CENTRAL  SOUTH  DAKOTA  MEDICAL NEWS
THE CLINICAL VIEW
By: Phillip E. Hoffsten, M.D.
11/2/2000

What is Autoimmunity?

I recently saw a lady from Denver, Colorado in consultation. She was visiting her sister in Pierre and wanted another opinion about her illness.

She was 28 years old and married with two children ages 4 and 6 and worked as a secretary for a large corporation in Denver. She had been healthy with no significant medical attention throughout her entire life until the last three months. Then, in the early part of the summer, she went to a weekend family gathering where she was exposed to the sun a great deal and developed a mild sunburn. Over the next 10 days, she became progressively sicker with fevers, nausea, weakness, fatigue and aching in multiple joints especially the hands, feet, wrists and ankles. She developed swelling and fluid retention gaining 8 pounds and noticed that she put out a decreased amount of urine compared to her normal routine. One morning, she awakened so ill that she could not go to work and sought medical attention at that time. The doctor who saw her in Denver diagnosed a disease called systemic lupus erythematosus, an autoimmune disease. She was started on high doses of cortisone and several other drugs that suppress the immune system in the body. In the course of several weeks, her symptoms reversed and her doctor told her that she needed to stay on medications to prevent a recurrence of this condition. The cortisone was making her face round, she was gaining weight and her blood pressure was difficult to control. These were all side effects of the cortisone treatment.  She wanted to know what autoimmunity was, what was systemic lupus erythematosus, did she have this condition, and did she need to continue to take this horrible medicine with all of its horrible side effects. She said she was feeling much better now and wondered why she could not stop the cortisone.

I reviewed her history and obtained copies of the records from Denver. It is amazing how quickly a fax machine works these days. Instead of waiting for the mail, we had copies of her records transmitted from Denver in less than 30 minutes. On reviewing the records, it was very obvious that she did have systemic lupus erythematosus, and I told her that her doctor was very astute for having made the correct diagnosis and providing the correct treatment. Making the diagnosis requires a recognition of the person's symptoms and signs and then putting this together with the appropriate laboratory examinations to arrive at the proper diagnosis. Her doctor had done that very skillfully.

I next explained to her what autoimmunity meant. In the 1950s, two research doctors working in Australia and England received the Nobel Prize in medicine for their work in this area. It was well known for years before that skin taken from one person and transplanted to another will be rejected by the receiving person in about 7 to 10 days. If this same procedure is done when the individual is still in the womb or just born, skin can be transferred from one individual to another and the receiving individual accept the skin as their own. In other words, at about the time of birth, there is a grand inventory taken by the body. All of the things that are self are recognized and protected throughout the person's life. The body has an immune system equipped to recognize things that are not self and reject them. Examples would be pneumonia, ragweed allergy, many different kinds of infections, and tissue that might be transplanted from another individual. Somehow, the body is equipped with a beautiful system that recognizes what is part of you and what is not part of you. The immune system then rejects those things that are not part of you.

But, as with all systems in this world, nothing is perfect and things can break. What happens if the body's surveillance system to recognize the self breaks down? What would happen if the body lost its ability to recognize the thyroid gland or the skin or the heart or the brain or the liver or the kidney or any one of a host of other organs as part of the self?

The answer to that question is that the body then begins to treat the unrecognized part as if it is foreign and attacks it in the same way it would attack a pneumonia or any other invading infection. When this attack involves a vital organ such as the kidneys or the skin, significant medical illness with failure of vital organ systems can occur. This is what happened to the lady whom I saw in consultation. She developed an autoimmune response to her own kidneys and was destroying her kidneys as though they were a foreign agent.

Now for the bad news. The body's immune system is more or less permanent. You get measles one time but then you become immune to it for the rest of your life. The same is true for polio and many other infections. The immunity may diminish some with time but a booster shot will bring the immunity back up to a high level again very quickly, and unfortunately the same is true for autoimmunity except the person provides their own booster shot everyday and their own autoimmunity tends to grow if left unchecked. The lady with systemic lupus erythematosus had her autoimmunity diminished by the medicine she was receiving. But, if she stopped taking the medication, her kidneys were still in her body, they were still perceived by her body as foreign and the autoimmune response for her to kill her own kidneys would come again and she would get sick all over again if she stopped taking her medications. Unfortunately, autoimmune diseases tend to be a permanent condition and need to be cared for and treated throughout the person's life. As you might imagine, this was not music to the ears of the lady who was consulting me but I thanked her for making me a consultant. Usually, people go from Pierre to Denver because a doctor 100 miles away must know more than the local doctors. I guess she felt the same but lived in Denver so she came to Pierre for a second opinion.  Unfortunately, we do not have effective medications to treat autoimmune diseases without bothersome side effects. For the most part, however, the side effects of the medication are probably more acceptable than the disease process itself.

Research on better medications and better understanding of autoimmune diseases is intense at this time. If there is anything beneficial about the AIDS epidemic, it is that research into immunity has been intensely stimulated. Better treatments for autoimmune disease and better understanding of the mechanisms of these diseases can be expected in the future. For the time being, cortisone and several other medications are the only things that we have.