Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (aka Cryptogenic fibrosing alveolitis) involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker, causing an irreversible loss of the tissue’s ability to transfer oxygen into the bloodstream.

Symptoms

Shortness of breath, particularly with exertion
Chronic dry, hacking cough
Fatigue and weakness
Discomfort in the chest
Loss of appetite
Rapid weight loss

What is the prevalence of pulmonary fibrosis?

There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with pulmonary fibrosis. As a consequence of misdiagnosis, the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same number as that of people who die from breast cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research also indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.

Causes

Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:

Inhaled environmental and occupational pollutants
Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis
Certain medications
Therapeutic radiation

Differential diagnosis

The diagnosis of CFA is usually made in a patient presenting with the above signs and characteristic CT changes. The differential diagnosis of the chest X-ray appearance includes extrinsic allergic alveolitis, bronchiectasis, chronic left heart failure, sarcoidosis, industrial lung disease and lymphangitis carcinomatosa.

Treatments

There are currently no effective treatments or a cure for pulmonary fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress. Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. The median survival time for patients with CFA is approximately 5 years, although mortality is very high in the acute form. Treatment with prednisolone (30 mg daily) is usually prescribed for disabling disease, though its benefit has still to be proved by appropriate controlled trials. Azathioprine or cyclophosphamide may be added if there is no response. Supportive treatment includes domiciliary oxygen therapy. In severe disease, single lung transplantation can be offered.

Notable Individual Sufferers

Evel Knievel