显微镜下多血管炎合并自身免疫性溶血性贫血的临床特点及治疗转归

Clinical characteristics and treatment outcomes of microscopic polyangiitis combined with autoimmune hemolytic anemia

目的:探讨合并自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)的显微镜下多血管炎(microscopic polyangiitis,MPA)患者的临床表现、实验室指标特点、治疗和预后情况,以提高对本病的认识及诊疗水平。方法:收集2006年10月至2013年11月在北京大学人民医院住院的MPA患者的临床资料,回顾性分析合并AIHA的MPA患者临床特点、治疗转归及预后。结果:(1)在63例MPA患者中,12.7%(8/63)合并AIHA。(2)合并AIHA的MPA患者与单纯MPA患者比较,发热、乏力、血尿、新发高血压的发生率明显增高(87.5%vs.29.1%;100%vs.49.1%;100%vs.60%;75%vs.20%,P均<0.05);实验室指标中血红细胞、血红蛋白、血清白蛋白、补体C3指标明显减低[分别为(2.3±0.5)×1012/L vs.(3.0±0.7)×1012/L;(66.2±13.1)g/L vs.(90.0±20.3)g/L;(26.1±4.4)g/L vs.(33.5±6.4)g/L;(0.7±0.2)g/L vs.(0.9±0.3)g/L,P均<0.05];红细胞沉降率、Ig G及伯明翰血管炎评分明显增高[(102.1±25.7)mm/1h vs.(76.5±31.1)mm/1h;(20.9±6.1)g/L vs.(14.5±6.0)g/L;(23.7±5.7)vs.(17.3±4.1),P均<0.05],提示患者更易并发多系统损害。(3)8例合并AIHA的MPA患者中,6例使用糖皮质激素冲击联合免疫制剂,2例使用小剂量糖皮质激素治疗;1例治疗过程中死亡,7例贫血症状缓解,伯明翰血管炎评分减低。(4)随访4年,7例合并AIHA患者中2例死亡,2例因贫血、肾功能指标异常反复住院,3例病情缓解。结论:MPA合并AIHA在临床中可能并不少见,患者更易并发多系统损害;糖皮质激素冲击联合免疫抑制剂治疗短期疗效较好。

Objective： To investigate the clinical characteristics,laboratory features,outcome and prognosis of microscopic polyangiitis（ MPA） patients with autoimmune hemolytic anemia（ AIHA）.Methods： In this study,63 cases diagnosed as MPA from October 2006 to November 2013 in Peking University People＇s Hospital were reviewed. The clinical characteristics,outcome and prognosis of MPA patients with AIHA were retrospectively analyzed. Results： There were 12. 7%（ 8 /63） MPA patients with AIHA. The patients with AIHA had higher prevalence of fever,fatigue,hematuresis,albuminuria and new-onset hypertension,compared with non-AIHA group（ 87. 5% vs. 29. 1%; 100% vs. 49. 1%; 100%vs. 60%; 75% vs. 20%,all P〈0. 05）. In patients with AIHA,there were significantly lower levels of red blood cell（ RBC）,hemoglobin（ Hb）,alanine albumin（ Alb） and complement C3 [（ 2. 3 ± 0. 5） ×10^12/ L vs.（ 3. 0 ± 0. 7） × 10^12/ L;（ 66. 2 ± 13. 1） g / L vs.（ 90. 0 ± 20. 3） g / L;（ 26. 1 ± 4. 4） g / L vs.（ 33. 5 ± 6. 4） g /L;（ 0. 7 ± 0. 2） g /L vs.（ 0. 9 ± 0. 3） g /L,all P〈0. 05]. However,the levels of erythrocyte sedimentation rate（ ESR）,Ig G and Birmingham Vasculitis Activity Score（ BVAS） were higher,compared with those in the patients without AIHA [（ 102. 1 ± 25. 7） mm /1h vs.（ 76. 5 ± 31. 1） mm /1h;（ 20. 9 ± 6. 1） g /L vs.（ 14. 5 ± 6. 0） g /L;（ 23. 7 ± 5. 7） vs.（ 17. 3 ± 4. 1）,all P〈0. 05]. The study suggested that there were more severe multi-system damage in the patients with AIHA than in those without AIHA. In the MPA patients with AIHA,six cases received treatment of methylprednisolone combined with immunosuppressant,and two received treatment of methylprednisolone only. Among the eight cases,three were recovered from anemia and four improved. One died of severe lung infection. After 4 years follow-up,in the seven MPA patients with AIHA,three received remission,two died,and two suffered from anemia and abnormal renal function. Conclusion： MPA with AIHA is more complicated by multi-system damage and not rare in clinical settings. Glucocorticoid combined with immunosuppressant is beneficial to induce remission for MPA patients with AIHA.