Idiopathic Pulmonary Fibrosis
A disease of unknown etiology characterized by alveolitis leading to diffuse alveolar damage and pulmonary fibrosis. Many believe the disease is immunologic due to antigen or antigen-antibody complex induced alveolar membrane injury.
CXR and CT findings: IPF has typical CXR and high resolution CT findings.
- linear interstitial changes in the lower lungs with a peripheral distribution
- associated lower lung volume loss
- end-stage changes of honeycombing particularly in the periphery of the lower lungs
- absence of significant pleural reaction
- absence of adenopathy
Clinical Clues:
1. Classically finger clubbing and so-called “Velcro” rales in the lung bases are found. Onset in middle aged to older patients with a mean time from diagnosis to death of 4-5 years. This may be changing with an increase lead time in diagnosis due to the increasing use of CT.
2. There is no definite association with smoking or lung cancer although either may make the clinical and radiologic pattern worse
3. No specific or direct therapy available (?? total lung transplant)
Diagnosis: Usually made clinically and with chest imaging. Many clinicians will do a biopsy to fully exclude underlying unsuspected treatable diseases.
"Aunt Sophies": Rheumatoid lung disease, scleroderma lung disease, chronic aspiration, asbestosis, rarely drug reactions, non-specific interstitial pneumonitis (NSIP)
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