坏死性肉芽肿性血管炎误诊为肺癌原因讨论

Cause Analysis and Literature Review:Necrotic Granulomatous Angiitis Misdiagnosed as Lung Cancer

目的分析坏死性肉芽肿性血管炎的临床特点及诊断方法,以减少误诊。方法对我院收治的坏死性肉芽肿性血管炎1例临床资料进行回顾性分析并复习相关文献。结果患者因反复发热伴血痰1周入院,外院经胸部CT检查诊断为肺癌,转我院。我院行胸部CT增强扫描示两肺多发肿块,部分病灶内见空泡征;纤维支气管镜检查未见新生物;肺穿刺活检示急慢性炎症伴大片坏死及出血。查血核周型抗中性粒细胞胞浆抗体(p-ANCA)和人蛋白酶3特异性抗中性粒细胞胞质抗体(PR3-ANCA)均阳性。确诊为坏死性肉芽肿性血管炎,予大剂量糖皮质激素联合环磷酰胺冲击治疗,症状明显好转,病灶大部分吸收。随访6个月,糖皮质激素序贯治疗中,病情平稳。结论坏死性肉芽肿性血管炎较少见,临床应提高警惕,病理检查及ANCA检测对诊断有重要意义。

Objective To analyze clinical features and diagnostic methods of necrotic granulomatous angiitis so as to reduce misdiagnosis rate. Methods Clinical data of a patient with necrotic granulomatous angiitis in our hospital were retrospectively analyzed, and related literature were also reviewed. Results The patient was admitted for recurrent fever and blood sputum for a week, and was misdiagnosed as having lung cancer by thorax CT examination in another hospital. In our department, enhancement CT showed multiple masses in both lungs with vacuolar signs in some masses; but no neoplasm was revealed by fiberoptic bronchoscopy. Pulmonary puncture biopsy showed active chronic inflammation with massive necrosis and hemorrhage. Results of perinuclear anti-neutrophil cytoplasmic antibodies （p-ANCA） and proteinase 3-specific anti-neutrophil cytoplasmic antibodies （PR3-ANCA） tests were positive. Necrotic granulomatous angiitis was confirmed, and high-dose glneocorticoids combined with cyelophosphamide （CTX） impingement treatment was given. The patient＇s symptom significantly improved, and most lesions were absorbed. The patient＇s pathogenetic condition was stable during glucocorticoids sequential therapy as shown by 6 months of follow-up. Conclusion Necrotic granulomatous angiitis is rare in clinical practice, so clinicians should pay more attention. The pathological and ANCA tests are very important in final diagnosis.