抗中性粒细胞胞浆抗体阳性与阴性在Churg Strauss综合征患者中临床表现的差异

The Difference of Clinical Manifestation in Patients of Churg Strauss Syndrome Between the Patients with Antineutrophil Cytoplasmic Antibody Positive and Negative

目的比较血清中抗中性粒细胞胞浆抗体(ANCA)阳性与阴性在Churg Strauss综合征患者中临床表现的不同,结合文献探讨ANCA及嗜酸粒细胞增多在Churg Strauss综合征中的意义。方法对北京协和医院2001年1月至2010年11月收治的25例Churg Strauss综合征患者的临床资料进行回顾性分析。结果 Churg Strauss综合征患者ANCA阳性率为32.0%,以P-ANCA为主;ANCA阳性和ANCA阴性患者肺受累发生率分别为16.7%和82.4%,两者比较有显著差异(P<0.05);心脏受累发生率分别为60.0%和64.2%(P>0.05);外周神经受累发生率分别为85.7%和66.7%(P>0.05);肾脏受累发生率分别为75.0%和35.3%(P>0.05)。结论 Churg Strauss综合征依ANCA的状态不同可能存在不同亚型,在临床表现上也有一定的差异。

Objective To clarify the differences of clinical manifestations in patients of Churg Strauss syndrome（CSS） with or without antineutrophil cytoplasmic antibody（ANCA） positive,and primarily explore the roles of ANCA and eosinophilia in CSS according to current literatures.Methods The clinical data of 25 patients with CSS,admitted to Peking Union Medical College Hospital from January 2001 to November 2010,were retrospectively investigated.Results The positive rate of ANCA in Churg Strauss was 32.0%,with P-ANCA predominantly（in 6 patients）.The lung involvement in the patients between ANCA positive and ANCA negative patients was 16.7% and 82.4% respectively with significant difference.The heart involvement in the patients between ANCA positive and ANCA negative patients were 60.0% and 64.2%,respectively.The peripheral neuropathy between ANCA positive and ANCA negative patients was 85.7% and 66.7%,respectively.The renal manifestations between ANCA positive and ANCA negative patients were 75.0% and 35.3%,respectively.But the differences in heart,peripheral neuropathy,or renal manifestations were not significant.Conclusion The status of ANCA may represent different clinical phenotype of CSS.