Chausow A M, Kane T, Levinson D, Szidon J P
Am Rev Respir Dis. 1984 Jul;130(1):142-4. doi: 10.1164/arrd.1984.130.1.142.
A patient with scleroderma presented with hypercapnic respiratory failure. Evaluation of pulmonary mechanics revealed severe restriction caused in part by respiratory muscle weakness. Treatment with prednisone corrected hypoventilation, improved symptoms, increased lung volumes, returned respiratory muscle strength to normal range, but did not change the degree of lung stiffness. This case demonstrates that restrictive patterns in scleroderma can be due to either lung or chest wall disease and that the latter may be reversible. If respiratory muscle weakness is present with restrictive ventilatory patterns in patients with scleroderma, a therapeutic trial of corticosteroid is warranted.
一名硬皮病患者出现高碳酸血症性呼吸衰竭。肺力学评估显示存在严重的限制性通气障碍，部分原因是呼吸肌无力。泼尼松治疗纠正了通气不足，改善了症状，增加了肺容量，使呼吸肌力量恢复到正常范围，但并未改变肺硬度。该病例表明，硬皮病中的限制性通气模式可能是由肺部或胸壁疾病引起的，且后者可能是可逆的。如果硬皮病患者在存在限制性通气模式的同时伴有呼吸肌无力，进行皮质类固醇治疗试验是必要的。
