长期误诊为肺感染的血管炎合并顽固心衰一例报告

A case report of the vasculitis misdiagnosed as pneumonia with stubborn heart failure

韦格纳肉芽肿(WG)是一种以毛细血管、微小动静脉受累为主的全身坏死性肉芽肿性血管炎,典型的WG三联征为上下呼吸道、肾脏病变,只有呼吸道改变而无肾脏改变为局限性WG。局限性WG因症状及影像学表现不特异,尤易误诊。笔者发现1例仅表现肺部症状及顽固心衰的局限性WG,胸部CT表现为两肺多发不规则片状模糊密度增高影,被长期误诊为肺感染。经查两次蛋白酶-3(PR-3)-抗中性粒细胞胞浆抗体(ANCA)阳性,最终诊断为ANCA相关性血管炎、WG。本文回顾了该患者的诊治经过并复习相关文献资料。

Wegener’s granulomatosis(WG)is a kind of systemic necrotizing granulomatous vasculitis characterized by capillary and small arteriovenous involvement.The typical WG triad is lesions of the upper and lower respiratory tract and kidneys.Only changes in the respiratory tract without renal lesions are localized WG.Localized WG is very easy to be misdiagnosed because of its nonspecific symptoms and radiological manifestations.The author found one case of localized WG,which showed pulmonary symptoms and refractory heart failure.The chest CT showed that multiple irregular nidus,blurred and density enhanced lesions in both lungs.The patient was misdiagnosed as pulmonary infection for a long time.The patient was eventually diagnosed as asantineutrophil cytoplasmic antibody(ANCA)associated vasculitis and WG after two times of examination of protease-3(PR3)ANCA were positive.This article reviewed the diagnosis and treatment of the patient,and reviewed the relevant literatures.