PULMONARY FIBROSIS

What is Pulmonary Fibrosis?

Symptoms

• Breathlessness, especially during exercise
• Chronic dry cough
• Shortness of breath
• Chest discomfort
• Fatigue and weakness
• Loss of appetite
• Rapid weight loss
• Enlargement at end of fingertips so they take on a bulbous appearance, often referred to as “clubbing” (the condition may progress to the point where any exertion is impossible)

Causes

• Exposure to cigarette smoke
• Inhaled environmental pollutants
• Occupational pollutants, including asbestos, ground stone, metal dust or mouldy hay
• A number of diseases can cause pulmonary fibrosis
• Sarcoidosis
• Scleroderma
• Rheumatoid Arthritis
• Lupus
• Tuberculosis
• Systemic Lupus Erthematosis
• Grain handler’s lung
• Bagassosis
• Detergent worker’s lung
• Hermansky Pudlak Syndrome
• Certain strong mediations
• Nitiofurantoin [for urinary tract infections]
• Amiodarone [for an irregular heart rate]
• Bleomycin, cyclophosphamide and methotrexate [for fighting cancer]
• Therapeutic radiation

Diagnosis

A number of different diagnostic techniques are used to diagnose pulmonary fibrosis. Indicators which may point towards a patient having pulmonary fibrosis include:

• Chest x-rays
• Pulmonary function tests – These tests reveal the efficiency of a patient’s breathing mechanism
• Bronchoalveolar lavage – Cells in the lower respiratory tract are removed and examined
• Injection with gallium 67 – Inflamed areas absorb gallium 67 which can later be seen on sensitized film
• Lung biopsy – allows an examination of cells in and around the alveoli

Prognosis

The outcomes of pulmonary fibrosis differ for each individual. In many cases pulmonary fibrosis remains mild and takes a number of years to slowly progress. However, in other cases pulmonary fibrosis may be due to underlying diseases such as rheumatoid arthritis. These underlying diseases may progress and affect the pulmonary fibrosis itself. Pulmonary fibrosis tends to result in a slow progressive scarring of the lungs. Unfortunately some patients do not respond positively to therapy and their lungs will slowly deteriorate over a period of months or years until they are no longer able to function adequately. Alternatively other patients have a positive response to therapy. The duration and speed of the onset of pulmonary fibrosis differs on a case by case basis.

Treatment

If one of the known causes of pulmonary fibrosis exists then that underlying disease can be treated in order to limit the inflammation in the lungs. Limited inflammation will prevent the formation of scar tissue in the lungs and hopefully stop the progression of pulmonary fibrosis. However, due to the lack of standard diagnostic criteria, no effective treatments or a cure for pulmonary fibrosis currently exist.

Drugs known as corticosteroids are usually administered in order to try to stop the inflammation. It is best to give corticosteroid medications as early in the course of the disease as possible, as there appears to be a better chance of improvement. However treatment with corticosteroids has not proven to be effective in all cases. In addition corticosteroid medications have a number of side effects, meaning that the safety and benefit of using these medications must be continually reassessed by a doctor or physician.
Although much more research is required regarding drug therapy for pulmonary fibrosis, a number of supportive therapy programs exist to help patients adjust to symptoms of this condition. These include education and rehabilitation programs, which assist patients to breathe efficiently and to perform daily activities while keeping breathlessness to a minimum.

Supplemental oxygen can be administered in order to treat breathlessness and to improve a patient’s quality of life and exercise capacity. Removing the patient from the environment in which pollutants are being inhaled can also assist in limiting the progression of the disease. Chest infections require early treatment. Some patients may be considered for a single lung transplant. In addition, patients must quit smoking, as tobacco aggravates shortness of breath.