To the Editor： A 65-year-old woman with calcinosis, Raynaud＆#39;s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia （CREST） syndrome was admitted to hospital because of progressive dyspnea on e...To the Editor： A 65-year-old woman with calcinosis, Raynaud＆#39;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.展开更多
Background Pulmonary capillary hemangiomatosis （PCH） is a rare disease and no Chinese case has been reported yet. The disease is often misdiagnosed and its clinical characteristics are incompletely described. The ai...Background Pulmonary capillary hemangiomatosis （PCH） is a rare disease and no Chinese case has been reported yet. The disease is often misdiagnosed and its clinical characteristics are incompletely described. The aim of this study was to describe two Chinese cases and to clarify the clinical and radiographic parameters of patients with PCH. Methods Two PCH cases were presented and other cases were searched from the English literature. All available clinical and radiographic data were collected from 62 literature reported PCH cases. A pooled analysis of total 64 cases was made. Results Dyspnea and hemoptysis were the most common clinical symptoms of PCH. Pulmonary hypertension （PH） was found in 78% of the reported cases. PCH typically showed characteristic diffuse or patchy ground-glass opacities （GGOs） and/or multiple ill-defined centrilobular nodules in the computed tomography. Conclusions The diagnosis of PCH requires a high clinical suspicion. However, both clinical presentations and radiographic studies often provide clues to the diagnosis, which may prompt early lung biopsy for a definite diagnosis.展开更多
文摘To the Editor： A 65-year-old woman with calcinosis, Raynaud＆#39;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.
基金This work was supported by a grant from the Major International Joint Research Project of Natural Science Foundation of China （No. 30810103904）.
文摘Background Pulmonary capillary hemangiomatosis （PCH） is a rare disease and no Chinese case has been reported yet. The disease is often misdiagnosed and its clinical characteristics are incompletely described. The aim of this study was to describe two Chinese cases and to clarify the clinical and radiographic parameters of patients with PCH. Methods Two PCH cases were presented and other cases were searched from the English literature. All available clinical and radiographic data were collected from 62 literature reported PCH cases. A pooled analysis of total 64 cases was made. Results Dyspnea and hemoptysis were the most common clinical symptoms of PCH. Pulmonary hypertension （PH） was found in 78% of the reported cases. PCH typically showed characteristic diffuse or patchy ground-glass opacities （GGOs） and/or multiple ill-defined centrilobular nodules in the computed tomography. Conclusions The diagnosis of PCH requires a high clinical suspicion. However, both clinical presentations and radiographic studies often provide clues to the diagnosis, which may prompt early lung biopsy for a definite diagnosis.
