系统性红斑狼疮肺部受累的临床表现

Pleuropulmonary manifestations of systemic lupus erythematosus

目的研究系统性红斑狼疮(SLE)肺部受累的发生率、临床表现和预后。方法回顾性分析320例SLE住院患者肺部病变的临床资料。结果320例SLE患者中肺部受累142例(发病率为44.4%)。有肺部受累者死亡10例(7.0%),无肺部受累者死亡2例(1.1%),两组比较差异有统计学意义(P<0.05)。急性狼疮性肺炎4例,发病率为1.3%,主要表现为低氧血症、呼吸困难、发热、咳嗽,影像学表现为单侧或双侧弥漫、以肺底为主的斑片状实变影,存活率100%。弥漫肺泡出血6例,发病率为1.9%,咯血、呼吸困难、低氧血症、咳嗽、贫血、血性肺泡灌洗液是其主要表现。影像学表现为新出现的双侧弥漫斑片影或实变影。其他肺部表现包括胸膜病变80例(25%),慢性间质性肺病22例(6.9%),肺动脉高压49例(15.3%),肺栓塞6例(1.9%),肺部感染61例(19.1%)。结论(1)SLE可以累及呼吸系统的任何部位,可以在疾病的任何阶段出现。(2)SLE肺部受累中危重症并不少见,肺部受累提示预后不良。(3)SLE肺部受累的临床及影像学表现可以相互重叠。

Objective To analyze the incidence, clinical features, and prognosis of pleuropulmonary involvement in patients with systemic lupus erythematosus （SLE）. Methods The medical records of 320 SLE inpatients hospitalized in Peking Union Medical College Hospital 2004, 50 males and 270 females, aged 33±14 （8-75）, were retrospectively analyzed. Results Pleuropulmonary involvement occurred in 142 cases with an incidence rate of 44.4%. The incidence of pleural involvement was 25% （80/320） and the incidence of pulmonary parenchyma involvement was 33.8% （108/320）. The mortality rate of the SLE patients with thoracic involvement was 7.0% , significantly higher than that of the SLE patients without thoracic involvement （ 1.0% , P=0.006）. The incidence of acute lupus pneumonitis （ALP） was 1.3% and the survival rate of ALP was 100%. The clinical manifestations of ALP included hypoxemia, dyspnea, fever, and cough and chest radiograph characteristically showed unilateral or bilateral patchy acinar infiltrates predominantly in the lower lung zone. The incidence of diffuse alveolar hemorrhage （DAH） was 1. 9% with a mortality rate of 66. 7%. The clinical manifestations of DAH included hemoptysis, hypoxemia, cough, anemia and bloody bronchoalveolar lavage and the chest radiographic findings included new acinar infiltrates that were typically diffuse and bilateral and might be patchy. The other common pleuropulmonary complications of SLE included pleural disease （25%） , chronic interstitial pneumonitis （6.9%）, pulmonary hypertension （ 15.3% ）, pulmonary embolism （ 1.9% ） , and pulmonary infection （19. 1% ）. Conclusion SLE may affect all components of the respiratory system. Thoracic involvement is an adverse prognostic sign. Life-threatening pulmonary involvement is not uncommon. The clinicoradiographic features of the SLE-associated pulmonary syndromes overlap. Aggressive diagnostic evaluations such as blood culture, sputum culture, fibreoptic bronchoscopy, and lung biopsy are very important.