显微镜下多血管炎周围神经病变的特点

Clinical features of microscopic polyangiitis associated with peripheral neuropathy

目的分析显微镜下多血管炎（MPA）周围神经病变（PN）特点。方法回顾性分析北京协和医院2008年1月至2014年1月83例MPA中37例（44．6％）出现PN者的临床特点、治疗转归及危险因素。结果（1）37例中男15例，女22例，年龄（64＂4-9）岁，起病至确诊时间平均24．5个月。（2）PN表现：感觉异常或疼痛94．6％；肌无力或足下垂40．5％。同时累及上、下肢为56．7％，仅下肢受累为40．5％。多发性单神经病56．8％，多神经病43．2％，无颅神经受累病例。（3）单因素分析显示，MPA伴发PN者肌痛发生率高（56．8％比32．6％，P〈0．05），外周血血小板计数较高[（×10’／L），329＂4-109比245±117，P〈0．01]，红细胞沉降率较快[（mm／1h），92±29比75±28，P〈0．01]，血清白蛋白水平较低[（∥L），294-5比314-6，P〈0．05]。两组患者在年龄、性别、病程、发热、皮疹、关节炎、肺间质病变、肾脏受累、消化道受累、中枢神经系统病变、五因子评分等方面差异无统计学意义。（4）多因素分析显示，外周血血小板计数升高可能是MPA发生PN的危险因素（OR=1．005，P〈0．05）。结论MPA的PN常见，多为感觉与运动混合型，下肢受累、多发性单神经病多见。外周血小板计数升高可能是MPA发生PN的危险因素，PN在MPA患者可能不提示不良预后。

Objective To analyze clinical features of patients with microscopic polyangiitis （MPA） associated with peripheral neuropathy （PN）. Methods The clinical data of 37 （44. 6% ） hospitalized MPA patients associated with PN in Peking Union Medical College Hospital from January 2008 to January 2014 was retrospectively analyzed, using the rest 46 hospitalized MPA patients without PN as control. Results （1） Out of the 37 MPA patients with PN, there were 15 men and 22 women, with the average age of （64 ± 9 ） years old and average time between the diagnosis and onset of the disease of 24. 5 months. （2） Neurologic manifestations of these patients： mononeuritis multiplex （56. 8% ） and distal symmetrical polyneuropathy （43.2%） were the predominant peripheral nervous system manifestations. Lower limbs were more frequently affected, and mixed （sensory and motor） or sensory abnormalities were more commonly seen than single motor abnormalities. （ 3 ） Compared with MPA patients without PN, the incidence of myalgia and the count of platelet in peripheral blood, erythrocyte sedimentation rate （ESR） were statistically higher, while the serum level of albumin was statistically lower in MPA patients with PN. There were no statistical differences in age, sex, fever, rash, arthritis, interstitial lung disease, gastrointestinal lesions, renal and central nervous system involvement between two groups. （4） The multivariant Logistic regression analysis suggested that thrombocytosis was the risk factor for PN in MPA patients （ OR = 1. 005, P 〈0. 05 ）. Conclusions PN is common in MPA patients. Mononeuritis multiplex and distal symmetrical polyneuropathy, mixed neuropathy, and lower limbs involvement are predominant peripheral neurologic manifestations. Thrombocytosis may be one of the risk factors of PN in MPA patients, and PN may have minor effect on the prognosis of MPA.