PURPOSE: The results of a study done in 2005, at this adult Cystic Fibrosis (CF) center revealed that the commonest adult chest Xray (CXR) was diffuse bilateral disease (DBL). This contrasts the usually reported upper lobe (UL)predominance. This raised the question whether UL disease progresses to all segments of lung (DBL) or if it conveys a survival advantage. Objective: To review the earliest available CXR in those adult CF patients with DBL on CXR.
METHODS: In the primary study 109 CF patients were identified and their CXR reviewed. These CXR’s were defined as center intake CXRs. Lack of CXR or genotype data excluded all but 68 pts from the initial study. DBL pattern was present in 44 of 68 pts (65%). The earliest available CXRs on these patients were requested from the pediatric CF program. Of these 44 patients only 19 were found to have available archived CXRs. These were reviewed by two unblinded independent reviewers. Statistical Analysis: Descriptive statistics were used for analyses.
RESULTS: There were 9males and 10 females in the 19 films reviewed. The mean and median age of the adult population (19 films) were 30 and 29 (range 20-44). The mean and median age of the patients at time of archived films (which went back 15 years on average) were 16 and 11 (range 8-31)yr. All but two films had DBL distribution in their historical films. There was no evidence of UL disease on the earliest available films. In two patients unilateral (1) or lower lobe (1) disease predominated in archived films.
CONCLUSION: In reviewed films there was no evidence that DBL had evolved from previously UL disease. This sharply contradicts prior studies and suggests:1) that upper lobe disease does not progress to diffuse bilateral disease; 2: that DBL disease pattern conveys a survival advantage.
CLINICAL IMPLICATIONS: This is a small single center study and a large multi-center review is needed to further evaluate whether diffuse bilateral disease in CF conveys a survival advantage.
DISCLOSURE: Vaidehi Kaza, None.