Interstitial lung disease(ILD) and pulmonary hypertension(PH) caused by connective tissue disease(CTD) are one of the main causes of morbidity and mortality in patients. Although International Heart and Lung Transplant Society (ISHLT) suggested that ILD and PH related to CTD are rare indications for lung transplantation(LTx) in 2006, many LTx centers are concerned that the multi-system involvement of CTD will affect the survival outcome after lung transplantation, and CTD is regarded as a relative contraindication for LTx. However，long-term and short-term survival after LTx in CTD patients is similar to that in common indications for LTx such as idiopathic pulmonary fibrosis(IPF)，and no higher complications after transplantation in many lung transplant centers. This suggests that LTx may be beneficial for CTD patients who progress to end-stage lung disease, and CTD patients should not be considered a contraindication to LTx. In the future, more prospective studies are needed to analyze the risk factors of LTx in CTD patients to improve survival and reduce the risk of complications. This narrative review summarizes the selection and evaluation of candidates for CTD before LTx, and describes the clinical outcome of CTD after LTx in large-capacity lung transplantation center, the purpose is to help rheumatologists decide when to refer patients with CTD lung involvement to an LTx center and the conditions to consider before transplantation, and to provide confidence to lung transplant experts. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved.