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.
Riassunto
Obiettivo
L’honeycombing rappresenta un reperto chiave per la diagnosi di fibrosi polmonare idiopatica; nonostante questo, la sua patogenesi ed evolutività restano ancora poco conosciuti. Scopo del nostro studio è stato quello di analizzarne il comportamento evolutivo attraverso il follow-up di pazienti sottoposti a trapianto monopolmonare.
Materiali e metodi
Sette pazienti sottoposti a trapianto mono-polmonare sono stati studiati evolutivamente; di questi 2 (28,6%) sono stati esclusi dalla nostra analisi perché deceduti nel post-trapianto, mentre i restanti 5 (71,4%) sono stati valutati mediante tomografia computerizzata (TC) per 67,6±38,56 mesi. Per ogni TC sono stati analizzati: estensione della malattia, dimensioni delle cisti (score visivo medio e dimensioni di cisti target) e, per le TC acquisite dopo l’anno 2006, volume del polmone nativo.
Risultati
Tutti i pazienti (5/5, 100%) hanno evidenziato un progressione della malattia (con riduzione consensuale dei volumi polmonari in 2/5, 40%) e una evoluzione dell’honeycombing (con incremento numerico e dimensionale delle cisti in 4/5, 80%). Tutte le cisti target sono andate incontro a modificazioni dimensionali (ingrandimento o riduzione) con evidenza di complicanze in 3/5 pazienti (60%), quali rottura spontanea con pneumotorace e sviluppo di inclusi (micetomi).
Conclusioni
I nostri dati dimostrano che l’honeycombing è un processo dinamico ed evolutivo durante il quale la tendenza globale del pattern cistico è quella dell’incremento dimensionale; le singole cisti, possono però subire un diverso destino, potendo andare incontro ad ingrandimento, riduzione o complicanze. Ciò si spiega in considerazione dell’eterogeneità dei processi patogenetici alla base dell’honeycombing, con cisti che possono presentare anomale comunicazioni con le vie aeree come lo sviluppo di un meccanismo a valvola.
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Mineo, G., Ciccarese, F., Attinà, D. et al. Natural history of honeycombing: follow-up of patients with idiopathic pulmonary fibrosis treated with single-lung transplantation. Radiol med 118, 40–50 (2013). https://doi.org/10.1007/s11547-012-0810-4
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DOI: https://doi.org/10.1007/s11547-012-0810-4