弥漫性肺泡出血在系统性红斑狼疮和ANCA相关性血管炎的临床特征比较分析

Comparative Analysis of the Clinical Features of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus and ANCA-Related Vasculitis

摘要: 目的：探讨弥漫性肺泡出血(DAH)患者在系统性红斑狼疮(SLE)和ANCA相关性血管炎(AAV)的临床特征和预后比较分析。方法：选取2012年1月至2019年12月就诊于青岛大学附属医院的SLE-DAH和AAV-DAH的病例，对两组患者的临床特征进行回顾性对比分析。结果：DAH患者共计42例，其中SLE-DAH组患者22例，AAV-DAH组患者20例，两组患者在性别(χ² = 23.100, P = 0.000)、年龄(t = −2.625, P = 0.015)、原发病确诊到DAH病程时长(z = −2.051, P = 0.040)、C3下降值(z = −5.001, P = 0.000)、血WBC计数(z = −2.166, P = 0.030)、抗SSA/SSB抗体(χ² = 12.099, P = 0.001)、抗心磷脂抗体测定(χ² = 8.984, P = 0.003)、Coomb’s试验(χ² = 8.984, P = 0.003)、胸腔积液(χ² = 4.582, P = 0.032)以及多浆膜腔积液(χ² = 6.018, P = 0.014)，比较有统计学意义(P < 0.05)；咯血或痰中带血、呼吸困难、发热、肺部听诊啰音、肺部感染、低蛋白血症、PLT计数、24小时尿蛋白升高值、动脉血氧分压值无统计学意义(P > 0.05)。SLEDAI评分为：12~27，平均17.91 ± 4.40，伯明翰系统性血管炎活动评分(BVAS)为：15~36，平均21.30 ± 5.71。两组患者使用大剂量激素冲击治疗、环磷酰胺、吗替麦考酚酯、机械通气、血浆置换等治疗，死亡率皆小于50%，但患者死亡率比较无统计学意义(P > 0.05)。结论：弥漫性肺泡出血是系统性红斑狼疮和ANCA相关性血管炎都可以出现的一种危重并发症，但是在两种不同的自身免疫性疾病之间又有不完全一致的表现形式。在临床诊疗过程中，若患者存在咯血、呼吸困难、血红蛋白急骤下降等表现时应考虑弥漫性肺泡出血可能，也要考虑继发于自身免疫病的情况。早期诊断、早期治疗对患者的生存率至关重要。

Abstract: Objective: To investigate the clinical features and prognosis of diffuse alveolar hemorrhage (DAH) in patients with systemic lupus erythematosus (SLE) and ANCA-related vasculitis (AAV). Methods: The SLE-DAH and AAV-DAH cases in the Affiliated Hospital of Qingdao University from January 2012 to December 2019 were selected, and the clinical diagnosis and treatment characteristics of the two groups of patients were retrospectively compared and analyzed. Results: There were 42 patients with DAH, including 22 patients in the SLE-DAH group and 20 patients in the AAV-DAH group. In the two groups of patients, gender (χ2 = 23.100, P = 0.000), age (t = −2.625, P = 0.015), the length of time from the diagnosis of the primary disease to the course of DAH (z = −2.051, P = 0.040), C3 decline (z = −5.001, P = 0.000), blood WBC count (z = −2.166, P = 0.030), anti-SSA/SSB antibodies (χ² = 12.099, P = 0.001), anti-cardiolipin antibody determination (χ2 = 8.984, P = 0.003), Coomb’s test (χ² = 8.984, P = 0.003), pleural effusion (χ² = 4.582, P = 0.032), and more Serous effusion (χ² = 6.018, P = 0.014) are statistically significant (P < 0.05); Hemoptysis or blood in sputum, dyspnea, fever, pulmonary auscultation rales, lung infection, hypoalbuminemia, PLT count, 24-hour urine protein elevation, and arterial partial pressure of oxygen were not statistically significant (P > 0.05). The SLEDAI score was 12~27, with an average of 17.91 ± 4.40, and the Birmingham Systemic Vasculitis Activity Score (BVAS) was 15~36, with an average of 21.30 ± 5.71. The two groups of patients were treated with high-dose glucocorticoid shock therapy, cyclophosphamide, mycophenolate mofetil, mechanical ventilation, plasma exchange and other treatments. The mortality rate was less than 50%, but the mortality rate of the patients was not statistically significant (P > 0.05). Conclusion: Diffuse alveolar hemorrhage is a critical complication that can occur in both systemic lupus erythematosus and ANCA-associated vasculitis, but there are incompletely consistent manifestations between two different autoimmune diseases. In the course of clinical diagnosis and treatment, if the patient has hemoptysis, dyspnea, sudden drop in hemoglobin, etc., the possibility of diffuse alveolar hemorrhage should be considered, and the situation secondary to autoimmune disease should also be considered. Early diagnosis and early treatment are critical to the survival rate of patients.