Connective tissue diseases (CTD) are a group of systemic autoimmune diseases that involve a variety of organs. Because the lungs and pleura are rich in collagen, blood vessels, and other connective tissues, most connective tissue diseases can damage multiple organs of the respiratory system, including the respiratory muscles, pleura, pulmonary vessels, airways, lung parenchyma, and interstitial lung, and some patients have respiratory manifestations as the first symptoms. About 1/4 or more of these patients with connective tissue disease (CTD) will develop connective tissue disease-associated interstitial lung disease (CTD-ILD). Interstitial lung disease (ILD) is one of the important causes of death in patients with connective tissue disease (CTD) and one of the difficulties for physicians in clinical diagnosis and treatment of CTD-ILD at present. Tian Mei from the Department of Pulmonary Diseases, Affiliated Hospital of Shandong University of Traditional Chinese Medicine presented new advances in the diagnosis and treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD) at the 19th National Academic Conference on Rheumatology of the Chinese Medical Association on June 28, 2014, Prof. Ulrich Costabel from the Pulmonary Hospital of the University of Duisburg-Essen, Germany, and proposed The concept of differential management was introduced. In terms of diagnosis, Prof. Ulrich Costabel suggested that, first of all, it is necessary to confirm the progression of CTD-ILD, which is currently detected mainly by using high resolution CT (HRCT). The early stage of CTD-ILLD is the inflammatory stage, and HRCT shows a ground glass, fine grid shadow; as the disease progresses the middle stage of CTD-ILD may start to show fibrosis, and HRCT shows a coarse grid, and there may be a little foveal manifestation under the pleura; in the late stage of disease progression CTD-ILD may show extensive fibrosis, and HRCT shows significant foveal manifestation in the whole lung. Second, the acute exacerbation of CTD-ILD needs to be observed and monitored, and the current statistics suggest that the acute exacerbation rate of CTD-ILD is 3%/year. In terms of treatment, Prof. Ulrich Costabel suggests that patients with CTD-ILD need to be managed differently: for asymptomatic patients with CTD-ILD with normal lung function and a small amount of scattered fibrosis on CT: the recommended treatment plan is regular observation of symptoms and lung function tests. For patients with chronically progressive CTD-ILD with impaired lung function: for patients with pathology showing NSIP, the recommended treatment regimen is prednisone combined with azathioprine, with conversion to cyclophosphamide CTX if there is no response; for patients with pathology showing UIP, consider the recommended treatment regimen of prednisone and an immunosuppressant, but to some extent there may be no response to therapy if there is no response, pirfenidone, the only currently approved therapeutic agent with anti-pulmonary fibrosis effects, may be considered. For patients with CTD-ILD presenting with acute invasive lung injury (mainly PM/DM and RA): if the pathologic presentation suggests mechanized pneumonia or cytosolic NSIP, the recommended treatment regimen is early oral high-dose prednisone, tapered after stabilization of symptoms. For patients with diffuse alveolar injury/acute onset CTD-ILD (presenting with acute respiratory failure): the recommended regimen is high-dose methylprednisolone shock therapy (500-1000 mg i.v.) combined/not combined with sedation of CTX.