摘要 目的 了解肺脏受累的原发性小血管炎的临床特点。方法 对1993～1998年间13例肺脏受累的原发性小血管炎进行回顾性分析。结果 13例中7例为显微镜下多血管炎(MPA)，6例为Wegener肉芽肿(WG)。发病年龄17～68岁，平均48.8岁。抗中性粒细胞胞浆抗体(ANCA)阳性率69%(9/13)例，MPA的ANCA阳性率为100%(7/7)，WG为33%(2/6)。肺部症状包括咯血占69%(9/13)、呼吸困难占23%(3/13)、干咳占15%(2/13)、胸痛占15%(2/13)。胸部X线在MPA多表现为多发斑片状影(3/7)或大片状阴影(2/7)，WG则主要是单发或多发团块影或结节影(5/6)，可有空洞形成。MPA的肺部表现多误诊为肺部感染71%(5/7)。WG多误诊为肿瘤67%(4/6)。使用肾上腺皮质激素及环磷酰胺(CTX)治疗有效，尤其肺部损害可明显吸收。结论 伴肺脏受累的原发性小血管炎诊断困难，须引起注意。ANCA对MPA的诊断有重要价值。激素及CTX对该组疾病疗效显著。

    

Abstract Objective To investigate the clinical characteristics ofprimary small vessel vasculitis with involvement of lungs. Methods 13 cases of primarysmall vessel vasculitis with involvement of lungs from 1993 to 1998 were analyzedretrospectively. Results Among 13 cases 7 were microscopic polyangiitis (MPA) and 6 wereWegener granulomatosis (WG). The ages of onset were from 17 to 68 years old with averageage 48.8 years old. 69%(9/13) were ANCA positive, among them 100%(7/7) MPA were ANCApositive (6/7 were P-ANCA positive, 1/7 was C-ANCA positive), while 33%(2/6) WG were ANCApositive (one case of P-ANCA and another case of C-ANCA was positive respectively). Themajor symptoms of respiratory system included hemoptasis 69%(9/13), dyspnea 23%(3/13), drycough 15%(2/13) and chest pain 15%(2/13). The chest x-rays showed multiple patchy shadowsin both lungs were mainly found in MPA (3/7) and single or multiple masses or nodularshadows were mainly found in WG (5/6) with or without cavity formation. The appearance oflungs in MPA 71%(5/7) had been explained as “pulmonary infection” and that of WG hadbeen explained as “primary lung cancer or metastatic carcinoma”. Symptoms ofrespiratory system might occur before (3/5) or after (2/5) occurrence of acute renalfailure. The treatments with corticosteroid and CTX were effective in these cases, inparticularly, pulmonary lesions improved obviously. Conclusions It is very difficult tomake diagnosis of primary small vessel vasculitis with involvement of lungs and thisshould be paid more attentions. ANCA detection is very useful in diagnosis of MPA.Corticosteroid and CTX are most effective in treating these diseases.

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