Idiopathic pulmonary fibrosis
 Discussion of Idiopathic pulmonary fibrosis
- IPF is the most common entity of the idiopathic intersitial pneumonias (IIPs).
- IPF is the term for the clinical syndrome associated with the morphologic pattern of usual interstitial pneumona (UIP).
- With a median survival time ranging from 2 to 4 years, IPF has a substantially poorer prognosis than NSIP, COP, RB-ILD, DIP, and LIP.
- Typical patient with IPF is 50 years old or older.
- Patients present with progressively worsening dyspnea and nonproductive cough.
- A history of cigarette smoking seems to be a risk factor for the development of IPF; however, it does not appear to affect the course of the disease.
- A combination therapy of cyclosporin A and corticosteroids seems to be efficacious for acute exacerbations of IPF. In addition, patients should be considered candidates for lung transplantation early after diagnosis.
- The histologic hallmark of UIP is the presence of scattered fibroblastic foci.
- Typically, the lung involvement is heterogeneous and areas of normal lung alternate with interstitial inflammation and honeycombing. Therefore, biopsy samples from more than one lobe should be obtained in any patient with suspected IIP, and high-resolution CT should serve as a guiding tool for determining the appropriate anatomic location of the biopsy site.
 Imaging Findings for Idiopathic pulmonary fibrosis
 Plain film
- Chest radiograph is normal in most patients with early disease.
- In advanced disease, the chest radiograph shows decreased lung volumes and subpleural reticular opacities that increase from the apex to the bases of the lungs.
- Subpleural reticular opacities, macrocystic honeycombing combined with traction bronchiectasis, and the apicobasal gradient represents a trio of signs that is highly suggestive of UIP.
- Typically, imaging findings are heterogeneous, with areas of fibrosis alternating with areas of normal lung (Fig 5).
Patient #1: Radiographs and CT demonstrate usual intersitial pneumonia
 See Also
- Nonspecific interstitial pneumonia (NSIP)
- Cryptogenic organizing pneumonia (COP)
- Respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)
- Desquamative interstitial pneumonia (DIP)
- Lymphoid interstitial pneumonia (LIP)
- Acute interstitial pneumonia (AIP)
 External Links
 References for Idiopathic pulmonary fibrosis
- Christina Mueller-Mang, Claudia Grosse, Katharina Schmid, Leopold Stiebellehner, and Alexander A. Bankier. What Every Radiologist Should Know about Idiopathic Interstitial Pneumonias. RadioGraphics 2007 27: 595-615.
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