Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests

Purpose. To describe the high resolution CT (HRCT) imaging and functional features of the emphysema/fibrosis syndrome. Patients and methods. A total of 61 patients were included based on HRCT. We have quantified the extent of fibrosis and emphysema lesions and a combined score was calculated. The score were correlated to pulmonary function test parameters and specific HRCT features were described. Results. The emphysema and fibrosis scores correlated with functional parameters of obstruction and restriction respectively. The combined score correlated with the reduction in DLCO and degree of pulmonary hypertension. Three HRCT patterns were identified : progressive transition (n = 23, 38%) with diffuse emphysema (centrilobular and/or bullous) and zone of transition between bullae and honeycombing; paraseptal emphysema (n = 13, 21%) with predominant subpleural bullae of enlarging size at the bases; separate processes (n = 14, 23%) with independence areas of fibrosis and emphysema. Eleven patients (18%) could not be classified. The HRCT imaging features changed based on TLC(p = 0.04) and FEV/FVC(p = 0.01). Conclusion. The emphysema/fibrosis syndrome may be associated with different patterns on HRCT corresponding to specific profiles on pulmonary function tests.