背景:IgG4相关疾病的胸腔内表现包括一系列状况和严重程度,并且有时会导致急性呼吸衰竭,如本文所述的病例所报道。
方法:一名69岁的男性前吸烟者,因呼吸困难入院,发烧,咳嗽,疲劳,有3个月的减肥史.他接受了高流量氧疗和无创通气治疗严重呼吸衰竭。胸部计算机断层扫描显示多焦凝聚和毛玻璃混浊,伴有外淋巴间质增厚,纵隔淋巴结肿大和双侧胸腔积液。IgG4的血清浓度升高提示IgG4相关疾病。他出现了肾衰竭并接受了肾活检。组织病理学分析支持诊断,显示密集的淋巴细胞浸润,IgG4+细胞/hpf计数高于60,和storiform纤维化-漩涡,纤维化的“车轮”模式,可能有斑块分布。患者对类固醇治疗反应良好。
结论:尽管IgG4相关性疾病的呼吸道症状通常较轻,胸部特征可以演变成急性呼吸衰竭,很少有额外的胸部表现。
BACKGROUND: The intrathoracic manifestations of IgG4-related disease include a range of conditions and severity, and can on occasion cause acute respiratory failure as reported in the case described here.
METHODS: A 69-year-old male former smoker, was admitted to our hospital with dyspnea, fever, cough, fatigue, and a 3-month history of weight loss. He received high flow oxygen therapy and non-invasive ventilation for severe respiratory failure. Chest computed tomography revealed multifocal condensations and ground glass opacities, accompanied by thickening of the perilymphatic interstitium, mediastinal lymphadenopathy and bilateral pleural effusion. Elevated serum concentrations of IgG4 suggested an IgG4-Related Disease. He developed renal failure and underwent a renal biopsy. Histopathological analysis of which supported the diagnosis by showing dense lymphocytic infiltrate with a count of IgG4+ cells/hpf higher than 60, and storiform fibrosis - a swirling, \"cartwheel\" pattern of fibrosis which may have a patchy distribution. The patient responded well to steroid therapy.
CONCLUSIONS: Although respiratory symptoms are usually mild in IgG4-relatd disease, thoracic features can evolve into acute respiratory failure with few extra thoracic manifestations.