PDF(Pubmed)
Abstract：
IgG4 related disease (IgG4-RD) is a multisystem inflammatory disease and can affect several organs including salivary glands, orbits, lungs, pancreas, kidneys and lymph nodes. Up to 40 % of patients have allergic manifestations including asthma, chronic rhinosinusitis, eczema and asthma. Commonly pulmonary manifestations include pulmonary nodules ranging from <1 to 5 cm in diameter, interstitial opacities and mediastinal lymphadenopathy. Rarely, IgG4-RD presents as isolated tracheal disease. Symptoms include dyspnea and stridor due to airway narrowing. Diagnosis of IgG4-RD including tracheal IgG4-RD requires a biopsy. The histologic specimen is characterized by lymphoplasmacytic infiltrate with high density of IgG4 positive plasma cells, and storiform fibrosis (a cartwheel appearance of fibroblasts and inflammatory cells). Up to 30 % of patients with IgG4-RD have normal serum IgG4 levels. The mainstay of therapy is glucocorticoids for those with systemic disease. Rituximab is an alternative for those who cannot tolerate glucocorticoids or those with disease recurrence. Patients with tracheal disease often require balloon dilation. Recurrence is common in patients and up to two thirds of patients have residual disease despite treatment. These patients often require surgical resection of affected area for symptomatic relief.
摘要：
IgG4相关疾病（IgG4-RD）是一种多系统炎症性疾病，可影响包括唾液腺在内的多个器官。轨道,肺,胰腺,肾脏和淋巴结。高达40%的患者有过敏表现，包括哮喘，慢性鼻-鼻窦炎,湿疹和哮喘.常见的肺部表现包括直径<1至5cm的肺结节，间质混浊和纵隔淋巴结肿大。很少,IgG4-RD表现为孤立的气管疾病。症状包括由于气道狭窄引起的呼吸困难和喘鸣。IgG4-RD包括气管IgG4-RD的诊断需要活检。组织学标本的特点是淋巴浆细胞浸润，高密度的IgG4阳性浆细胞，和storiform纤维化（成纤维细胞和炎症细胞的车轮外观）。高达30%的IgG4-RD患者具有正常的血清IgG4水平。对于患有全身性疾病的患者，主要的治疗方法是糖皮质激素。利妥昔单抗是那些不能耐受糖皮质激素或患有疾病复发的患者的替代药物。气管疾病患者通常需要球囊扩张。复发在患者中很常见，尽管接受了治疗，但仍有多达三分之二的患者患有残留疾病。这些患者通常需要手术切除受影响的区域以缓解症状。
