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首页> 外文期刊>Internal medicine journal >Ventilatory failure in shrinking lung syndrome is associated with reduced chest compliance
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Ventilatory failure in shrinking lung syndrome is associated with reduced chest compliance

机译：肺萎缩综合征的通气衰竭与胸部顺应性降低有关

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摘要

Shrinking lung syndrome is an extremely rare feature of systemic lupus erythematosus (SLE). We report on a 49-year-old woman with SLE who presented with dyspnoea in type 2 respiratory failure requiring mechanical ventilation. Medical imaging investigations revealed markedly reduced lung volumes and the absence of pulmonary emboli, pulmonary fibrosis or any significant parenchymal infiltrate consistent with shrinking lung syndrome. We observed significantly reduced chest compliance during positive pressure ventilation and noted that this contrasts with a widely held view that diaphragmatic weakness is the major pathophysiological mechanism for ventilatory failure in these patients. She was treated with high-dose steroids and cyclophosphamide and weaned slowly off full mechanical ventilation. This report highlights an unusual cause of respiratory failure in a patient with SLE and provides support for reduced chest compliance rather than the diaphragmatic weakness as being the significant pathophysiological mechanism for ventilatory failure in these patients.
机译：萎缩性肺综合征是系统性红斑狼疮（SLE）极为罕见的特征。我们报告了一位49岁的SLE妇女，她因2型呼吸衰竭而出现呼吸困难，需要进行机械通气。医学影像学检查显示肺体积明显减少，并且没有肺栓塞，肺纤维化或与萎缩的肺综合征相一致的任何实质性实质浸润。我们观察到正压通气期间胸部顺应性显着降低，并指出这与广泛持有的观点相反，diaphragm肌无力是这些患者通气衰竭的主要病理生理机制。她接受了大剂量的类固醇和环磷酰胺治疗，并逐渐从完全的机械通气中断奶。该报告强调了SLE患者呼吸衰竭的异常原因，并为减少胸部顺应性而不是diaphragm肌无力提供了支持，因为这是这些患者通气衰竭的重要病理生理机制。

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