (hEDS) — is characterized by joint hypermobility (loose joints) and stretchy and fragile skin. People with this type of EDS have a high risk of joint dislocation. EDS can also cause organs and blood vessels to rupture due to abnormalities in connective tissue. Occurring in approximately 1 out of 10,000 to 15,000 people, hEDS is believed to be present in a small portion of the population. Other types of EDS have a much lower prevalence (how common a specific condition is within a population) and are considered very rare.

Unlike EDS, lupus is an autoimmune disease in which antibodies in the immune system mistakenly attack healthy tissue. Both EDS and SLE, the most common type of lupus, can affect various parts of the body and have similar signs and symptoms that can be difficult to tell apart. Common symptoms for both conditions include painful joints and fatigue, among a variety of other symptoms that are different in everyone.

“EDS is genetic. I got it from my mom’s side of the family, and I passed it on to both my kids,” a MyLupusTeam member wrote. “What I thought were EDS exacerbations turned out to be lupus.”

Connections Between Lupus and Ehlers-Danlos Syndrome

Although the causes of lupus and EDS are different, research indicates that some rheumatic and autoimmune conditions such as rheumatoid arthritis or lupus were more common in people with hEDS than in the general population. Furthermore, both conditions occur more frequently in women.

One MyLupusTeam member wrote, “I have EDS and was just informed recently that I have UCTD (undifferentiated connective tissue disease), which is similar. I was told connective tissue diseases can overlap, and I am thinking it is much more common than what is currently being identified.”

Although people with EDS are more likely to have inflammatory conditions like lupus, we have a limited understanding of the reasons why this risk is higher. Having EDS with lupus may increase the risk of complications, such as joint dislocation, bleeding due to weakened skin, and in severe cases, rupture of blood vessels or organs, which can be a life-threatening condition.

The Importance of an Accurate Diagnosis

Although some symptoms of EDS and lupus can be similar, there are distinctive features of EDS that can help provide an accurate diagnosis. Loose joints, delicate skin, and stretchy skin are important characteristics of EDS that help identify the condition. Although EDS is a genetic disorder, there is not currently a genetic test for hEDS. However, other more rare subtypes of EDS can be diagnosed through genetic testing.

“The good: My rheumatologist doesn’t think I have lupus, but will be running more lab tests. The bad: He diagnosed me with Ehlers-Danlos syndrome and fibromyalgia,” a MyLupusTeam member wrote.

Having a correct diagnosis of either EDS or lupus is crucial to ensure that the right treatment is given. This accurate diagnosis can help control EDS symptoms or manage symptoms and slow down the progression of lupus.

EDS is typically managed using over-the-counter (OTC) pain relievers like ibuprofen (Advil, Motrin IB, etc.), acetaminophen (Tylenol, etc.), or naproxen sodium (Aleve). Blood pressure medication may also be prescribed to reduce stress on blood vessels, and physical therapy is recommended to help prevent hypermobile joint dislocation.

Treatment Challenges When Both Conditions Are Present

For people who have both EDS and lupus, it can be challenging to treat symptoms that can occur with either condition. Other comorbidities with lupus — coinciding health conditions — that cause symptoms such as joint pain, chronic pain, or chronic fatigue, can further complicate treatment.

One MyLupusTeam member shared their frustration. “Having comorbid Ehlers-Danlos syndrome with borderline lupus is hard because I don’t always know which one is causing the issues. My neck pain is constant.”

Another member wrote, “Well, can anyone tell when you are having pain whether it is from arthritis, fibromyalgia, Ehlers-Danlos, Sjögren’s, Lyme disease, Baastrup’s disease, lupus, or spinal stenosis, etc.? Sometimes, I can’t figure out which thing, or all that is the cause.”

Maintaining your treatment plans and engaging in open communication with your health care team are essential for your medical care.

When symptoms are confusing, it’s important to give your doctor a detailed description of what you are experiencing. You may want to write down your symptoms to be sure you are giving your doctor a full report of symptoms that can be further evaluated. It can take patience and time to determine appropriate treatment options for conditions with similar symptoms such as lupus and EDS.

Find Your Team

MyLupusTeam is the social network for people with lupus and their loved ones. On MyLupusTeam, more than 223,000 members come together to ask questions, give advice, and share their stories with others who understand life with lupus.

Have you been diagnosed with Ehlers-Danlos syndrome? Share your experience in the comments below, or start a conversation by posting on your Activities page.