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[Course of idiopathic pulmonary fibrosis of the Wells grade III at presentation. Study using high-resolution
M Zompatori1, L Fasano, G Battista
1Policlinico S. Orsola-Malpighi, Bologna.
Purpose:
We studied the HRCT and functional evolution of idiopathic pulmonary fibrosis (IPF) patients presenting with Wells grade III--prevalent fibrosis.
Material And Methods:
We sequentially studied the HRCT and functional findings of 16 IPF patients, at presentation and at 1 year. All patients had a typical grade III IPF pattern; those with the most severe clinical presentation were treated (9/16). The main HRCT parameters were the extent of interstitial involvement and emphysema (visual score) and the mean diameter of lung cysts in honeycombing regions.
Results And Conclusions:
Most of our grade III IPF patients exhibited a slowly progressive deterioration, with no accelerated parenchymal opacification. Deterioration was found on HRCT images in 56.2% of patients (p = .02), with a mean monthly increase of .56%. Fibrosis extent, evaluated as HRCT visual score at presentation, was significantly correlated with viability and PaO2, values (p = .01). Follow-up HRCT scores were also significantly correlated with viability (p = .004). The mean diameter of honeycomb lung cysts increased in 25% of patients. Emphysema was associated at presentation in 50% of patients--all of them former smokers; it was less diffuse than interstitial involvement (15% of total lung volume versus 46.7% at presentation) and was not seen to progress on follow-up images. The comparison between treated (T) and untreated (NT) patients confirmed more severe HRCT and functional damage in T patients at presentation. Moreover, T patients presented a significantly more rapid deterioration, despite treatment, than NT patients, who had less severe and slower HRCT and functional evolution, excluding DLCO deterioration (p = .01). To conclude, grade III IPF patients can be subdivided into two subgroups, with rather different prognosis and evolution, on the basis of HRCT and functional findings at presentation. The current treatment seems useless in grade III IPF. HRCT findings, integrated with the visual score of disease extent, and lung function tests can be used to monitor grade III IPF evolution.