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Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is one of the interstitial diseases of the lung. There are many causes of interstitial lung disease, though the subgroup referred to as "idiopathic" implies that no specific cause is found. This disorder is believed to represent an autoimmune phenomenon with the body attacking the lung tissue and resulting in gradual scarring of the lung. This disease is not uncommon in middle-aged or elderly individuals. Patients often present with gradually progressive shortness of breath and activity, cough, and abnormal breath sounds referred to as crackles. These patients are often initially believed to have either congestive heart-failure or pneumonia, however, do not respond to treatments for the above.

The diagnostic workup of idiopathic pulmonary fibrosis often includes a CT scan of the chest that may demonstrate the classic features and distribution of scarring. The diagnosis is confirmed by a lung biopsy, which is generally done by a thoracic surgeon either by a thoracoscope inserted in the chest or by an open lung biopsy. Some physicians perform a bronchoscopy and biopsy to evaluate for other causes of interstitial lung disease, though the diagnosis of idiopathic pulmonary fibrosis is difficult to confirm on such small specimens. Recently, the pathologists have identified several different subtypes of this disease based on the biopsy appearance and each correlates with a differing clinical course and outcome.

Treatment initially involves prednisone to suppress the autoimmune process. In patients who do not respond to prednisone, an additional immunosuppressant agent is added. These involve either very low dose chemotherapeutic drugs or other immunosuppression agents that are used in transplant patients. Most recently, interferon injections are being looked at as a treatment for idiopathic pulmonary fibrosis. Currently, many research centers are involved in a national trial to determine the effectiveness of interferon for this disease.

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