Some people have symptoms of lupus but don’t meet the criteria for a diagnosis. There are several names for this. In the past, it was called borderline lupus. Other terms include incomplete lupus, pre-lupus, forme fruste lupus (a French term), evolving lupus, undifferentiated connective tissue disease, and latent lupus. Many doctors use these terms to describe the same condition, and members of MyLupusTeam have used them as well.

If your rheumatology provider has used any of these terms to describe your condition, it’s important to understand what they mean. Here’s what you need to know about this diagnosis so you can make informed decisions and take care of your health.

How Is Lupus Diagnosed?

To understand borderline or incomplete lupus, it helps to know how lupus is diagnosed. The process can be complicated, and the criteria for diagnosis have been updated several times since 1982.

A lupus diagnosis means having multiple symptoms that affect different parts of your body, including various organs and systems. First, you must have a blood test that shows high levels of certain antibodies, called antinuclear antibodies (ANAs). A positive ANA test result is required to be considered for a lupus diagnosis. If your test result is negative, lupus can be ruled out. If your ANA test shows a positive result, your doctor will check for other symptoms. Each symptom is given a certain number of points. If your total adds up to 10 or more, you may be diagnosed with lupus.

The process is complicated. Symptoms are grouped into categories based on the organ or system they affect, and you must have at least one symptom from certain categories to be diagnosed.

Most of the time, doctors will need to run multiple laboratory and imaging tests to look for these different symptoms. These may include:

• Multiple blood tests
• Skin biopsies
• Kidney tests
• Chest X-ray
• Ultrasound of the heart or abdomen to detect serositis (a type of inflammation)
• Urine samples

This process can take a while. It’s important to follow up so you can find out if you qualify for a lupus diagnosis and understand how it may be affecting your body.

What Is Incomplete Lupus?

If you have some symptoms that match the criteria for lupus, but not enough for a diagnosis of lupus, your rheumatologist may call it incomplete lupus or use one of the other terms mentioned earlier.

Doctors used to use these terms to describe people in the early stages of a connective tissue disease, which affects the tissues that support and connect different parts of the body, like joints, skin, and organs. They believed these people would likely develop symptoms of an autoimmune disease, a condition where the immune system mistakenly attacks healthy cells, including connective tissues.

However, doctors noticed that many people with these symptoms didn’t fall into any existing disease categories. They were never diagnosed with lupus, rheumatoid arthritis, or any other connective tissue disease with clear, specific symptoms.

Doctors also noticed that many of these people had unusual sets of symptoms. Some symptoms were consistent with lupus. Others were consistent with other connective tissue diseases. These findings have led some healthcare providers to believe that undifferentiated connective tissue disease may be its own condition, at least for some people.

Up to 25 percent of people with connective tissue disease symptoms are diagnosed with undifferentiated connective tissue disease by their doctors. This doesn’t mean doctors don’t understand what’s happening. It just means the person’s symptoms don’t fit clearly into a specific diagnosis, like lupus.

Will an Undifferentiated Diagnosis Develop Into Classical Lupus?

Some people will continue to have an incomplete or undifferentiated diagnosis while others may develop additional symptoms that lead to a more specific diagnosis. Their diagnosis will then change to systemic lupus erythematosus (SLE) or another specific connective tissue disease.

Some MyLupusTeam members have experienced having an undifferentiated diagnosis progress to SLE. One shared, “On Friday, I finally got a diagnosis of lupus. I have been borderline for two years and have probably had it since I was in my 40s.”

Others haven’t progressed to a lupus diagnosis. One member said, “I have had borderline lupus for over 30 years,” and another added, “I have had borderline lupus for almost 50 years.”

Certain factors may increase the chances of developing SLE after an undifferentiated diagnosis. However, it’s important to note that these factors may not yet be useful for doctors. Right now, the data is limited. More research is necessary to confirm if and how these factors are linked to disease progression.

Age

The first risk factor is being diagnosed at a younger age. Research doesn’t yet show a specific age that increases the risk of developing SLE, but the risk may be higher for those diagnosed at a younger age.

Serositis

Another risk factor is serositis, which is inflammation of the tissues surrounding your organs and body cavities. People with this symptom are more likely to develop SLE than those without it.

Antibodies

Studies suggest that a specific antibody may help predict who will develop SLE. This is called the anti-double stranded DNA antibody (anti-dsDNA). If you have this in your blood, you may be more likely to develop SLE from an unclear diagnosis. Other antibodies, such as anti-Smith antibody (anti-Sm), or anti-Ro, may predict who develops SLE but with lower certainty than anti-dsDNA.

Other Factors

Certain factors have less strong evidence connecting them to disease progression. These include kidney disease, butterfly rash, sensitivity to light, and alopecia (hair loss). More research is needed to understand how these factors help predict the progression to a confirmed lupus diagnosis.

Talk to Your Doctor

If you’ve been diagnosed with undifferentiated connective tissue disease, emerging lupus, or a similar condition, ask your rheumatologist what it means for you. You can ask what needs to happen for a diagnosis of lupus or which symptoms they do and don’t see in your test results.

These conversations can help you know your body better. They can also help you get the best treatment for your needs. Your rheumatology team should still work to help you feel better even if your symptoms don’t qualify for a lupus diagnosis right now. You should be able to find treatments that improve your quality of life and overall sense of well-being.

Find Your Team

MyLupusTeam is the social network for people with lupus and their loved ones. On MyLupusTeam, more than 235,000 members come together to ask questions, give advice, and share their stories with others who understand life with lupus.

Are you living with some lupus symptoms but not enough to get a diagnosis? What has that been like for you? Share your experience in the comments below, start a conversation by posting on your Activities page, or connect with like-minded members in Groups.