Idiopathic pulmonary fibrosis (IPF) is a rare form of fibrotic lung disease with no known etiology that progresses over the course of several years and is characterized by scar tissue formation within the lungs, dyspnea, and a significantly shortened lifespan after diagnosis.
Although etiology is unknown, cigarette smoking and certain environmental exposures have been implicated in the development of the disease.
The diagnosis can be made on clinical grounds when an appropriate history of progressive symptoms (typically dyspnea and cough) is accompanied by characteristic radiographic findings and restrictive pulmonary physiology, and in the absence of findings that suggest an alternative diagnosis.
To date, no treatments have been shown to be effective in preventing or reversing the course of idiopathic pulmonary fibrosis. As a result, the most recent guidelines recommend against the use of pharmacologic therapy in most patients, although some therapies are available for consideration in a minority of patients. Important supportive measures include smoking cessation, pulmonary rehabilitation, and supplemental oxygen when appropriate. Some patients may be eligible for lung transplantation.
Chest CT scan image of idiopathic pulmonary fibrosis
From the collection of Jeffrey C. Munson, MD, MS
- weight loss, fatigue, and malaise
1st Tests To Order
- high-resolution CT (HRCT)
Other Tests to Consider
- rheumatoid factor
- surgical lung biopsy
- bronchoalveolar lavage (BAL)
- transbronchial biopsy
- admission to the hospital + high-dose corticosteroid
- cytotoxic therapy
all patients not currently experiencing acute exacerbation
- smoking cessation + pulmonary rehabilitation ± oxygen
- proton-pump inhibitors
- lung transplantation