系统性红斑狼疮合并肺间质病变的临床特征及预后

Clinical Characteristics and Prognosis of Systemic Lupus Erythematosus Associated with Interstitial Lung Diseases

目的探讨系统性红斑狼疮合并肺间质病变（SLE-ILD）患者的临床特征及预后。方法回顾性分析2001年1月-2006年12月在本院就诊的798例系统性红斑狼疮患者（SLE）中36例由高分辨CT（HRCT）诊断合并肺间质病变（ILD）患者的临床资料。采用Logistic回归分析患者的影像学特点、实验室检查、临床表现及药物反应对预后的影响。结果SLE患者中ILD发生率为4．5％。HRCT表现多为斑片影。对ILD的敏感程度明显高于胸部X线检查．两者符合率仅为25％。肺活检病理可见肺泡炎及间质纤维化改变。36例患者中。3例死于感染相关的呼吸衰竭，1例死于神经精神性狼疮．其余患者随访至今ILD病情稳定或稍有好转。WBC〈4．0×10^9／L或〉12．0×10^9／L且中性粒细胞比例〉75％的患者合并感染危险显著升高（P〈0．05）。感染并发症、中枢神经系统受累和呼吸衰竭是预后不良的危险因素（P〈0．05）。结论HRCT可提高SLE患者ILD病变的诊断率，激素及环磷酰胺对大部分患者有效。防治感染在SLE-ILD患者的治疗中至关重要。

Objective To investigate the clinical characteristics and prognosis of interstitial lung disease （ILD） in patients with systemic lupus erythematosus （SLE）. Methods Totally 798 SLE patients who were admitted to our hospital from Jan 2001 to Dec 2006 were analyzed retrospectively. Thirty six patients defined ILD demonstrated by high resolution computerized tomography （HRCT） were indentified. They were followed up for 1-57 months. Characteristics of imageology, laboratory tests, clinical manifestations, and response to treatment were tested by Logistic regression to analyze the prognosis. Results The prevalence of ILD with SLE was 4.5%. The sensitivity of HRCT was much higher than X-ray and the concordance of both was 25% only. The alveoli inflammation and interstitial fibrosis lesions were common in pathology. During the investigation, 30 of the patients （83.3%） were sensitive to prednisone combined cyclophosphamide treatment and had good prognosis. Four patients died. Three died of respiratory failure caused by infection, the others died of lupus encephalopathy. If the patients had infectious complications, they were associated with elder onset, high temperature, high white blood cell count and high percentage of neutrophil, and blood system involvement, as well as high C-reaction protein level （P 〈 0.05）. Poor prognosis was associated with infection complications, nervous system involvement and respiratory failure （P 〈 0.05）. Conclusions HRCT can promote the diagnostic rate of ILD in SLE. Most patients are sensitive to prednisone and cycl and ophosphamide and have good prognosis. It is vital to control infection since it leads to poor prognosis high mortality.