Idiopathic pulmonary fibrosis (IPF) is a rare form of fibrotic lung disease with no known etiology that progresses over the course of several years. It is characterized by scar tissue formation within the lungs, dyspnea, and a significantly shortened lifespan after diagnosis.
Although the etiology is unknown, cigarette smoking and certain environmental exposures have been implicated in the development of IPF.
In the absence of findings that suggest an alternative disease process, IPF can be diagnosed on clinical grounds when an appropriate history of progressive symptoms (typically dyspnea and cough) is accompanied by characteristic radiographic findings and restrictive physiology on pulmonary function testing.
Initial therapy is with an antifibrotic agent. Important supportive measures include smoking cessation, pulmonary rehabilitation, and supplemental oxygen when appropriate. Some patients may be eligible for lung transplantation.
Idiopathic pulmonary fibrosis (IPF) is a rare, chronic, life-threatening disease that manifests over several years and is characterized by the formation of scar tissue within the lungs and progressive dyspnea. It is the most common interstitial lung disease among the idiopathic interstitial pneumonias, which share clinical features of shortness of breath, diffuse pulmonary infiltrates on imaging, and varying degrees of inflammation, fibrosis, or both on lung biopsy.
History and exam
Key diagnostic factors
Other diagnostic factors
- weight loss, fatigue, and malaise
- advanced age
- male sex
- family history
- cigarette smoking
- organic and inorganic dust exposure
- gastroesophageal reflux
1st investigations to order
- high-resolution CT (HRCT)
- antinuclear antibodies (by immunofluorescence)
- rheumatoid factor
- anti-cyclic citrullinated peptide
- myositis panel
Investigations to consider
- pulmonary function tests
- surgical lung biopsy
- bronchoalveolar lavage (BAL)
- transbronchial biopsy
- C-reactive protein (CRP)
- erythrocyte sedimentation rate (ESR)
all patients not currently experiencing acute exacerbation
- Nonspecific interstitial pneumonia
- Cryptogenic organizing pneumonia
- Acute interstitial pneumonia (AIP)
- Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline
- Home oxygen therapy for adults with chronic lung disease
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