Idiopathic pulmonary fibrosis (IPF) is a clinically distinct disease that is characterized by progressive dyspnea and irritating cough, ches
Idiopathic pulmonary fibrosis (IPF) is a clinically distinct disease that is characterized by progressive dyspnea and irritating cough, chest X-ray showed bilateral lower lung reticular opacities, lung function and restrictive ventilatory disorder, of unknown cause, the disease continued to progress, eventually died of respiratory failure and death.
Idiopathic pulmonary fibrosis (IPF) is a clinically distinct disease that is characterized by progressive dyspnea and irritating cough, chest X-ray showed bilateral lower lung reticular opacities, lung function and restrictive ventilatory disorder, of unknown cause, the disease continued to progress, eventually died of respiratory failure and death. The disease is an independent and highly lethal disease. The prognosis is the same as that of lung cancer, which needs to be further studied to reveal the etiology and pathogenesis of .
First, the understanding of the progress of IPF
In 1935 Hamman and rich first reported 4 cases of patients with severe dyspnea, cyanosis, died in the second half of this year, was published in 1949 4 cases of pulmonary pathological changes, and named the acute diffuse interstitial pulmonary fibrosis. In 1975 Liebow proposed desquamative interstitial pneumonia according to the pathological characteristics (DIP), interstitial pneumonia (UIP) the pathological characteristics of the IPF will be divided into 5 categories :uip, dip, bronchiolitisassociated interstitial pneumonia / bronchiolitis obliterans with interstitial lung disease (BIP) or bronchiolitis obliterans organizing pneumonia (BOOP), lymphoid interstitial pneumonia (lip) and giant cell interstitial pneumonia. It is concluded that dip and UIP may be the different stages of the course of IPF, and that dip is the early stage of alveolar inflammation, while UIP is the stage of alveolar septum and interstitial fibrosis. Some people think that, according to the type of infiltrating cells and the pathological changes of IFP should be divided into different types with dip or UIP as two, due to some differences on prognosis and response to treatment in 1998, Katzenstein proposed a new classification of IPF, divided into 4 categories: UIP, dip, acute interstitial pneumonia (AIP) and nonspecific interstitial pneumonia (NSIP). This includes the classical UIP and dip as well as the usual mentioned AIP (or Hamman-Rich syndrome), which increases the newly proposed nsip. The pathological features of UIP, NSIP, dip and AIP in the IPF classification proposed in 1998 are summarized as follows: [2, 3], see table 1.
Recently, the American Thoracic Society (ATS) and the European Respiratory Society (ERs) proposed new international consensus on the diagnosis of IPF:
1.uip is consistent with the pathological types of IPF, and dip, the respiratory bronchioles associated interstitial lung disease (RBILD), NSIP, lip, AIP and idiopathic bronchiolitis obliterans with interstitial pneumonia (idiopathic BOOP) for different diseases, and should be excluded from ipf.
2 IPF should have a specific clinical diagnostic criteria and should be differentiated from other diffuse parenchymal lung diseases.
3 patients with suspected IPF, such as those with no typical IPF findings in clinical, physiological, or radiological features, and without surgical indications, should be treated with surgical lung biopsy. The main purpose of histopathology is UIP and the other on appropriate treatment has a better response to the organization of NSIP type identification .
Two, IPF definition and diagnostic criteria
IPF is defined as a special type of chronic fibrous interstitial pneumonia confined to the lungs, and surgical biopsy (through thoracoscopy or thoracotomy) presents a histological change of uip. IPF etiology is unknown, such as the following aspects can be clearly diagnosed ipf:
1 interstitial lung disease, other than known causes of interstitial lung disease, such as drug side effects, environmental factors, and collagen vascular disease.
2 pulmonary function abnormalities including restrictive ventilation dysfunction (decreased with increase in FVC forced expiratory volume in 1 second / FVC ratio) and (or) gas with resting or exercise exchange dysfunction (Alveolar arterial oxygen pressure difference increase), or lung carbon monoxide diffusion function (DLCO) decreased.
3 routine chest X-ray or chest high resolution CT (HRCT): a typical manifestation of abnormal CXR for peripheral lung area reticular opacities, mainly occurred in the basal part of the lung. These reticular shadows are usually bilateral, asymmetrical and often accompanied by a reduction in lung volume. The chest HRCT mainly showed patchy shadow, accompanied by varying degrees of frosted glass like shadow, its scope is relatively limited. In the more severe lesion area, there is a common manifestation of traction bronchiectasis and (or) the formation of the pleural honeycomb lung.
In the absence of surgical lung biopsy, the diagnosis of IPF remains unclear. But as with all of the following major diagnostic criteria, and 4 items to 3 items in the standard, the correctness of the clinical diagnosis of IPF significantly increased .
Main diagnostic criteria: (1) except for the known causes of interstitial lung disease (ILD), such as the toxic effects of certain drugs, environmental pollution and ild. (2) abnormal pulmonary function changes: restrictive ventilatory dysfunction and (or) gas exchange disorder. (3) the reticular shadow at the bottom of bilateral lung, with a little change of glass like appearance on hrct. (4) evidence of no other disease by transbronchial lung biopsy or bronchoalveolar lavage (BAL).
Secondary diagnostic criteria: (1) age greater than the age of 50. (2) difficulty in breathing after the onset of the disease, and is not explained. (3) disease duration > 3 months. (4) bilateral lungs with audible and inspiratory crackles (dry or Velcro).
ATS and ers emphasize the importance of surgical lung biopsy in the diagnosis of IPF, and suggest that surgical lung biopsy (open lung biopsy or video-assisted thoracoscopic lung biopsy) can provide the best lung tissue samples. Surgical lung biopsy can identify the type of ILD that is easier to treat and determine its efficacy. Of course, surgical lung biopsy should be carefully used to weigh the relationship between potential risk and correct diagnosis. If the patient's age >, 75 years old, obesity, heart disease and severe pulmonary function damage, surgical complications may increase. However, there are relatively few complications of video-assisted thoracoscopic lung biopsy.