[Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis].
[Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis].
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[弥漫性全细支气管炎伴髓过氧化物酶特异性抗中性粒细胞胞浆抗体相关血管炎]。
DOI：
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发表时间：
1998
期刊：
影响因子：
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通讯作者：
K. Toyama
中科院分区：
文献类型：
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作者：
D. Miyamoto;Y. Ichinose;M. Kikawada;H. Kusumoto;N. Yanagisawa;E. Kanai;K. Minemura;M. Yonemaru;K. Toyama
一位46岁女性于1996年7月因小腿发热和肌痛被转介到我科。她有20年的脓痰病史，1983年诊断为弥漫性泛细支气管炎。体格检查发现肺部有低髓干啰音及右腿感觉迟钝。她的白色血细胞计数为16，100/mm 3，包括4%嗜酸性粒细胞，血小板计数为80.0 x 10（4）/mm 3。血清IgE水平为2，200 U/ml，冷血凝素滴度较高。肺功能检查显示混合性呼吸功能障碍，动脉血气分析显示室内空气中PaO 2为55.8 Torr。从她的痰中培养出绿脓杆菌。胸部X线片和CT扫描显示弥漫性结节影和支气管扩张改变伴轻度过度充气。右侧S6区也可见浸润性病变。使用广谱抗生素并没有缓解她的症状。血清髓过氧化物酶特异性抗神经细胞胞浆抗体（MPO ANCA）水平为245 EU/ml，67 Ga造影显示腹部有明显蓄积。腹部血管造影显示肝动脉、脾动脉、胆囊动脉和上级肠系膜动脉的外周分支呈珠状外观和不规则性。由于高MPO-ANCA水平和血管造影异常，诊断为MPO-ANCA相关性血管炎。患者接受甲基强的松龙治疗，每日1 g，持续3天，随后接受泼尼松龙60 mg和环磷酰胺50 mg每日治疗。她的病情明显改善，MPO-ANCA水平几乎恢复正常。治疗期间，她的血压显著升高，血清肌酐水平正常，尿液分析正常。血浆肾素活性为13.3 ng/ml/hr。肾血管造影显示双侧肾动脉外周分支狭窄和不规则。这些发现导致诊断为血管炎引起的肾血管性高血压。她的血压控制与血管紧张素转换酶抑制剂和钙拮抗剂。偶尔有与慢性支持性肺病相关的血管炎的报道，这表明慢性呼吸道感染与ANCA相关血管炎之间存在因果关系。全身性血管炎应被视为慢性化脓性肺病的潜在并发症。
A 46-year-old woman was referred to our department in July 1996 with complaints of fever and myalgia in her calves. She had a 20-year history of purulent sputum; diffuse panbronchiolitis had been diagnosed in 1983. Physical examination revealed low-pithed rhonchi over the lung fieldis and hypesthesia of the right leg. She had a white blood cell count of 16,100/mm3, including 4% eosinophils, and a platelet count of 80.0 x 10(4)/mm3. The serum IgE level was 2,200 U/ml, and the cold hemagglutinin titer was high. Pulmonary-function tests showed mixed ventilatory dysfunction, and arterial blood gas analysis revealed a PaO2 of 55.8 Torr on room air. Pseudomonas aeruginosa was cultured from her sputum. A chest X-ray film and CT scan showed diffuse nodular shadows and bronchiectatic changes with mild hyperinflation. An infiltrative lesion in right S6 area could also be seen. Administration of broad-spectrum antibiotics did not alleviate her symptoms. The level of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) in serum was 245 EU/ml, and 67Ga scintigraphy showed marked accumulation in the abdomen. Abdominal angiography demonstrated a bead-like appearance and irregularities in the peripheral branches of the hapatic artery, the splenic artery, the cystic artery, and the superior mesenteric artery. Because of the high MPO-ANCA level and the angiographic abnormalities, MPO-ANCA-related vasculitis was diagnosed. She was treated with 1 g of methylprednisolone daily for 3 days, followed by 60 mg of prednisolone and 50 mg of cyclophosphamide daily. Her condition improved dramatically, and the MPO-ANCA level became almost normal. During treatment, her blood pressure rose markedly with a normal serum creatinine level and normal urinalysis. Plasma renin activity was 13.3 ng/ml/hr. Renal angiography showed stenoses and irregularities in the peripheral branches of renal arteries bilaterally. These findings led to a diagnosis of renovascular hypertension due to vasculitis. Her blood pressure was controlled with an angiotensin-converting enzyme inhibitor and a calcium antagonist. Vasculitis associated with chronic supportive lung disease has occasionally been reported, which suggests a casual relation between chronic respiratory infection and ANCA-related vasculitis. Systemic vasculitis should be taken into account as a potential complication of chronic suppurative lung disease.