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一例复发性韦格纳肉芽肿,表现为支气管内肿物。

A relapsing case of Wegener's granulomatosis presenting as an endobronchial mass.

作者信息

Yilmaz Adnan, Damadoğlu Ebru, Aksoy Ferda, Düzgün Sevim, Yağci Tuncer Leyla, Yalçinsoy Murat

机构信息

Department of Pulmonology, Süreyyapaşa Center for Chest Disease and Thoracic Surgery, Istanbul, Turkey.

出版信息

Tuberk Toraks. 2006;54(1):56-60.

Abstract

Wegener's granulomatosis (WG) relapse is frequent. Although lung involvement occurs in 85% of patients, endobronchial presentation of the disease is uncommon. We reported a relapsing case of WG presenting as an endobronchial mass. A 56- year-old man present ed with recurrence of WG following 14 months of cyclophosphamide and prednisolone therapy and 36 months of complete remission. At his first presentation, he was diagnosed as having WG with involvement of kidney, lung, upper airways, skin, joints and eyes. His chest X-ray showed bilateral patchy consolidation. Cytoplasmic-anti-neutrophil cytoplasmic antibodies (c-ANCA) was also present in high titres. c-ANCA was negative after therapy. At the time of relapse, he presented with nasal symptoms and hemoptysis. His chest X-ray showed right paracardiac opacity. Fiberoptic bronchoscopy revealed a mass lesion subtotally obstructing the proximity of right lower lobe. He has been given prednisolone in tapering doses and cyclophosphamide for 10 months.

摘要

韦格纳肉芽肿(WG)复发很常见。虽然85%的患者会出现肺部受累,但该疾病的支气管内表现并不常见。我们报告了一例以支气管内肿块形式出现的WG复发病例。一名56岁男性,在接受环磷酰胺和泼尼松龙治疗14个月且完全缓解36个月后出现WG复发。初次就诊时,他被诊断为患有累及肾脏、肺部、上呼吸道、皮肤、关节和眼睛的WG。他的胸部X线显示双侧斑片状实变。同时还检测到高滴度的胞浆抗中性粒细胞胞浆抗体(c-ANCA)。治疗后c-ANCA转阴。复发时,他出现鼻部症状和咯血。他的胸部X线显示右心旁模糊影。纤维支气管镜检查发现一个肿块病变,几乎完全阻塞右下叶附近。他接受了逐渐减量的泼尼松龙和环磷酰胺治疗10个月。

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