Natural history of honeycombing: follow-up of patients with idiopathic pulmonary fibrosis treated with single-lung transplantation.

ABSTRACT

PURPOSE: Although honeycombing is one of the key features for the diagnosis of idiopathic pulmonary fibrosis (IPF), its origin and evolution are still poorly understood. The aim of our study was to analyse the natural history of honeycombing in patients treated with single-lung transplantation. MATERIALS AND METHODS: We considered seven patients who underwent single-lung transplantation; two of them (28.6%) were excluded from our analysis because they died in the posttransplantation period, whereas the remaining five (71.4%) were evaluated with computed tomography (CT) over 67.6±38.56 months. Each CT scan was assessed for disease extension and cyst size (visual score and size of target cysts); CT scans acquired after 2006 were also assessed for native lung volume. RESULTS: All patients showed disease progression (with a concurrent reduction in lung volume in two, 40%) and a progression of honeycombing, with increased number and size of cysts in four (80%). We observed dimensional changes in all target cysts (enlargement or reduction); three patients (60%) also had radiological evidence of complications, such as spontaneous rupture with pneumothorax and development of mycetomas within the cysts. CONCLUSIONS: Honeycombing is a dynamic process in which the overall trend is represented by a dimensional increase in cystic pattern; however, single cysts may have a different evolution (enlargement, reduction or complications). This behaviour could be explained by the variety of the pathogenetic processes underlying honeycombing, with cysts that may present abnormal communication with the airway, including the development of a check-valve mechanism.