Mixed connective tissue disease is a progressive disease and symptoms in most of the patients worsen with time. Management strategies of this condition are available, but the disease also causes mortality due to various complications. Risk factors include age, gender, family history and other autoimmune diseases in the patient.

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Is Mixed Connective Tissue Disease Fatal?

Mixed connective tissue disease is a life-threatening condition and the risk of fatality further increases when the symptoms are more of systemic sclerosis or polymyositis. The fatality may be due to pulmonary arterial hypertension, thrombocytopenic purpura, cardiovascular conditions, and systemic infection. Even the treatment to delay the condition is available but the disease is progressive, and the symptoms worsen with time.

A large number of people have their symptoms worsens in a period of 6 to 12 years. Although various complications are caused due to mixed connective tissue disease, a large number of fatalities occurred due to pulmonary arterial hypertension. The presence of the marker i.e. Immunoglobulin G (IgG) anticardiolipin indicates the presence of pulmonary hypertension. The risk of cardiac complications further increased due to the presence of antiphospholipid antibodies. Pericarditis is the most frequently occurring cardiac complication in mixed connective tissue disease.

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The patient who dies also suffers from other complications such as esophageal hypomotility, malignancy, and serositis. It has been found that this disease is more common in women than in men. It is also to be noted that mixed connective tissue for not reduce the life expectancy except in cases where the condition has complications such as pulmonary hypertension, or heart disease.

Mixed Connective Tissue

Mixed connective tissue is a rare, chronic autoimmune disorder whose symptoms overlap with the three diseases i.e. systemic sclerosis, Systemic Lupus Erythematosus, and

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Polymyositis. Thus this condition is also known as overlap syndrome. The condition which is supposed to be causing the disease includes hyperactive immune response, T-lymphocyte activation, vascular endothelial proliferation and hyperactivity of Beta-lymphocyte. Some patients also develop anti-Sm auto-antibodies within a few years after the diagnosis.

These patients are more prone to developing interstitial lung disease and the risk of developing skin sclerosis decreased in these patients. U1 small nuclear ribonucleoprotein 70 kDa is a protein present in the human. Antibodies against the fragments of this protein are the primary cause of mixed connective tissue disease. Majority of the patient suffering from this condition develops Raynaud’s phenomenon. The symptoms of this phenomenon are excessive reactions to cold and the fingers of the patient become blue or purple. Other symptoms of this mixed connective tissue disease include fatigue, fever, weakness, joint pain, and skin disorders. The severity of the symptoms varies from patient to patient.

Risk Factor Of Mixed Connective Tissue Disease

Various factors increase the risk of mixed connective tissue disease. These factors include:

Gender. Women are significantly at higher risk for developing mixed connective tissue disease. The ratio of occurrence of this condition in women and men is 3.1 to 16.1.

Age. Although mixed connective tissue disease can occur at any age, generally the people at the age range of 15 years to 25 years are at higher risk.

Family History. Although there is no conclusive evidence to show that people with a family history are more prone to develop mixed connective tissue disease, various researches indicates that there might be a genetic component involved as this disease commonly found in those people who have a family history of this disease.

Any Other Autoimmune Disease. Mixed connective tissue disease is a chronic, rare autoimmune disease. Autoimmune disease is the condition in which the immune system of the body starts damaging its own cells. Thus, the person suffering from any other autoimmune disease such as hypothyroidism is at higher risk for developing this condition.

Conclusion

Various complications occur due to mixed connective tissue disease. This may lead to fatal implications and death of the patient may occur. Pulmonary arterial hypertension is the primary cause of death. Pericarditis is the leading cause of deaths due to cardiovascular complications. The patient may also die from systemic infections.

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Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: February 7, 2019

This article does not provide medical advice. See disclaimer

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