Myasthenia Gravis: What Remission Looks Like

By Richard J. Nowak, MD, as told to Keri Wiginton

Myasthenia gravis (MG) is a chronic autoimmune condition without a cure. But it’s treatable. And as long as you work with your doctor to optimize your treatment plan, which can be different from one person to the next, there’s a good chance you can live symptom-free or close to it.

What Is Remission?

This medical term gets thrown around loosely. But there are specific definitions for remission. You can read about each one by searching online for the Myasthenia Gravis Foundation of America (MGFA) post-intervention status. But I’ll break down the basics.

The first and hardest stage to achieve is complete stable remission. This means your doctor finds no evidence of disease activity or muscle weakness during a checkup, and you haven’t been on medical treatment for at least 1 year.

You might be symptom-free earlier than the 12-month mark, but you can’t technically call this remission based on the definition.

What’s a bit more common is something termed pharmacological remission. The same definition as complete stable remission applies here, except that you take medication or are on some form of therapy to control your MG symptoms.

Then there is also minimal manifestations status. We don’t consider this remission, but it’s the group that many people with MG fall into. It means the disease doesn’t affect your ability to get through daily life, but you may still have some muscle weakness or fatigue that comes and goes.

How Do You Get to Remission?

Your symptoms might go away on their own, but the chances of spontaneous remission are rare. What’s more likely is you’ll need some form of treatment that targets your immune system. You might need medication, surgery, or another therapy.

As a neurologist, I consider several things when deciding the best treatment for someone. That includes how serious your disease is, where you have muscle weakness, your age, what other health conditions you have, and what antibodies you test positive for.

Muscle-strengthening drugs like pyridostigmine may be enough to control mild symptoms that don’t bother you very much. But this isn’t a medication that targets the underlying cause of MG.

If you have significant active disease, regardless of exacerbation or crisis, you’re going to need more aggressive treatment to get your symptoms under control. And corticosteroids are usually the first thing we try. These are drugs like prednisone that calm down your immune system.

Steroids are often the go-to treatment for MG because they work faster — typically within days to weeks — than many other immunosuppressants. But they aren’t a long-term solution. That’s because they can cause unwanted side effects when used chronically, including weight gain, osteoporosis, and elevated blood sugars.

Once your symptoms are under control, you’ll likely switch to another medication so you can lower your dose of steroids or stop them.

For the next phase of your MG treatment plan, you may take other drugs that change how your immune system works. Commonly, this includes the immunosuppressants azathioprine or mycophenolate. These take about 6 to 12 months to work, but they come with fewer side effects than steroids.

If you have MuSK antibody-positive generalized MG, we tend to favor a medication called rituximab. This treatment works really well for folks in this group, and it’s not unusual for people to go into remission after two to four treatment cycles.

Your doctor may suggest rituximab as a treatment if you have acetylcholine receptor (AChR) antibody-positive generalized MG and other therapies haven’t worked. But the evidence is mixed on how well this drug works for this group.

We also have newer immunotherapies approved to treat AChR-associated MG. These include faster-acting drugs like C5 complement inhibitors and FcRn inhibitors.

What research shows is that the majority of people feel better on these newer medications, and a good number may go into remission. But they certainly don’t work for everyone, so it’s good to explore all options available.

Where Does Thymectomy Fit In?

There’s good evidence that removal of the thymus can not only lessen MG symptoms but also reduce the need for immunosuppressive therapies like steroids. This is true whether or not someone has a thymoma, or a tumor on their thymus.

If you’re early in your disease course, we do suggest an elective thymectomy for younger people with AChR antibody-positive generalized myasthenia gravis. This group typically includes folks who are in their 20s to early 50s.

Why don’t we routinely recommend thymectomy for older people? There isn’t good data to show that removal of the thymus gland will benefit those 65 and older. That’s likely because the thymus is quite large when you’re a child. And it shrinks and turns to fat little by little as you get older.

But you should always talk to your doctor about thymectomy if they don’t bring it up first. The surgery might be an option.

What Does Remission Look Like?

You may have absolutely no symptoms. And I do treat people with MG who get to remission and tell me they feel as if they don’t have the disease at all.

Sometimes there’s not complete recovery, but that doesn’t mean the disease is active. You may have elements of “fixed weakness.” This happens if you have longstanding disease and MG “scars” or causes permanent damage to the muscle surface. No amount of immunotherapy can fix this kind of damage.

For example, eyelid muscles are thin and easily injured. And you might always have a subtle or moderate eyelid droop if these muscles get hurt, even if you get to remission.

It’s also not uncommon for people with MG to also have other health problems. And if you have arthritis, for instance, you may continue to have pain, weakness, or mobility problems despite being in remission from a MG perspective.

What’s the Best Treatment for Remission?

We have quite a number of knowledge gaps in that area. We don’t have good markers for who might go into remission on certain drugs or who might have a partial response or even lack of response.

But your neurologist should give you all your choices and go over the possible risks and benefits of each treatment.

And it should be an open conversation. If your first treatments aren’t helping your condition and you’re experiencing a significant number of symptoms or you have exacerbations, then it’s a good idea to revisit your treatment plan with their neurologist or get a second opinion.

This is a chronic disease that will require close routine follow-up, regardless of MG status. Exacerbations can occur even after remission, especially if there are triggers like infection.

Photo Credit: Stone / Getty Images

SOURCE:

Richard J. Nowak, MD, director, Yale Myasthenia Gravis Clinic, Yale Medicine; assistant professor of neurology; Yale School of Medicine.

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