is myasthenia gravis a progressive disease?
 

I'm finding different answers on the internet, but first I need to clarify what I mean by the term "progressive." I think we have all experienced our symptoms getting worse. The question I'm asking is whether it keeps getting worse your whole life (without effective treatment, of course), or whether the symptoms reach a maximum severity after a certain amount of time.

"Maximum weakness occurs during the first year in two-thirds of patients. In the era before corticosteroids were used for treatment, approximately one-third of patients improved spontaneously, one-third became worse, and one-third died of the disease. Spontaneous improvement frequently occurred early in the course. Symptoms fluctuated over a relatively short period of time and then became progressively severe for several years (active stage). The active stage is followed by an inactive state in which fluctuations in strength still occurred but are attributable to fatigue, intercurrent illness, or other identifiable factors. After 15 to 20 years, weakness often becomes fixed and the most severely involved muscles are frequently atrophic (burnt-out stage)." source: http://www.myasthenia.org/HealthProf...rviewofMG.aspx

This is a different picture from ALS, for example, in which the symptoms progress until the patient dies.

Here's another one:

"Symptoms of myasthenia gravis usually progress to maximum severity within 3 years." source: http://www.mda.org.au/Disorders/NMJ/MG.asp

Here's another:

"In most patients, the severity of disease approaches and may attain a maximum during the first 1 to 3 years." source: Myasthenia Gravis and Myasthenic Disorders By Andrew Engel (I found this here: http://books.google.com/books?id=aKE...ity%22&f=false)]

If you look up the last quote, you'll see statistics about the size of the study, etc. I conclude that there is at least some evidence that unlike some other chronic neuromuscular diseases, myasthenia gravis often stabilizes after a certain amount of time, instead of continually progressing in severity.

Abby

I just want to clarify that by the word "stabilizes" above, I just mean "stays the same." I'm not sure if I used the word right.

Abby

Perfect timing on this. I think the term progressive is, to some extent, difficult to quantify. I, too, have read that it isn't progressive, but it often does get worse. I think that they mean over a long period of time that it doesn't progress, like, say ALS does (which you mentioned). Right now, I am progressing. I started 2 years ago with eye issues that I blew off. Last year that developed into eye issues that I could no longer ignore, plus progressive weakness through my body (arms, legs, hands), and some trouble with breathing that I & my doctors chalked up to asthma. Then here we are today. Another year later, with worsened symptoms all around and breathing that may land me in the ER. I've been battling it all weekend and my neostigmine is acting like a placebo & doing nothing. So yes, to some degree, I believe MG is progressive. But I guess in the big picture, no it isn't because it "typically" doesn't lead to total disability or death (sometimes it can, but not usually). Confused yet? ;)

I have noticed my general health seems to get worse at times. In particular my breathing. The last time (1year ago approx) I was admitted to hospital with breathing problems. My lung capacity was 35% and 65% when I was discharged.
When it is too hot my breathing gets worse as does other MG symptoms. I can't exercise or walk too far as I end up wiped out for several days. I often wonder if I didn't have MG would my health have gotton so bad. Who knows , at times my doctor puts my weakness down to MG. Its easy to blame MG when in my case it could be due to my refusal to slow down.

The answer is right there in your citation:

Why do you think those people died? because they had a stable disease?
I have not yet heard of someone dying of a stable disease.

Every autoimmune disease has a variable and many times unpredictable course.
For example: I have patients with immune thrombocytopenia who had a severe disease, requiring hospitalization and aggressive treatment, who after a few months fully recovered. I have others who had a very mild illness, never requiring treatment for many years, and yet others who had a very rocky course, achieved remission for a long period and then had recurrence of their illness, which was now much harder to control.

MG as opposed to thrombocytopenia is a disease that affects your function, and you eventually adjust to it. This may give the illusion of a stable or "burnt out" disease.

6 years ago, when I woke up in the morning hardly able to move, talk or breath I ended up in the ICU and told I was pre-intubation.
Now, when I wake up in the morning like that, I take my meds, and lie down with my respirator for a while. I then get up, and go to work, return home early so I can rest more. Use my respirator on and off for a few days, until I gradually get back to my previous base-line.

So, is my illness stable, burnt-out or did I just learn to live with it better?

Abby, I replied to you on a new thread. There is conflicting info out there, however, all autoimmune diseases by their very nature are progressive to some degree. At least from all of my reading and in my opinion. ;)

Seamus, You'd better learn how to scale things back or you're going to end up in an MG crisis!

Kage12, I'm totally disabled. I believe it's more common than you might think. A friend of mine is too. Another friend with MG can work but is on other meds than Mestinon. This disease is whack because there is no "alogrithm" for it.

Well said, Alice.

Even if it doesn't progress, it can sure mess up someone's life.

Annie

Using the right terminology will make it less confusing:

progressive= getting worse.
fatal= causing death.
Life threatening= may cause death, if not properly managed.
Placebo= makes you better, even if there is no known pharmacological agent involved.
Not responding to a known pharmacological agent that made you better before=dangerous.
total disability?-Which illness leads to "total disability". Would you define Steven Hawking as "totally disabled"? Ability and Disability are not the result of the illness alone, but the result of the way in which society helps people fulfill their abilities even if they have disabilities. (in some ways we all have abilities and disabilities, even healthy people).
Death-we will all die some day. I don't think there are clear answers as to how much MG or its treatment increases our risk of dying at an earlier age.
MG can definitely be a life-threatening or even fatal illness if not recognized and managed properly.
My personal opinion=If you have breathing difficulties that do not respond to neostigmine, it is time to go to the ER or at least get a proper evaluation by a pulmonologist who has a good understanding in neuromuscular diseases.