I’m Okay…My Biggest Lie

I read an article recently on my primary condition, Mixed Connective Tissue Disease (MCTD or Sharp’s Syndrome) and found it particularly disturbing.  I am always looking for new information on MCTD as it is a rare autoimmune disease with little to no research done for it, so it’s not likely to make front page news if a breakthrough is made.  Instead of finding a new treatment or therapy that could potentially help, I found this…

Mixed connective tissue disease is an autoimmune disease that was first characterized in 1972. It is also called Sharp’s syndrome and is commonly referred to in its acronym, MCTD. As the name suggests, the disease is a combination of several disorders that affect various connective tissues in the body. Lupus, scleroderma and polymyositis are the primary disorders whose symptoms affect individuals with MCTD. Like many autoimmune diseases, MTCD progresses over time. That is, some of the symptoms associated with the condition do not develop simultaneously but instead occur in sequence over a long period of time. Because of these traits, MTCD often prove difficult to diagnose, while treatment is limited to the management of symptoms and the extension of mixed connective tissue disease life expectancy.

As an autoimmune disease, MCTD is a degenerative disorder that is caused by the overactivity of the immune system. In this condition, like in any of its kind, the immune system mistakes healthy cells for foreign bodies that need to be destroyed. Thus, normal cells deteriorate and eventually die. The outlook of MCTD is generally worse than that of single connective tissue disorders, such as systemic lupus erythematosus. Even in the presence of prednisone treatment, the condition tends to continuously deteriorate the patient’s body. This is because MCTD is a progressive autoimmune disease that naturally develops into worse disorders or creates more complicated and fatal symptoms. One common condition met by patients with MCTD is progressive systemic sclerosis, which has a poor prognosis. 

Mixed connective tissue disease life expectancy is decidedly short, and death is inevitable in most cases. However, up to 30 percent of cases are mild, and there are individuals that experience remission. This is particularly true of patients with no anti-RNP antibody in their blood.

You can see the reason for being disturbed by this.  My rheumatologist and I have discussed MCTD but she has never mentioned that it can be a life threatening condition.  The article mentions that remission happens for 30% of patients and this is usually true for those without the anti-RNP antibodies, which unfortunately, I do have.  This is kind of a shock to me.  I have held out hope that one day, if I did everything right, I would go into remission.  I knew it wasn’t a cure, but remission could possibly give me some time with a little less pain.  Now, that hope is gone and I don’t know what to think anymore.  I was aware that MCTD was progressive, I just didn’t know that it was fatal.  I may have to just sit with this new information awhile before I can say, “I’m okay” again.