Owens G R, Fino G J, Herbert D L, Steen V D, Medsger T A, Pennock B E, Cottrell J J, Rodnan G P, Rogers R M
Chest. 1983 Nov;84(5):546-50. doi: 10.1378/chest.84.5.546.
The pulmonary function and chest roentgenograms were evaluated in 88 patients with the CREST syndrome variant of progressive systemic sclerosis (PSS or scleroderma). Seventy-two percent of the patients had abnormal pulmonary function. An isolated decrease in diffusing capacity was the most common abnormality noted, followed by restrictive abnormalities and airway obstruction. Chest roentgenograms revealed interstitial infiltrates consistent with pulmonary fibrosis in 33 percent. When compared to a contemporaneous group of 77 patients with PSS and diffuse scleroderma, patients with the CREST syndrome had similar abnormalities on pulmonary function testing and chest roentgenogram. However, patients with the CREST syndrome had a lower mean diffusing capacity despite a higher mean vital capacity; this combination of findings suggests primary pulmonary vascular disease. Calcified granulomata were identified significantly more often in PSS-CREST patients, while superior rib notching occurred exclusively in patients with PSS and diffuse scleroderma. The CREST variant of PSS is associated with frequent roentgenographic and pulmonary function abnormalities similar to those seen in PSS with diffuse scleroderma.
对88例进行性系统性硬化症（PSS或硬皮病）的CREST综合征变异型患者的肺功能和胸部X线片进行了评估。72%的患者肺功能异常。最常见的异常表现为单纯弥散功能降低，其次是限制性异常和气道阻塞。胸部X线片显示33%的患者有与肺纤维化一致的间质浸润。与同期的77例PSS和弥漫性硬皮病患者相比，CREST综合征患者在肺功能测试和胸部X线片上有相似的异常表现。然而，CREST综合征患者尽管平均肺活量较高，但平均弥散功能较低；这些结果表明存在原发性肺血管疾病。PSS-CREST患者中钙化肉芽肿的检出率明显更高，而上肋骨切迹仅见于PSS和弥漫性硬皮病患者。PSS的CREST变异型与常见的X线和肺功能异常有关，类似于弥漫性硬皮病的PSS所见。
