Incidence, Prevalence, and Clinical Course of Idiopathic Pulmonary Fibrosis

Abstract

Background: Limited data exist regarding the population-based epidemiology of idiopathic pulmonary fi brosis (IPF). The objective of the study was to describe the trends in the incidence, prevalence, and clinical course of IPF in the community. Methods:We conducted a population-based study of adult patients with IPF in Olmsted County, Minnesota, from 1997 to 2005. Two methods were used to identify IPF cases, as defi ned by the 2002 American Thoracic Society/European Respiratory Society consensus statement: (1) usual interstitial pneumonia (UIP) on a surgical lung biopsy specimen or a defi nite UIP pattern on a high-resolution CT image (narrow criteria) and (2) UIP on a surgical lung biopsy specimen or a defi nite or possible UIP pattern on CT image (broad criteria). Results: Of 596 patients screened for the possibility of pulmonary disease or pulmonary fi brosis over 9 years of follow-up, 47 cases had IPF. Of these, 24 met the narrow criteria. The age-and sex-adjusted incidence was 8.8/100,000 and 17.4/100,000 person-years, for narrow and broad criteria, respectively. The age-adjusted incidence was higher in men than in women, and among patients aged 70-79 years. During the study period, the incidence of IPF decreased ( P , .001). On December 31, 2005, the age-and sex-adjusted prevalence was 27.9/100,000 and 63/100,000 persons by narrow and broad criteria, respectively. Thirty-seven patients experienced a total of 53 respiratory exacerbations (26 IPF related, 27 non-IPF related), and 34 (72%) patients died. The primary cause of death was IPF related in 16 (47%) patients. Median survival for narrow-criteria and broad-criteria incidence cases was 3.5 and 4.4 years, respectively. Conclusions: The incidence of IPF in Olmsted County decreased over the study period. Nonprimary IPF respiratory exacerbations are as frequent as primary IPF respiratory exacerbations and an important cause of death.CHEST 2010; 137( 1 ):129-137Abbreviations: ATS/ERS 5 American Thoracic Society and European Respiratory Society; HRCT 5 high-resolution computed tomography; IPF 5 idiopathic pulmonary fi brosis; REP 5 Rochester Epidemiology Project; UIP 5 usual interstitial pneumonia

The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

“…11,[13][14][15] In the two UK-based studies, analyses confirmed that the incidence of IPF had increased over the two time periods studied. In the Gribbin and colleagues 11 study, after adjusting for age and sex, the annual increase in incidence of IPF was estimated to be 11% (rate ratio 1.11, 95% CI 1.09 to 1.13; p < 0.0001).…”

“…Age-and sex-adjusted prevalence rates among people aged ≥ 50 years in the study undertaken in Olmsted county 14 was 27.9 (95% CI 10.4 to 45.4) cases per 100,000 people using the narrow criteria for IPF. For the broad criteria, the estimated prevalence was 63 (95% CI 36.4 to 89.6) cases per 100,000.…”

“…Overall estimates suggest prevalence of between 14 and 30 per 100,000 persons when using a narrow definition for IPF. 14,15 Mortality Mortality rates were presented from the two UK studies using data from THIN's primary care database. 11,13 In the Navaratnam and colleagues 13 study, data were also analysed from routine mortality data from the Office for National Statistics (ONS) derived from death certificates in England and Wales between 1968 and 2008 and applied to the 2008 population.…”

Significance of Combined Emphysema in Idiopathic Pulmonary Fibrosis and Serum Surfactant Protein-D as a Prognostic Factor

“…IPF is a disease with poor prognosis and diverse causes of death, [4][5][6][7] and it has not yet been determined whether features of CPFE in IPF patients have an effect on its prognosis and/or causes of death.…”

Serial Measurements of Tricuspid Regurgitation Pressure Gradient by Echocardiography Predict Prognosis In Idiopathic Pulmonary Fibrosis

“…1 The prediction of individual patient survival is difficult because of its heterogeneity, although the overall prognosis is poor with a median survival of 2.4-3.5 years. [2][3][4] Pulmonary Hypertension (PH) is an important comorbidity of advanced IPF that has a significant negative impact on survival. [5][6][7] Variable prevalence (range, 32%-84%) of PH has been reported.…”