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MedPix® Medical Image DatabaseDisease Topic 1225
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Contributor: William J. Quinn - Tripler Army Medical Center
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More Like This ? Idiopathic Pulmonary Fibrosis
Factoid 1225 - Created: 2001-02-21 16:11:44-05 - Modified: 2001-02-26 11:52:21-05
ACR Codes: 68.792
Progressive inflammation, fibrosis, and destruction of lung (end-stage lung) of unknown cause. Synonyms: usual interstitial pneumonia (UIP, pathology term), cryptogenic fibrosing alveolitis (British term). Hamman-Rich syndrome is an acute, rapidly fatal form of IPF, and IPF has been termed "chronic Hamman-Rich syndrome." Prognosis: mean survival 4 years (range 0.4 to 20 years). Lung biopsy is necessary for diagnosis. Treatment: steroids, cytotoxic agents.
Clinical Findings:
-clubbing
-nonproductive cough
-dyspnea
-weight loss
Radiographic Features:
Distribution:
-primarily lower lung zones and peripheral subpleural involvement.
HRCT pattern:
-early: ground glass attenuation
-later: reticular pattern in lower lobes
-end-stage: honeycombing
-traction bronchiectasis indicates fibrosis
Reference(s):
Weissleder, et al. "Primer of Diagnostic Imaging", pp32-33, Mosby, 1997.
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Location:
Chest, Pulmonary (ex. Heart)
Sublocation:
Pulmonary (lung)
Category:
Idiopathic or Unknown
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Prepared by: William J. Quinn
Affiliation: Tripler Army Medical Center - || - Author Profile
Approved by: David S. Feigin, M.D.
Affiliation: Johns Hopkins Hospitals - || - Editor Profile
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