显微镜下多血管炎合并肺部疾病患者的临床特点分析

Clinical features of microscopic polyangiitis with pulmonary involvement

目的探讨显微镜下多血管炎(MPA)合并肺部疾病患者的临床特点。方法选取2009年6月至2018年7月首都医科大学附属北京安贞医院收治的MPA合并肺部疾病患者60例,回顾性分析患者的一般资料、临床表现、实验室检查和胸部CT、肺功能、肾脏病理、治疗及转归等临床资料。结果 60例患者中男38例、女22例,发病年龄40～88岁,平均(69±11)岁,30例以肺部症状为首发表现,临床表现为咳嗽、咳痰42例,咯血13例,呼吸困难24例。抗髓过氧化物酶-抗中性粒细胞胞质抗体(ANCA)阳性率为91. 7%(55/60),抗蛋白酶3-ANCA阳性率为11. 7%(7/60)。影像学表现为网格影者40例,磨玻璃影者35例,条索、斑片影者30例,胸膜增厚、胸腔积液各11例,结节影8例,典型普通型间质性肺炎3例。25例患者行肺功能检查,弥散功能障碍24例,限制性通气功能障碍7例,肺动脉高压10例(19. 2%),平均肺动脉收缩压为(50±10) mm Hg(1 mm Hg=0. 133 k Pa)。48例接受治疗,34例患者应用糖皮质激素联合环磷酰胺治疗,14例患者单用糖皮质激素治疗,其中17例接受甲泼尼龙冲击治疗。除3例患者合并肺部感染致呼吸衰竭、感染性休克死亡外,其余患者均好转出院。结论 MPA肺部受累常见,肺部症状可以为首发表现,临床表现无特异性,磨玻璃影及肺间质改变为主要影像学异常,行ANCA检测及组织病理学检查有助于早期诊断、早期治疗。

Objective To analyze the clinical characteristics of pulmonary involvement in patients with microscopic polyangiitis(MPA).Methods Clinical data of 60 cases of MPA with pulmonary involvement admitted to Beijing Anzhen Hospital,Capital Medical University from June 2009 to July 2018 were retrospectively reviewed.General information,clinical manifestations,laboratory results,chest CT,pulmonary function,renal pathology,therapeutic choice and outcomes were analyzed.Results There were 38 males and 22 females;the onset age was 40-88 years and the mean age was(69±11)years.Pulmonary involvement was the initial manifestation in 30 patients.Common symptoms were cough and expectoration(42 cases),haemoptysis(13 cases)and dyspnea(24 cases).Positive rate of antimyeloperoxidase-antineutrophil cytopasmic antibody(ANCA)was 91.7%(55/60).Positive rate of antiproteinase-3-ANCA was 11.7%(7/60).Chest CT manifestations consisted of reticular markings(40 cases),ground glass opacity(35 cases),patchy infiltration(30 cases),pleural thickening(11 cases),pleural effusion(11 cases),nodules(8 cases)and usual interstitial pneumonitis(3 cases).The most frequent abnormality of lung function test was reduced diffusing capacity for carbon monoxide(24/25)and restrictive ventilation dysfunction(7/25).Incidence of pulmonary hypertension was 19.2%(10/52)and the mean pulmonary arterial systolic pressure was(50±10)mm Hg.In 48 patients receiving medical treatments in hospital,34 patients had glucocorticoids plus cyclophosphamide and 14 patients were treated with glucocorticoids;17 patients had high dose methylprednisolone pulse therapy.Three patients died of pulmonary infection-induced respiratory failure and infectious shock;the other patients showed good therapeutic response.Conclusions Lung lesion is a common comorbidity and frequent initial manifestation in patients with MPA.Clinical manifestations are nonspecific;typical CT manifestations are ground glass opacity and pulmonary interstitial abnormality.Serum ANCA and histopathologic biopsy can help diagnose MPA with pulmonary involvement.