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本文报告一例有全身皮肤和胃肠道病变的硬皮病患者由于膈肌有病变继发肺换气不足,引起呼吸衰竭而死亡.肺部病变过去曾有报告:包括肺纤维化、肺部囊性改变和肺气肿、纤维性胸膜炎、小支气管的替代性纤维化末端动脉炎和肺钙质沉着,而膈部硬皮病则以前从未见诸文献,本文报道一例.患者,43岁,白种妇女,因硬皮病住院,住院后检查肌酐和血尿素氮均正常,肌酐廓清试验66ml/min/1.73m~2,抗DNA抗体14.1%,抗核抗体和抗核因子均阴性;肌酸磷酸激酶活性前后2次分别为312和660Iu/L(正常36～188Iu/L),患者逐渐发生过度换气的迹象,动脉血二氧化碳分压进行性升高.在此之前,其肺功能检查肺活量及一秒钟
This article reports an example of a patient with scleroderma with systemic skin and gastrointestinal lesions who died of respiratory failure due to inadequate ventilation of the diaphragm in the diaphragm and pulmonary failure. There have been reports of pulmonary lesions in the past including pulmonary fibrosis, Change and emphysema, pleuritis fibrosis, bronchial fibrosis and calcitonar pulmonary fibrosis, while the diaphragm of the scleroderma has never been seen before the literature, this article reports a case of patients, 43 years old, white The women were hospitalized for scleroderma and had normal creatinine and blood urea nitrogen after hospitalization. The serum creatinine clearance test was 66ml / min / 1.73m ~ 2, anti-DNA antibody was 14.1%, anti-nuclear antibody and anti-nuclear factor were all negative. Creatine Before and after phosphate kinase activity was 312 and 660Iu / L (normal 36 ~ 188Iu / L), the patient gradually signs of hyperventilation, arterial partial pressure of carbon dioxide increased sex. Prior to this, the pulmonary function test lung capacity And a second