Mixed connective tissue disease is a rare type of auto-immune disorder where the body’s defense mechanism attacks its own cells. It has characteristics of three different connective tissue disorders that include systemic lupus erythematosus or SLE, scleroderma/systemic sclerosis and polymyositis.

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What Is The Prognosis For Mixed Connective Tissue Disease?

It most commonly occurs in women in the age group of 20 to 30 years. In some cases features of rheumatoid arthritis might also be present. In the long run the prognosis for most people with mixed connective tissue disease is good. Although the prognosis for the patient depends on the signs and symptoms experienced by them, in about 80% of the patients a 10-year survival rate is seen for the disease. Some people can also remain symptom free for a couple of years even without treatment. However in 13% of the cases the disease can worsen over time and lead to fatal complications in about a time-span of 6 to 12 years. People who have systemic sclerosis/scleroderma and polymyositis have shown the worse prognosis. The most common cause of death in patients of mixed connective tissue disease has found to be pulmonary hypertension.

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Diagnosis And Management Of Mixed Connective Tissue Disease

The diagnosis is made on the basis of the history suggestive of connective tissue disease and a physical examination will show positive signs associated with the disease. A blood test will confirm the diagnosis which shows the presence of antibodies found in mixed connective tissue disease.

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As for the treatment of mixed connective tissue disease goes medications can only control the symptoms and not completely cure the disease. NSAIDs are given to suppress the inflammation of the muscles and joints and also help relieve pain. When there is involvement of the lungs and heart glucocorticoids may be prescribed to manage the infection. In some severe cases immunosuppressant drugs might be required for a long period of time. Since different people present with different complaints the management is planned accordingly depending upon the signs and symptoms.

The final outlook for mixed connective tissue disease has shown different outcomes in different patients. With proper care and treatment a person can survive 10 to 12 years after the diagnosis has been made in about three-fourths of the cases while the rest have shown a decline in health even with treatment.

Causes And Symptoms Of Mixed Connective Tissue Disease

The exact cause of mixed connective tissue disease still remains unknown. Studies have shown genetic predisposition but why the immune system attacks its own body cells still remains unclear.

The symptoms usually include features of other connective tissue disorders such as rheumatoid arthritis, scleroderma, polymyositis and SLE (systemic lupus erythematosus). The very first symptom that develops either months or years before other symptoms is the Raynaud’s phenomenon where the blood vessels in your hands and feet temporarily constrict to reduce blood flow to them. This occurs as a result of compensatory mechanism to conserve heat in the body in times of stress and cold. It is not a serious health problem in most people but it can cause major damage in others. It is followed by pain or inflammation of the joints, weakness in muscles, episodes of fever and tendency to get tired easily. There can be edema of the hands due to collection of fluid in the tissues leading to swelling. Skin manifestations include reddish brown patches over the skin and knuckles, purple eyelids, alopecia or hair loss and telangiectasia around the fingernails where the small blood vessels in the fingers dilate especially around the nails (periungal). There can be decreased motility of the esophagus, difficulty in breathing, increase in pulmonary blood pressure, and inflammation of the muscular layers of the heart (pericarditis and myocarditis). Other symptoms include diseased kidney, anemia and decrease in white blood cell count along with lymph node enlargement, increase in size of liver and spleen. Neurological manifestations include headaches mostly caused due to vasoconstriction, seizures, cerebral hemorrhage or thrombosis and neuropathies in different areas of the body.

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Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: February 12, 2019

This article does not provide medical advice. See disclaimer

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