Usual interstitial pneumonia pattern: idiopathic pulmonary fibrosis

Case contributed by Dr Hani Salam

Presentation

Progressive cough and shortness of breath. No history of arthritis, skin lesions or eye complaints.

Patient Data

Age: 65 years
Gender: Male

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. There is mild to moderate oesophageal dilatation and small hiatus hernia. Mild enlargement of the mediastinal lymph nodes are seen.

Case Discussion

This case provides us with the typical UIP pattern interstitial lung disease. The differential diagnosis of this pattern is not limited to IPF but also include other conditions such as, mixed connective tissue and collagen vascular disorders with IPF, asbestosis, chronic hypersensitivity pneumonitis, chronic pulmonary drug toxicity, recurrent bouts of aspiration and fibrosing NSIP. The presence of distal oesophageal dilatation raise the possibility of mixed connective tissue disease/scleroderma associated with IPF. The presence of hiatus hernia raise the possibility of repeated micro/aspiration as etiological factor. Open lung biopsy showed idiopathic pulmonary fibrosis (IPF).

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Case information

rID: 41974
Case created: 30th Dec 2015
Last edited: 20th Jun 2017
System: Chest
Inclusion in quiz mode: Included

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