Guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis

  In 2000, the American Thoracic Society European Respiratory Society (ATS/ERS) published a consensus on the diagnosis and treatment of idiopathic pulmonary fibrosis. After 11 years, many important advances have been made in both clinical and basic research on IPF.  In 2011, leading experts in the field of interstitial lung disease (ILD), idiopathic interstitial pneumonia (IIP) and IPF from the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Society (ALAT) systematically reviewed the literature on IPF before May 2010 and jointly developed the first evidence-based The first evidence-based guidelines for the diagnosis and treatment of IPF (2011 guidelines) were developed. The following are the new views and understanding of the 2011 guideline to share with you.  1. Definition of IPF For the first time, the radiological manifestation of UIP type is included in the definition of IPF, and the importance of identifying the UIP type manifestation of high resolution CT (HRCT) is emphasized.  IPF is a specific type of chronic progressive fibrotic interstitial pneumonia of undetermined etiology, prevalent in the elderly, with lesions confined to the lungs and histopathological and/or imaging manifestations with UIP features. The possibility of IPF should be considered in adult patients with chronic exertional dyspnea of unknown clinical manifestation, accompanied by cough, bilateral basilar bursts and pestle fingers.  2, IPF epidemiology There are no large-scale epidemiological studies on the incidence and prevalence of IPF. However, the overall incidence of IPF shows a significant increasing trend. A study based on the population of Bernalillo County, New Mexico, USA, showed that the prevalence of IPF was estimated to be 10.7/100,000 for men and 7.4/100,000 for women. Studies from the United Kingdom have reported an overall IPF incidence of only 4.6 per 100,000, but the incidence of IPF is estimated to have increased by 11% per year between 1991 and 2003, and this increase does not appear to be related to an aging population or an increase in the diagnosis of patients with mild disease.  Based on a recent registry from a large US healthcare database, the estimated prevalence of IPF in the total US population is (14.0-42.7)100,000 and the incidence is (6.8-16.3)/100,000. It is unclear whether the incidence and prevalence of IPF are influenced by various factors such as geography, country, culture or race.  3, IPF potential risk factors ① Smoking: smoking risk and familiality are strongly associated with the development of disseminated IPF, especially smoking more than 20 packs per year.  ② Environmental exposure: IPF is associated with a variety of environmental exposures, such as exposure to metal dust (copper-zinc alloy, lead, steel), wood dust (pine), farming, stonework, polishing, hair conditioner, exposure to livestock, plant, animal dust, etc.  ③ Pathogenic microorganisms: Some studies suggest that infections, especially chronic viral infections, including EBV, hepatitis virus, cytomegalovirus, and human herpes virus, may be associated with the development of IPF.  Gastroesophageal reflux: Several studies suggest that most patients with IPF have abnormal gastroesophageal reflux, and that abnormal gastroesophageal reflux leading to recurrent microaspiration is one of the risk factors for IPF. However, most patients with IPF have “recessive reflux” and lack clinical symptoms of GERD, so they are easily overlooked.  ⑤ Genetic factors: Familial IPF is autosomal dominant and accounts for the proportion of all IPF patients.