Our results suggest that idiopathic pulmonary fibrosis is probably more common in the United States than previously reported.

Recognition of the common occurrence of acute fatal deterioration in patients with mild to moderate IPF has important implications for monitoring patients and supports early referral for lung transplantation.

Rationale: Several predictors of mortality in patients with idiopathic pulmonary fibrosis have been described; however, there is a need for a practical and accurate method of quantifying the prognosis of individual patients. Objectives: Develop a practical mortality risk scoring system for patients with idiopathic pulmonary fibrosis. Methods: We used a Cox proportional hazards model and data from two clinical trials (n ¼ 1,099) to identify independent predictors of 1-year mortality among patients with idiopathic pulmonary fibrosis. From the comprehensive model, an abbreviated clinical model comprised of only those predictors that are readily and reliably ascertained by clinicians was derived. Beta coefficients for each predictor were then used to develop a practical mortality risk scoring system. Measurements and Main Results: Independent predictors of mortality included age, respiratory hospitalization, percent predicted FVC, 24-week change in FVC, percent predicted carbon monoxide diffusing capacity, 24-week change in percent predicted carbon monoxide diffusing capacity, and 24-week change in health-related quality of life. An abbreviated clinical model comprising only four predictors (age, respiratory hospitalization, percent predicted FVC, and 24-wk change in FVC), and the corresponding risk scoring system produced estimates of 1-year mortality risk consistent with observed data (9.9% vs. 9.7%; C statistic ¼ 0.75; 95% confidence interval, 0.71-0.79). Conclusions:The prognosis for patients with idiopathic pulmonary fibrosis may be accurately determined using four readily ascertainable predictors. Our simplified scoring system may be a valuable tool for determining prognosis and guiding clinical management. Additional research is needed to validate the applicability and accuracy of the scoring system.

We employed a retrospective cohort design to estimate the prevalence and economic burden of bronchiectasis. Data were obtained from the health-care claims processing systems of more than 30 US health plans (with a combined total of 5.6 million covered lives) and spanned the period January 1, 1999, to December 31, 2001. Study subjects consisted of all persons who were aged Ն18 years in 2001 and had diagnoses of bronchiectasis between 1999 and 2001; those with diagnoses of cystic fibrosis were excluded. For purposes of comparison, a cohort of persons without diagnoses of bronchiectasis was randomly selected and matched on age, sex, geographic region, and comorbid conditions. Prevalence of bronchiectasis ranged from 4.2 per 100,000 persons aged 18 -34 years to 271.8 per 100,000 among those aged Ն75 years. Prevalence was higher among women than men at all ages. Persons with bronchiectasis averaged 2.0 (95% confidence interval 1.7-2.3) additional days in hospital, 6.1 (6.0 -6.1) additional outpatient encounters, and 27.2 (25.0 -29.1) more days of antibiotic therapy than those without the disorder in 2001; average total medical-care expenditures were $5681 ($4862-$6593) higher for bronchiectasis patients. Our findings suggest that over 110,000 persons in the United States may be receiving treatment for bronchiectasis, resulting in additional medical-care expenditures of $630 million annually.