Walz-Leblanc B A, Urowitz M B, Gladman D D, Hanly P J
University of Toronto Rheumatic Disease Unit, Wellesley Hospital, ON, Canada.
J Rheumatol. 1992 Dec;19(12):1970-2.
A 35-year-old woman had a 13-year history of systemic lupus erythematosus (SLE) with recurrent flares since 1972 responding to corticosteroid therapy. In August, 1990 she presented with a 2-month history of dyspnea at rest, 4-pillow orthopnea and paroxysmal nocturnal dyspnea. Respiratory rate was 32-36/min, chest expansion 2 cm and crackles were present at the lung bases. On chest radiograph diaphragms were elevated. Pulmonary function tests (PFT) showed further reduction in lung volumes, maximum inspiratory pressures, maximum expiratory pressures and arterial blood gases. Ventilation/perfusion and gallium lung scans were normal. A diagnosis of "shrinking lungs syndrome" was made. Treatment with 40 mg of prednisone resulted in resolution of the patient's shortness of breath. PFT showed improvement in all variables. Corticosteroid therapy for acute "shrinking lungs syndrome" in active SLE can improve symptoms and pulmonary function.
一名35岁女性自1972年起患有系统性红斑狼疮（SLE），病程长达13年，病情反复，对皮质类固醇治疗有反应。1990年8月，她出现了2个月的静息呼吸困难、4个枕头支撑的端坐呼吸和阵发性夜间呼吸困难病史。呼吸频率为32 - 36次/分钟，胸廓扩张度为2厘米，两肺底部可闻及湿啰音。胸部X线片显示膈肌抬高。肺功能测试（PFT）显示肺容积、最大吸气压力、最大呼气压力和动脉血气进一步降低。通气/灌注扫描和镓肺扫描正常。诊断为“肺萎陷综合征”。给予40毫克泼尼松治疗后，患者的呼吸急促症状得到缓解。PFT显示所有指标均有改善。对于活动性SLE中的急性“肺萎陷综合征”，皮质类固醇治疗可改善症状和肺功能。
