Presentation
Progressive cough and shortness of breath. No history of arthritis, skin lesions or eye complaints.
Patient Data
PA and lateral chest radiographs show a reduction in lung volume and bilateral fairly symmetrical irregular linear opacities creating a background of coarse reticular pattern.
Bilateral symmetrical inter and intralobular septal thickening associated with architectural distortion traction bronchiectasis and honeycombing with more severe involvement toward the lung bases. Coronal MPR: typical distribution of fibrotic changes being peripheral and more toward the lung bases.
Mild to moderate esophageal dilatation and small hiatus hernia. Mild enlargement of the mediastinal lymph nodes are seen.
The pulmonary artery is mildly dilated which could reflect pulmonary arterial hypertension.
Case Discussion
This case provides us with the typical UIP pattern of interstitial lung disease. The differential diagnosis of this pattern is not limited to IPF but also includes other conditions such as:
- mixed connective tissue and collagen vascular disorders with IPF
- asbestosis
- chronic hypersensitivity pneumonitis
- chronic pulmonary drug toxicity
- recurrent aspiration
- fibrosing NSIP
The presence of distal esophageal dilatation raises the possibility of mixed connective tissue disease/scleroderma associated with IPF.
The presence of hiatus hernia raise the possibility of repeated microaspiration as an etiological factor.
Open lung biopsy showed idiopathic pulmonary fibrosis (IPF).