Desquamative interstitial pneumonia may progress to lung fibrosis as characterized radiologically

Background and objective: 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. Methods: 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. Results: 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. Conclusions: In a proportion of patients, desquamative interstitial pneumonia may progress to lung fibrosis with honeycombing on HRCT, despite therapy.