摘要To the Editor: A 65?year?old woman with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome was admitted to hospital because of progressive dyspnea on exertion for about 1 year. In the past, she had been diagnosed as gastroesophageal reflux disease and scleroderma for 10 years. Physical examination showed masked face, Raynaud's phenomenon, finger sclerodactyly, and skin telangiectasia on her neck and back [Figure 1a and 1b]. The titer of anticentromere antibody in serum increased to 1: 1000. Analysis of arterial gas on room air revealed the partial pressure of arterial oxygen (PaO2) was 54 mmHg (1 mmHg = 0.133 kPa) and carbon dioxide (PaCO2) was 32 mmHg. Echocardiogram showed severe tricuspid regurgitation and elevated pulmonary arterial pressure of 78 mmHg. Chest high?resolution computer tomography demonstrated diffuse mild ground?glass opacities in both lung fields [Figure 1c] and dilated central pulmonary artery [Figure 1d]. Open lung biopsy demonstrated characteristic more than two layers of capillaries on alveolar walls [Figure 1e], which was confirmed by CD34 immunohistochemical staining [Figure 1f]. Therefore, the patient was diagnosed as pulmonary capillary hemangiomatosis (PCH) associated with CREST syndrome, and treated by prednisone and ambrisentan. One month later, her symptoms obviously improved and repeated echocardiogram report showed pulmonary systolic pressure decreased to 46 mmHg.