In order to evaluate the clinical findings and survival of pulmonary idiopathic fibrosis patients, 132 cases with histologically-proven biopsy were studied, coming from Pavilhão Pereira Filho Hospital, from 1970 to 1996. The diagnosis was made in 120 patients by open lung biopsy and in 12 cases by transbronchial lung biopsy. The average age was 56 years; 78 were male and only 6 were black. Smoking was observed in 61 cases. Mean duration of symptoms before diagnosis was 22.7 months. Digital clubbing was present in 75 patients and teleinspiratory crackles in 100. Dyspnea was observed in all but two patients and cough was present in 89 cases. Lung function test values were: FVC, 62%; FEV1, 70%; DLCO, 43.4%; TLC, 76.7%; PaO2, 67.3 mmHg; PaCO2, 39.1 mmHg and SaO2, 92.3%. Bronchoalveolar cellularity values were: macrophages, 83.8%; neutrophils, 9.1%; lymphocytes, 6.1% and eosinophils, 0.6%. In X-ray, honeycombing was present in 79 cases, reduced total lung capacity in 107 and intrathoracic tracheal widening in 50. In CT, the mean reticular pattern profusion was 42.3% and the mean granular pattern profusion was 43.6%. The usual histologic pattern was found in 128 cases, and the descamative pattern in only 4. Information about survival was found in 121 cases until December 1997. The mean survival rate of all patients was 28 months and for dead patients was 24 months. Patient characteristics in this study were associated with advanced stage of disease, which was confirmed by small survival rates of those cases. The strong predominance of usual pattern and better patient selection may have contributed to these results.

Keywords: Pulmonary fibrosis, interstitial lung disease, clinical symptoms; Survival analysis.