The Connection Between Lupus and NMOSD

People with lupus can have other autoimmune diseases. One or more other autoimmune diseases appeared in 30 percent of people with lupus in a study published in 2000.1 Further research in recent years has shown that neuromyelitis optica spectrum disorder (NMOSD) may occur more often in people with lupus.2-5

What is NMOSD?

NMOSD, known in the past as Devic’s disease, is an autoimmune disease that affects the central nervous system (CNS). The immune system makes proteins that attack astrocytes: certain cells in the brain, optic nerves, and spinal cord. These defensive proteins are called anti-aquaporin-4 antibodies (AQP4-IgG).2-5

NMOSD does not have 1 clear cause, but many factors can play a part in such autoimmune diseases taking form. These include a person’s diet, genes, hormones, and stress. What is around people and what happens to them can also impact the immune system.1,2

What are the signs and symptoms?

An inflamed optic nerve (optic neuritis) and an inflamed spinal cord (myelitis) can signal NMOSD. With optic neuritis, the eye(s) may hurt, and you may not see clearly. With myelitis, a person may not be able to restrain their bladder and bowels, move their leg(s), and use other senses. Myelitis may bring pain in the arms, back, head, legs, and spine as well as muscle spasms and a rigid neck.2,3,5

Some people with NMOSD may have extra signs such as weakness. Having hiccups, a queasy stomach, and the need to throw up may occur if the brainstem becomes inflamed. NMOSD can cause brain damage and swelling and even lead to loss of movement and sight.2,3

NMOSD shares symptoms of other autoimmune diseases. NMOSD can appear to be lupus myelitis and multiple sclerosis (MS). As with most autoimmune diseases, the symptoms of NMOSD can come and go over time.2,3,5

Certain antibodies set NMOSD apart from diseases like MS. AQP4-IgG points to NMOSD. A form of NMOSD without AQP4-IgG exists and instead has myelin oligodendrocyte glycoprotein antibodies (MOG-IgG).2,3,5

Who is at an increased risk?

NMOSD affects men and women of all ages and races. Women in their late 50s and early 60s get it most. More white people have NMOSD than people of other races.3

Up to 10 out of every 100,000 people get NMOSD.3 Doctors do not know how many of these people have lupus as well.3,4

NMOSD tends to occur in people with a personal or family record of autoimmune diseases. Half of the people with NMOSD either have a different autoimmune disease or a family member who has one. (NMOSD is rarely the disease present in that family member.)3

How is NMOSD linked to lupus?

NMOSD and lupus can occur together. Myelitis and optic neuritis are symptoms of both diseases.2-5 Often what is termed “lupus myelitis” is really NMOSD.3

The main link between lupus and NMOSD is how the immune system overacts in response to something in the body. People who are prone to autoimmune diseases are more likely to get NMOSD. NMOSD exists in people with lupus as well as diseases like celiac and myasthenia gravis that do not affect the CNS.2,5

Lupus may increase the chance of a person getting NMOSD. The 2000 study noted above found that 78 percent of people with lupus and at least 1 other autoimmune disease had lupus first. This study also showed that lupus arose at a younger age in those who also had another autoimmune disease.1

What are treatments for NMOSD?

NMOSD is a separate disease that requires special treatment. Eculizumab, inebilizumab, and satralizumab are novel treatments for NMOSD. Doctors may order drugs to reduce spasms and relax tense muscles. They may also prescribe corticosteroids to calm inflamed tissues. If that does not work, they may suggest a plasma exchange to cleanse the blood of antibodies.2-5

Lupus and NMOSD share some treatment options. These include drugs to lower inflammation and curb the immune system. Azathioprine and rituximab are commonly used to treat both diseases and prevent symptoms from growing worse.2,3,5

While some treatment options for lupus and NMOSD may be the same, some differ. Treatment also gets more complex when both diseases occur in 1 person. For instance, doctors will not use certain treatments for lupus that could bother the nervous system.2,5

If you have lupus, talk to your doctor about NMOSD. He or she can assess your current state and health history. Your doctor can order blood tests, spinal tests, and x-rays to confirm the findings. The sooner NMOSD is detected, the sooner the right treatment can begin – which can lead to better results.2-5

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