The patient is a 40-year-old woman who was admitted to the hospital with chest tightness and shortness of breath after activity for 1 month; physical examination was unremarkable, with slightly low respiratory sounds in both lungs, heart rate of 80 beats per minute, and no murmurs in rhythm. 3, imaging manifestations: extensive cystic shadow of both lungs, diffuse honeycomb changes in both lung fields, interspersed with diffuse hairy glass-like shadow and small thin nodule-like shadow, with multiple large pulmonary alveoli, according to the medical history, consider the possibility of pulmonary lymphangioadenomyopathy Differential diagnosis: (1) fibroalveolitis and end-stage interstitial fibrosis (honeycomb lung): its cystic air cavity diameter of 1mm ~ 2.5mm, the distribution of more irregular, thicker wall and accompanied by irregular In addition, there is also a reduction in lung volume, obvious deformation of branches and structures, and obvious peripheral subpleural changes. (2) Neurofibromatosis: cystic air spaces can also be seen in this disease, but their distribution is different from that of PLAM, with cystic air spaces located in the apical part of the lung with enhanced basal line texture. (3) Bronchiectasis: the cystic cavities are distributed along the bronchi with thick walls, often with fluid levels, and cystic shadows are rare around the lungs, which is sufficient to differentiate it from PLAM. (4)Pulmonary histiocytosis X: similar to PLAM, but the cystic shadow is accompanied by multiple nodular shadows, cavities in the nodules, nodular and reticular changes in the interstitium, and the nodular and cystic lesions are mostly located in the upper lobes of both lungs, without celiac pleural fluid. (5) Emphysema: Similar to PLAM, it is more difficult to differentiate. However, emphysema lacks lobular septal thickening, and most of the cystic hypodense areas have residual lobular structures in the center, and the central lobular arteries can be seen in the hypodense shadow; whereas LAM cystic cavities tend to be uniform in size and distribution, with clear uniform thin walls and vascular shadows located outside the edges of the cystic shadow, and generally without extra large cystic cavities (similar to pulmonary alveolar changes). The combination of clinical history, age and gender can help to differentiate. Treatment: There is no specific treatment: symptomatic and supportive treatment; hormonal therapy; lung transplantation. Prognosis: The prognosis of this disease is poor. Most patients develop progressive pulmonary dysfunction 1-2 years after diagnosis and eventually die of respiratory failure.