MCTD and Polymyositis

Viewers Question:   I have been diagnosed with MCTD & polymyositis for 7 months with an inital CPK reading of >2100, subsequently going down to current level of 268 on a dosage of prednisone beginning at 40mg/daily to a current dose of 20 mg/daily. We tried to go down to 15 mg/daily and found the CPK going back up. My dr. wants to try methotrexate. What is your opinion? And, where is the best place in the US to receive treatment for these diseases? What other things, including diet and/or herbal remedies can be tried and/or should be avoided?

Doctors Answer:   My opinion is that only your treating doctor can give you advise on the best options for your particular situation.

Polymyositis is a multifaceted disease and its optimal treatment depends on both the manner in which it affects an individual and the individual's underlying condition.

Methotrexate is now commonly used to treat progressive polymyositis in conjunction with prednisone and is a reasonable option for many.

Community rheumatologists are typically well versed in the use of these and other medications for the treatment of polymyositis. Second opinions could be sought at the rheumatology department of the nearest university medical school.

Patients with polymyositis are often encouraged to incorporate high protein content into their diets. There are no herbal remedies that have been shown to be either effective or safe in the treatment of polymyositis.

18 yr Old Boy with Dermatomyositis

Dr. Shiel's Perspective: This was only a single case report, but it was an impressive one. The patient, an 18 year old boy, had had dermatomyositis since age 11 years. He was becoming more and more seriously ill despite powerful treatments with cyclophosphamide (Cytoxan), methotrexate, intravenous immunoglobulin, plasmapheresis, and steroids. He was unable to move out of bed.

After starting Remicade intravenous infusions, he gradually became stronger, his muscle enzyme tests normalized after being elevated in the thousands, and he eventually walked again. Remicade's action in blocking TNF-alpha is probably the reason he improved.

Dermatomyositis has been associated with elevated levels of TNF-alpha in the blood. Remicade was reported by a number of groups of researchers as a beneficial treatment of a serious form of eye inflammation, uveitis.

Dr. Shiel's Perspective: This interesting use of Remicade will likely slip into the arsenal of doctors treating uveitis, which is a horrible, painful cause of blindness. I have recently had the occasion to witness this result first hand in my practice. I had a man come to me with a history of uveitis due to Behcet's syndrome. This patient nearly went blind while undergoing a litany of traditional therapies for his eye until a university professor started him on Remicade infusions. He was able to discontinue all other medications and he remarkably stabilized.

ENBREL (etanercept) Enbrel is an injectable anti-tumor necrosis factor for treating rheumatoid arthritis. Tumor necrosis factor (TNF) is a protein that the body produces during the inflammatory response, which is the body's reaction to injury. TNF promotes the inflammation and its associated fever and signs (pain, tenderness, and swelling) in several inflammatory conditions, including rheumatoid arthritis.

Enbrel is a synthetic (man-made) protein that binds to TNF. Enbrel thereby acts like a sponge to remove most of the TNF molecules from the joints and blood. This prevents TNF from promoting inflammation and the fever, pain, tenderness, and swelling of joints in patients with rheumatoid arthritis (and apparently other forms of inflammatory arthritis, such as psoriatic arthritis, ankylosing spondylitis, and juvenile arthritis-see below).

Enbrel plus methotrexate was reported at this meeting to be more effective than Enbrel alone.

Dr. Shiel's Perspective: This may be true, to a degree, for both Enbrel and Remicade, but I use them individually or with methotrexate or other drugs on a case by case basis.

The long-term safety of Enbrel in more than 2,600 patients over 5 years was reported. There was no cumulative toxicity with extended use and the clinical response was sustained for up to 5 years of the study.

Dr. Shiel's Perspective: These long-term statistics are very helpful for doctors who are assessing the value of this relatively new treatment for rheumatoid arthritis.

Enbrel was found to be safe when used in combination with Kineret (see below).

Dr. Shiel's Perspective: A combination therapy using more than one antirheumatic drug is commonly necessary in the treatment of certain patients with rheumatoid arthritis. This combination awaits further studies of Kineret before it is used commercially.

ADALIMUMAB

This is a new drug that is not commercially available. Adalimumab (D2E7) is the first fully human, monoclonal antibody in development. Adalimumab is an investigational agent designed to block the activity of tumor necrosis factor alpha (TNF-a), which contributes to the inflammation in autoimmune diseases, such as rheumatoid arthritis. This drug, which is given by subcutaneous injection every 2 weeks, was effective in combination with methotrexate and seems to be well tolerated other than occasional injection site reactions. It is not yet commercially available, but is worth keeping an eye on.

TRENTAL (pentoxifylline)

Mouth and genital ulcers in patients with Behcet's disease healed and were reported as less frequent in 9 or 12 patients who were treated with Trental (pentoxifylline).

Dr. Shiel's Perspective: Trental also seemed to maintain the healed ulcers for up to the 29 months of the study. The effectiveness of Trental, the researchers said, seemed to be enhanced by the combination with colchicine in some patients. I felt this paper was significant because, to date, colchicine has been the mainstay of treatment of these often terribly painful sores. Now, it appears that we have other options.

Macro CPK

Oversized muscle enzymes (Macro CPKs) can be a cause of elevated blood enzyme levels.

Dr. Shiel's Perspective: This report is interesting for doctors. We check CPK blood levels when evaluating the heart, muscles, and brain because the enzyme can come from each of these areas in a characteristic form.

Sometimes, rheumatologists are asked to evaluate patients with elevated CPKs who do not have any noticeable problems with their heart, muscles, or brain. This report from the national meeting explains a new test for a special form of CPK that stays elevated in the blood because it is bound by an antibody (immunoglobulin).

Because the evaluation of CPKs can involve a muscle biopsy, the detection of a Macro CPK could spare a patient from unnecessary testing. The researchers told me that the test must be ordered as a "macro CPK level by gel filtration on sephacryl." I will be giving this a try in patients with elevated CPK levels who don't have muscle weakness.