CTICasesShort Telomere Syndrome with PPFE and Hepatopulmonary Syndrome
Imaging findings
CT chest shows upper-lobe predominant pleural thickening and subpleural consolidation (pleuroparenchymal fibroelastosis or PPFE). Abdominal CT shows cirrhosis with large esophageal/gastric varices. Lung bases show dilated subpleural vessels extending to the pleura, consistent with hepatopulmonary syndrome.
Key takeaways
Short telomere syndromes are associated with familial pulmonary fibrosis and cirrhosis. The fibrotic pattern is often PPFE or NSIP rather than typical UIP, and can coexist with hepatopulmonary syndrome.
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