Desquamative interstitial pneumonia may progress to lung fibrosis as characterized radiologically

In some patients, desquamative interstitial pneumonia may progress to lung fibrosis. The aim of this study was to assess the long-term radiological follow-up results in patients with desquamative interstitial pneumonia.

Among 75 patients suspected of having desquamative interstitial pneumonia, 31 who fulfilled the criteria were included in this study. Clinical characteristics at presentation, responses to treatment and long-term follow-up were evaluated.

The 31 patients were predominantly males (94%), and the mean age was 55 years; 93% (28/30) had a history of smoking. The clinical findings included high serum levels of lactate dehydrogenase and immunoglobulin G. Bronchoalveolar lavage (26 patients, 84% of cases) frequently showed an increased percentage of eosinophils (mean 17%). Computed tomography (CT) or high resolution (HR) CT at presentation showed ground glass opacities and/or consolidation in all patients, with one third of patients also showing thin-walled cysts within the ground glass opacities. There was no honeycombing on CT or HRCT scans at presentation. Corticosteroid therapy was effective early in the course of the disease; long-term follow-up (mean 99 months) of 31 patients showed only one death due to progression of the disease, but long-term follow-up of 14 patients (mean 125 months) by HRCT showed the development of new thin-walled cysts and honeycombing in five and lung cancer in four patients, respectively.

In a proportion of patients, desquamative interstitial pneumonia may progress to lung fibrosis with honeycombing on HRCT, despite therapy.