医学
自身免疫性胰腺炎
IgG4相关疾病
病理
疾病
鉴别诊断
泪腺
甲状腺
自身免疫性疾病
全身性疾病
淋巴
发病机制
内科学
作者
Anxo Martínez-de-Alegría,Sandra Baleato-González,Roberto García‐Figueiras,Anaberta Bermúdez-Naveira,Ihab Abdulkader-Nallib,José Antonio Díaz-Peromingo,Carmen Villalba‐Martín
出处
期刊:Radiographics
[Radiological Society of North America]
日期:2015-11-01
卷期号:35 (7): 2007-2025
被引量:114
摘要
Immunoglobulin G4 (IgG4)-related disease is a relatively recently proposed clinical-pathologic entity that is characterized by fibro-inflammatory lesions rich in IgG4-positive plasma cells and, often but not always, elevated serum IgG4 concentrations. IgG4-related disease was recognized as a systemic disease in 2003, when extrapancreatic manifestations were identified in patients with autoimmune pancreatitis. Since then, the disease has been reported as affecting virtually every organ system and has been identified in the biliary tree, salivary and lacrimal glands, periorbital tissues, lungs, lymph nodes, thyroid gland, kidneys, prostate gland, testicles, breasts, and pituitary gland. Its pathogenesis is poorly understood, but findings are consistent with both an autoimmune and an allergic disorder. Although definitive diagnosis requires histopathologic analysis, imaging plays an important role in demonstrating infiltration and enlargement of involved organs. Because of the systemic nature of the disease, imaging workup of IgG4-related disease should always include whole-body examinations to detect multiorgan involvement. Patients often present with subacute development of a mass in or diffuse enlargement of the affected organ, sometimes mimicking a neoplastic process. In every anatomic location, several inflammatory and neoplastic entities must be considered in the differential diagnosis. Because IgG4-related disease usually shows a marked response to corticosteroid therapy, radiologists should be familiar with its clinical and imaging manifestations to avoid a delay in diagnosis and unnecessary surgical interventions.
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