The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease.

Abstract

RATIONALE: In cystic fibrosis (CF), lung disease is the predominant cause of morbidity and mortality. Little is known about the spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease (SALD). No specific CT scoring system for SALD is available. OBJECTIVES: To design a quantitative CT scoring system for SALD, to determine the spectrum of structural abnormalities in patients with SALD and to correlate the SALD system with an existing scoring system for mild CF lung disease and pulmonary function tests (PFTs). METHODS: 57 patients with CF contributed one CT made during screening for lung transplantation. For the SALD system, lung tissue was divid..