Pulmonary Fibrosis

Simply put, pulmonary fibrosis is the formation of scar tissue in the lungs. The stiff, thick tissue makes breathing more difficult, and, as a result, the brain, heart and other organs don’t get the oxygen they need, leading to further complications. The definition for pulmonary fibrosis includes some 200 different pulmonary conditions, making it difficult for doctors to come up with firm numbers, but at least one study claims that pulmonary fibrosis affects about 200,000 people in the United States, with as many as 50,000 new cases diagnosed annually and as many as 40,000 deaths resulting primarily from pulmonary fibrosis and its complications. Pulmonary fibrosis is chronic and progressive, and there is currently no cure for the condition.

Symptoms and Complications

Like many diseases and conditions where lung damage is a factor, people with pulmonary fibrosis may show no symptoms until the disease has progressed and damage to the lung tissues is significant. Most people experience a gradual worsening of lung function with occasionally episodes of acute worsening. The most common symptom of pulmonary fibrosis is shortness of breath which becomes progressively worse over time. People with advanced pulmonary fibrosis may get breathless from minor physical activity, such as showering or getting dressed, and in the furthest advanced stages, may become short of breath from simply eating or talking. Other symptoms of pulmonary fibrosis include:

* Clubbing of the fingertips

* Fatigue and weakness

* Chronic hacking, dry cough

* Loss of appetite

* Unexplained weight loss

Complications of pulmonary fibrosis include increased respiratory infections and secondary organ failure due to lack of sufficient oxygen to the other organs of the body.

Risk Factors and Causes

There are a number of causes for pulmonary fibrosis, but a significant number of cases of the disease have no known cause. These are the main categories of causes and risk factors for pulmonary fibrosis:

* Idiopathic disorders: including pulmonary fibrosis with no known cause, and disorders with no known cause that cause lung scarring

* Connective tissue and autoimmune diseases: including scleroderma, lupus, rheumatoid arthritis and polymyositis

* Occupational and environmental factors: including inorganic dust, mold, gases and fumes and radiation that causes damage to the lungs

* Drug-induced: including damage from chemotherapy, radiation therapy, antibiotics, antiarrhythmics and anticonvulsants

* Infections: viral and bacterial infections that scar the lungs

* Genetic predisposition: including familial pulmonary fibrosis and Hermansky-Pudlak syndrome


Treatment for Pulmonary fibrosis is mainly palliative, that is, aimed at stabilizing the condition, extending the life expectancy and making the affected person more comfortable. Treatments include medications that can make breathing easier, lifestyle changes, alternative therapies and surgery, including lung transplantation. Researchers are studying new technologies and resources, including stem cell therapy and treatment with human growth factors.

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