Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

University of Washington Medical Center, Seattle, WA 98195-6522, USA.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 13). 11/2006; 174(7):810-6. DOI: 10.1164/rccm.200602-163OC
Source: PubMed


Idiopathic pulmonary fibrosis is a chronic interstitial lung disease of unknown etiology; its epidemiology in the United States has not been well characterized.
To estimate the annual incidence and prevalence of idiopathic pulmonary fibrosis in the United States.
Retrospective cohort design utilizing a large health care claims database spanning the period January 1996 through December 2000.
Persons with idiopathic pulmonary fibrosis were identified based on diagnosis and procedure codes. Using broad case-finding criteria, prevalence was estimated to range from 4.0 per 100,000 persons aged 18 to 34 yr to 227.2 per 100,000 among those 75 yr or older; annual incidence was estimated to range from 1.2 to 76.4 per 100,000. Using narrow case-finding criteria, prevalence ranged from 0.8 to 64.7 per 100,000 persons; comparable figures for incidence were 0.4 to 27.1 per 100,000 persons. Extrapolating these rates to the overall United States' population, prevalence was estimated to be 42.7 per 100,000 (incidence, 16.3 per 100,000) using broad criteria; with narrow criteria, prevalence was estimated to be 14.0 per 100,000 (incidence, 6.8 per 100,000).
Our results suggest that idiopathic pulmonary fibrosis is probably more common in the United States than previously reported.

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    • "Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive and fatal disease which affects adult subjects between 60 and 75 years old [1]. The average survival time has a range between 2 to 5 years since diagnosis, however the progression rate and the size of damage could result unpredictable [2] [3]. "
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    ABSTRACT: Abstract Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive and fatal disease. The average of survival time has a range between 2 to 5 years, but the progression rate and the size of damage could result unpredictable. This fibrotic illness is limited to lung with low or absent inflammation. Recently, it has been described new therapeutic options. Clinical trials were not powered to detect statistically significant differences in mortality; but these have shown a reduction in the rate of decline in lung function. The results remain variables due to the heterogeneity observed in these patients. The challenge is discover new predicting outcomes, biological indicators of disease progression, short-term measures of therapeutic response or predictors of survival time useful to take decisions about the treatment or help to determine the need of lung transplantation and contribute.
    01/2015; 02(999):1-1. DOI:10.2174/2212703802666150127002032
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    • "In 2011, approximately 886 IPF patients in the US underwent lung transplantation [19]. Using a prevalence of 14–43 per 100,000 [73] and a US adult population of approximately 311 million in 2011 [74], less than 1% of the estimated 42,000–130,000 US IPF patients had received a lung transplant during 2011. Not all IPF patients would be eligible for or willing to undergo transplantation, thus this probably overestimates the proportion not receiving a transplant. "
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    ABSTRACT: Background Idiopathic pulmonary fibrosis (IPF) is a distinct form of interstitial pneumonia with unknown origin and poor prognosis. Current pharmacologic treatments are limited and lung transplantation is a viable option for appropriate patients. The aim of this review was to summarize lung transplantation survival in IPF patients overall, between single (SLT) vs. bilateral lung transplantation (BLT), pre- and post Lung Allocation Score (LAS), and summarize wait-list survival. Methods A systematic review of English-language studies published in Medline or Embase between 1990 and 2013 was performed. Eligible studies were those of observational design reporting survival post-lung transplantation or while on the wait list among IPF patients. Results Median survival post-transplantation among IPF patients is estimated at 4.5 years. From ISHLT and OPTN data, one year survival ranged from 75% - 81%; 3-year: 59% - 64%; and 5-year: 47% - 53%. Post-transplant survival is lower for IPF vs. other underlying pre-transplant diagnoses. The proportion of IPF patients receiving BLT has steadily increased over the last decade and a half. Unadjusted analyses suggest improved long-term survival for BLT vs. SLT; after adjustment for patient characteristics, the differences tend to disappear. IPF patients account for the largest proportion of patients on the wait list and while wait list time has decreased, the number of transplants for IPF patients has increased over time. OPTN data show that wait list mortality is higher for IPF patients vs. other diagnoses. The proportion of IPF patients who died while awaiting transplantation ranged from 14% to 67%. While later transplant year was associated with increased survival, no significant differences were noted pre vs. post LAS implementation; however a high LAS vs low LAS was associated with decreased one-year survival. Conclusions IPF accounts for the largest proportion of patients awaiting lung transplants, and IPF is associated with higher wait-list and post-transplant mortality vs. other diagnoses. Improved BLT vs. SLT survival may be the result of selection bias. Survival pre- vs. post LAS appears to be similar except for IPF patients with high LAS, who have lower survival compared to pre-LAS. Data on post-transplant morbidity outcomes are sparse.
    BMC Pulmonary Medicine 08/2014; 14(1):139. DOI:10.1186/1471-2466-14-139 · 2.40 Impact Factor
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    • "(40 cases in total autopsies) [2]. On the other hand, it has been reported that the prevalence of idiopathic pulmonary fibrosis (IPF) in the United States and Japan were estimated to be 14.0 to 42.7 and 3.44 per 100,000 people, respectively [3,4]. It is unknown whether the prevalence of IPF and prevalence of lung cancer in IPF patients are influenced by ethnic, geographic or cultural factors or not, because there are no reports on direct comparison of incidence and prevalence rate between Caucasian and Japanese [5]. "
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    ABSTRACT: Background Non-small cell lung cancer (NSCLC) patients with interstitial lung disease (ILD) need to be approached carefully given the high incidence of pulmonary toxicity. Pemetrexed (PEM) is the key drug for the treatment of NSCLC. However, its safety, especially with respect to the exacerbation of ILD, and efficacy in NSCLC patients with ILD have yet to be established. Method We investigated the safety and efficacy of PEM monotherapy in NSCLC patients with or without idiopathic interstitial pneumonia (IIPs). The medical charts of these patients were retrospectively reviewed. Results Twenty-five patients diagnosed as having IIPs (IIPs group) and 88 patients without ILD (non-ILD group) were treated with PEM monotherapy at Juntendo University Hospital between 2009 and 2013. In the IIPs group, 12 patients were found to have usual interstitial pneumonitis (UIP) on chest computed tomography (CT) (UIP group) and the other 13 patients showed a non-UIP pattern on chest CT (non-UIP IIPs group). Three patients in the IIPs group (2 in the UIP group and 1 in the non-UIP IIPs group) and 1 in the non-ILD group developed pulmonary toxicity during treatment (3.5% overall, 12.0% in the IIPs group versus 1.1% in the non-ILD group). Moreover, all 3 patients in the IIPs group died of pulmonary toxicity. Overall survival tended to be longer in the non-ILD group than in the IIPs group (p = 0.08). Multivariate analyses demonstrated that IIPs was the only significant independent risk factor for PEM-related pulmonary toxicity. Conclusion We found that the incidence of PEM-related pulmonary toxicity was significantly higher amongst NSCLC patients with IIPs than among those without IIPs. Particular care must be taken when administering PEM to treat NSCLC patients with IIPs.
    BMC Cancer 07/2014; 14(1):508. DOI:10.1186/1471-2407-14-508 · 3.36 Impact Factor
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