关键词: 显微镜下多血管炎, 贫血, 溶血性, 自身免疫性, 体征和症状

Abstract:

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 ）×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,all P<0.05］. However, the levels of erythrocyte sedimentation rate (ESR), IgG 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.

Key words: Microscopic polyangiitis, Anemia, hemolytic, autoimmune, Signs and symptoms