Cryptogenic fibrosing alveolitis in Moravia and Silesia (4 million inhabitants) was evaluated using standard epidemiological and clinical parameters. During 1981-1990 the incidence of cryptogenic fibrosing alveolitis ranged from 0.74 to 1.28/100,000. The trend of incidence was increasing slightly. The prevalence rose from 6.5 to 12.1/100,000. The male/female ratio was 1:1.2; 54% of patients were less than 40 years old. Biopsy (mostly open lung biopsy) supported the diagnosis in 38% of cases. During 1981-1990 years 379 new cases were detected, 488 cases altogether were registered up to 1990. The death rate calculated by incidence and prevalence was 13% and 10% resp. The mean regional incidences varied from 0.34 to 2.69/100,000, regional prevalences varied from 4.1 to 27.6/100,000. No correlation with the regional incidences of sarcoidosis and tuberculosis was found. No significant differences in coal mining areas with a high occurrence of pneumoconiosis or in areas with developed agriculture were detected. Significantly higher values were found in the regions of the four major diagnostic centres: a mean incidence of 1.58/100,000 and a mean prevalence of 20.8/100,000 (p < 0.05). The results suggest that the level of diagnostic possibilities and efforts dominates over ecological and environmental factors in the detecting of cryptogenic fibrosing alveolitis.

Dept of Tuberculosis and Pulmonary Diseases University Hospital Olomouc Czech Republic

Idiopathic Pulmonary Fibrosis: Review of Current Knowledge