Incidence and Prevalence of Idiopathic Pulmonary Fibrosis
ABSTRACT 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.
- SourceAvailable from: Glenda Ernst[Show abstract] [Hide abstract]
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
- [Show abstract] [Hide abstract]
ABSTRACT: Background Nintedanib is in clinical development as a treatment for idiopathic pulmonary fibrosis (IPF). Data from the Phase II TOMORROW study suggested that nintedanib 150 mg twice daily had clinical benefits with an acceptable safety profile. Methods The INPULSISTM trials are replicate Phase III, randomized, double-blind, studies comparing the efficacy and safety of nintedanib 150 mg twice daily with placebo in patients with IPF. Eligible patients were aged ≥40 years with a diagnosis of IPF within 5 years before randomization who had undergone a chest high-resolution computed tomography (HRCT) scan within 1 year before screening, and who had a forced vital capacity (FVC) of ≥50% predicted and a diffusing capacity for carbon monoxide of 30–79% predicted. Participants were randomized 3:2 to receive nintedanib or placebo for 52 weeks. The primary endpoint is the annual rate of decline in FVC. The key secondary endpoints are change from baseline in the total score on the St. George’s Respiratory Questionnaire (a measure of health-related quality of life) over 52 weeks and time to first acute exacerbation. Results Enrolment of 1066 patients in 24 countries was completed in September 2012. Results will be reported in the first half of 2014. Conclusion The INPULSIS™ trials will determine the efficacy of nintedanib in patients with IPF, including its impact on disease progression as defined by decline in FVC, acute exacerbations and health-related quality of life. In addition, they will characterise the adverse event profile of nintedanib in this patient population. Trial registration: Registered at ClinicalTrials.gov (identifiers: NCT01335464 and NCT01335477).Respiratory Medicine 07/2014; 108(7). DOI:10.1016/j.rmed.2014.04.011 · 2.92 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Objective: Evaluation of Myofibroblasts by studying expression of Alpha smooth muscle actin: A marker of Fibrosis, Dysplasia and Carcinoma. Background: Myofibroblasts are cells that have contractile properties and are involved in inflammation, wound healing, fibrosis and oncogenesis in most of the organs and tissues. They are involved in healing and granulation tissue formation which occur after tissue injuries, also produce inflammatory mediators, growth factors and help in extracellular matrix reorganization by secretion of proteins like collagen, fibronectin, etc. Because of their component, Alpha smooth muscle actin ([alpha]-SMA), they are involved in the contraction of extracellular matrix and aid in tissue contraction. The myofibroblasts disappear by apoptosis after completion of repair, but their persistence causes a dysfunction in the repair mechanism, leading to excessive contraction and extracellular matrix (ECM) secretion and thus, fibrosis. The purpose of this study was to evaluate the presence of myofibroblasts in cases of Oral Submucous fibrosis (OSMF), which consisted of very early, early and moderately advanced OSMF, OSMF with dysplasia and oral squamous cell carcinoma (OSCC), by detecting (alpha)-SMA, which is a specific marker for myofibroblasts. Materials and Methods: The study sample consisted of three groups which comprised of 41 cases of OSMF, 10 cases of OSMF with dysplasia and 11 cases of OSCC. All the cases were subjected to immunohistochemistry by using (alpha)-SMA antibody for detection of myofibroblasts. Results: The presence of myofibroblasts was significantly higher in oral squamous cell carcinomas as compared to that in OSMF with dysplasia and OSMF. A statistical significance was also noted between the staining index and age of the individuals and the staining index and duration of the habit. Conclusion: Myofibroblasts play a role in fibrosis, as was seen in OSMF. Activated myofibroblasts secrete proteolytic enzymes and cause matrix degradation, which is instrumental in cancer cell invasion and metastasis. Further studies in which the myofibroblasts are targetted, may help in providing therapeutic regimens in fibrosis, dysplasia and cancer.Journal of Clinical and Diagnostic Research 04/2014; 8(4):zc14-zc17. DOI:10.7860/JCDR/2014/7820.4231 · 0.13 Impact Factor