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.

In order to evaluate which prognostic factors were significant to the survival of patients with idiopathic pulmonary fibrosis (IPF), 121 histologically confirmed cases of the disease were studied at the Pereira Filho Hospital from 1970 to 1996. All patients were submitted to a standard thorax X-ray and spirometry and answered a standardized questionnaire when admitted to hospital. They also underwent diffusion tests (34 cases), total lung capacity (28), blood gas analysis (106), bronchoalveolar lavage (39), rheumatoid analyses (45), and thoracic CT (24). For further analysis, the patients were classified into two groups: group A (2-year survival) with 55 patients, and group B (more than 5-year survival) with 24 patients; these features were also analyzed according to their significance to survival. Age, increased dyspnea index, long symptomatic period, FVC, DCO, PaO2 and SaO2 reduction, honeycombing intensity, and greater profusion of the reticular pattern on HRCT were considered indicative of worse prognosis. A reduced FEV1 and TLC were also associated with shorter survival. The use of those criteria which had shown statistical significance when evaluated together may determine a more accurate prognostic evaluation of IPF patients resulting in social and therapeutic benefits to patient management.

Keywords: Pulmonary fibrosis. Interstitial lung diseases. Prognosis. Prospective studies. Survival analysis.