| 英文摘要 |
A 39-year-old woman with past history of diffuse systemic sclerosis and interstitial lung disease received steroid pulse therapy and cyclophosphamide injection in 2011. Her interstitial lung disease got progressed slowly, but patient could still live independently without oxygen supply. This patient also suffered from peripheral digits ischemia due to scleroderma related vasculopathy. She was admitted due to shortness of breath, with rapid progression into respiratory failure. Echocardiography showed severe left ventricular dysfunction with LVEF 28.5%. Extracorporeal membrane oxygenation device was provided, but increasing creatinine kinase (CK) level (>90000U/l) with decompensated heart function was noted. Patient died of profound cardiac and respiratory failure. Afterwards, partial autopsy was performed under the agreement of her family in order to clarify the etiology. Gross examination of the lung shows bilateral peripheral cystic change, especially basal lungs, compatible with interstitial lung disease. Microscopic examination of the lungs showed fibrin exudate in the alveolar space, edema and chronic inflammatory cells infiltration and alveolar septum thickening (Fig. 1). |
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