Idiopathic pulmonary fiborsis (IPF) is mostly diagnosed in advanced stage

Background: IPF is lung disease of unfavorable prognosis. Earliest stages are responsive to treatment. Late diagnosis causes bad outcome.

Patients and methods: 32 cases of IPF were diagnosed in 2 years. Mean age was 64 years. Symptoms, chest radiograph, lung function were assessed in all, HRCT, lung bioppsy in 18 and BAL in 22 cases. In advanced stage of 20 cases no therapy has been introduced.

Results: Leading symptom was dyspnoe in 20 patients, cough in 8 others. X-ray showed reticular pattern, predilectionnaly peripheroposterobasal in 27 cases, ground glass only in 5. Reduction of lung volume was in 24 cases. Vital capacity was 68% and diffusion capacity 54% of reference in average. In BAL of 22 cases elevation of neutrophyls and eosinophyls in all and of lymphocites only in two cases was found. Lung biopsy in HRCT suggested zones showed mostly predominance of fibroplasia over active infiltration. In 3 cases fibroblastic foci were seen as well. In two years follow-up only in 3 cases slight regression, stagnation in 4 cases and progression in all the others was found. Conclusion: The diagnosis of IPF is still proven too late. Dyspnea and dry cough indicate lung HRCT and BAL early enough to indicate therapy of IPF. Lung biopsy is not allways obligate.