Primary desiccation syndrome is a systemic autoimmune disease with exocrine gland involvement as the main manifestation. Although exocrine gland involvement is the main hallmark, systemic involvement outside the gland can be seen in 2/3 of patients, and the lung is one of the most commonly affected target organs due to its richness in blood vessels and connective tissue, and its incidence fluctuates from 9-75% depending on the means of diagnosis. In the largest published study of patients with primary dry syndrome (1,010), it was found that 11% of patients had pulmonary involvement at the time of initial diagnosis of dry syndrome, and the proportion of pulmonary involvement increased to 19% after 10 years of follow-up. However, screening of pSS patients without respiratory symptoms revealed alveolar lavage fluid suggestive of alveolitis in 55% of patients, and pulmonary function abnormalities could be as high as 80%. This suggests that subclinical pulmonary involvement is very common in patients with pSS. Although pulmonary involvement is relatively mild and stable in most patients with pSS, it can also deteriorate dramatically. In a study of 30 pSS patients followed for pulmonary function, pulmonary involvement was found to increase over the years, and pulmonary function such as TLCO progressively deteriorated. Twenty-two were eventually followed to year 10, with one patient dying of pulmonary complications. Another survival analysis found that lung involvement severely affected the prognosis of the patients, with the 5-year survival rate having fallen to 84% and less than 20% at 15 years of follow-up. This shows that pulmonary involvement is a common and serious comorbidity in pSS patients. There are many types of pulmonary involvement in patients with dry syndrome, and multiple types of pulmonary involvement can be seen in a single patient, mostly in the lower lobes. The types of pulmonary involvement are mainly linear, ground glass, lattice-like shadow and pleural involvement. Lung pathology is the gold standard for diagnosis, but given that it is an invasive test, most people have difficulty in accepting it, so it is not much used clinically. HRCT and pulmonary function are more sensitive diagnostic methods, and we believe that these two examinations should be used as routine screening methods for patients with desiccation syndrome, and they can be used as monitoring indicators for the condition monitoring and efficacy evaluation of patients with pulmonary involvement. The current treatment for dry syndrome focuses on the use of topical drugs to improve the symptoms of dry mouth and eyes, and the topical drugs (artificial tears, artificial saliva and salivary agents, etc.) have been proved to be safe and effective. Hormones and disease-modifying anti-rheumatic drugs (DMARDs) are used to treat extraglandular damage. Current studies have shown that glucocorticoids, cyclophosphamide and hydroxychloroquine are effective in improving pulmonary involvement, but liver and kidney function, blood picture, fundus and blood glucose and other adverse drug reactions should be closely monitored to adjust the drug treatment plan. Biologic agents such as rituximab can be used as salvage therapy for refractory patients. Given that each patient’s response to each DMARDs drug is different, individualized dosing should be performed and patients should be closely monitored for changes in condition and adverse drug reactions.