Connective Tissue Diseases (CTDs) : Symptom Management

by Food and Drug Administration (FDA)

(Page 3 of 3)

Just as the causes of CTDs are unknown, so are the cures. All CTDs are chronic, but people may have long periods of remission when they are symptom-free. For this reason, treatment focuses on symptom "management."

Since few people with CTDs suffer from all symptoms characteristic of a specific disorder, the treatment plan for each patient must be individualized. Because the drugs used to treat severe symptoms can have very serious side effects — such as the bone deterioration Kline developed from taking corticosteroids — physicians continuously weigh the benefits of treating symptoms against the risks of adverse drug effects (see accompanying article, "Distinguishing CTDs").

Looking Ahead

Studies are under way in medical centers around the country to test investigational therapies for CTDs. These include the use of certain orphan drugs and combinations of new immunosuppressive drugs, biologic agents (antibodies produced in a laboratory), plasmapheresis (removal of plasma from blood cells, and reinfusion), and photophoresis (use of a drug activated by ultraviolet light).

"The upside to all the media attention focused on CTDs lately is increased awareness among physicians and the public about the toll these diseases take on people's lives," says hematologist Anderson. She and others hope this will spur greater interest in exploring the causes that underlie these disorders and result in better ways to manage — and even cure — connective tissue diseases.

CTDs have some symptoms in common and others that are specific to each disorder. Here's a look at the most common symptoms of scleroderma, PM/DM and SLE and how they are treated.

Scleroderma: The most common early symptom of scleroderma is Raynaud's phenomenon (but not everyone who has Raynaud's phenomenon develops scleroderma). In Raynaud's phenomenon, the blood vessels of the hands and feet constrict in response to cold exposure, and the affected skin turns white, blue, then red. Vasodilators — drugs that relax and dilate blood vessels — are used to treat this condition.

Swelling of the hands or feet is managed with diuretics (drugs that help eliminate excess water from the body) or nonsteroidal anti-inflammatory drugs (NSAIDs).

There are no proven treatments as yet to treat or alter the course of fibrosis, the skin thickening that gives scleroderma its name. Experimental therapies, such as the use of penicillamine, a drug that interferes with collagen production, are being explored.

Skin sores must be cleansed to prevent bacterial infection, usually caused by staphylococcus bacteria. If infection occurs, it may be treated initially by soaking the affected area in warm water and applying an antiseptic such as hydrogen peroxide or Betadine (povidone-iodine solution). If infection continues, a broad-spectrum antibiotic such as erythromycin may be prescribed.

ACE inhibitors (a type of blood pressure medication) are frequently prescribed to treat kidney involvement and prevent kidney failure. A variety of experimental therapies are being used to treat involvement of other organs.

A wide range of over-the-counter and prescription medicines are used to treat less severe symptoms, such as dry, itchy skin and digestion problems.

Physical therapy may help early in the process to prevent joint contracture. Range of motion exercises can also help protect joints and keep them limber.

Polymyositis/Dermatomyositis (PM/DM): Muscle weakness — particularly in the shoulders, arms and legs — is the hallmark of polymyositis. When the muscle weakness is preceded or accompanied by a red rash on the nose, cheeks, knees, or knuckles and a purple coloring on the eyelids, the disorder is called dermatomyositis. The two are usually grouped together as PM/DM.

Corticosteroids are the mainstay of treatment, says rheumatologist Kramer. In severe cases, immunosuppressive drugs such as methotrexate or cyclophosphamide may be prescribed. Physical therapy can help prevent muscles from shrinking as they heal.

Systemic Lupus Erythematosus (SLE): Milder symptoms of SLE, such as joint pain, rashes, and mild pleurisy (inflammation of the lung lining) often respond to NSAIDs. Corticosteroids are used to treat more severe symptoms, such as kidney disease, and milder symptoms that don't respond to NSAIDs.

Antimalarial drugs may be prescribed to reduce joint swelling pain and rash, including the patchy, crusty red skin patches associated with this disorder. Immunosuppressive drugs may be used to treat patients with very severe symptoms, such as advanced kidney disease or central nervous system disease.

SLE patients are advised to avoid the sun, which can precipitate or aggravate a flare-up of the disease. Daily sunblock use is recommended, even for those patients who don't spend much time in the sun.

About the Author

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