Matsui Shoko
a Health Administration Center , University of Toyama , Toyama , Japan.
Mod Rheumatol. 2019 Mar;29(2):251-256. doi: 10.1080/14397595.2018.1548089. Epub 2019 Jan 3.
IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings. Developing diagnostic criteria for IgG4-RRD, including clinicoradiological and pathological characteristics, is necessary for its appropriate diagnosis.
IgG4相关性疾病（IgG4-RDs），如自身免疫性胰腺炎和IgG4相关性米库利奇病，常伴有胸内病变，称为IgG4相关性呼吸道疾病（IgG4-RRD）。IgG4-RRD主观症状较少，通常在对IgG4-RD胸外病变患者进行检查时被发现。IgG4-RRD具有多种表现，包括通过淋巴管途径在胸部多个部位出现肿块、结节、增厚和浸润。虽然血清IgG4浓度升高以及伴有大量IgG4阳性浆细胞的淋巴浆细胞浸润的病理证据是IgG4-RD的特征性表现，但其他胸内疾病，如多中心Castleman病和恶性肿瘤，也可能有类似表现。制定包括临床影像学和病理特征在内的IgG4-RRD诊断标准对于其准确诊断是必要的。
