与IgG4相关的疾病:从诊断到缓解
Arjun Mahajan1, Alex Tinianow1, Guy Katz1
1Harvard Medical School, Boston, MA, USA; Department of Medicine, Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, MA, USA.
Best practice & research. Clinical rheumatology
|November 15, 2025
概括
免疫球蛋白G4相关疾病 (IgG4-RD) 是一种复杂的纤维炎症疾病. 进展包括B细胞枯竭疗法,但需要进行研究以获得更好的诊断和向治疗.
科学领域:
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
- 病理学 病理学 病理学
背景情况:
- 免疫球蛋白G4相关疾病 (IgG4-RD) 是一种系统性纤维炎症,影响多个器官.
- 了解IgG4-RD需要识别不同的临床表现,从腺体问题到血管并发症,以及炎症和纤维化的方面.
研究的目的:
- 提供关于IgG4-RD的当前知识的全面概述.
- 解决疾病机制,诊断方法和治疗策略.
- 突出现有的知识差距和未来的研究方向.
主要方法:
- 关于IgG4-RD的当前文献的综述.
- 分析诊断标准,整合临床,成像,病原体和血清学数据.
- 评估不断发展的治疗策略,包括B细胞枯竭疗法.
主要成果:
- IgG4-RD呈现出各种表现,需要综合诊断方法.
- B细胞枯竭疗法 (例如,伊内比利祖马布) 改善了治疗选择.
- 节约葡萄糖皮质激素的策略是首选的,但长期管理仍然具有挑战性.
结论:
- 有效管理IgG4-RD需要平衡疾病控制与最小化免疫抑制风险.
- 进一步的研究对于识别生物标志物,建立缓解标准和阐明病原发生的原因至关重要.
- 需要有针对性的疗法来解决IgG4-RD治疗中持续存在的挑战.
相关概念视频
Drugs for Treatment of Crohn's Disease in IBD Using Immunomodulatory Agents
473
Crohn's disease is an inflammatory bowel disorder marked by chronic inflammation of the GI tract. Various treatment strategies for Crohn's disease are employed, such as immunomodulatory agents, glucocorticoids, and biologics or anti-TNF therapy. Azathioprine (Imuran), a commonly used immunomodulatory drug for Crohn's disease, is converted in the body to mercaptopurine, which inhibits purine biosynthesis and cell proliferation. Both are utilized in severe cases of Inflammatory Bowel...
473
Autoimmune Disorders
1.4K
Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune...
Concept and Mechanism of Autoimmune Diseases
The immune...
1.4K
Classification of Illness
8.5K
The meaning of illness is individualized to each person who experiences an alteration in health. In contrast, disease is a medical term indicating a pathological change in the structure and function of the body or mind. It is a condition that has specific symptoms and boundaries.
An illness is a response to a disease in which the person's level of functioning is changed compared with a previous level. The general classification of illness includes acute and chronic.
Acute illness is severe...
An illness is a response to a disease in which the person's level of functioning is changed compared with a previous level. The general classification of illness includes acute and chronic.
Acute illness is severe...
8.5K
Inflammatory Bowel Disease IV: Pharmacological Management
459
Upon diagnosis, managing Inflammatory Bowel Disease (IBD) involves addressing several crucial aspects. The primary goals include resting the bowel, correcting malnutrition, and providing symptomatic relief. Resting the bowel may consist of medications to reduce inflammation and promote healing. Correcting malnutrition is essential, often requiring dietary adjustments and nutritional supplements. Symptomatic relief aims to ease pain, diarrhea, and other discomforts in IBD.
Pharmacologic...
Pharmacologic...
459
Inflammatory Bowel Disease II: Crohn's Disease
939
Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
939
Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF
470
Tumor Necrosis Factor (TNF), a proinflammatory cytokine, contributes significantly to the inflammation seen in Crohn's disease. It exists as soluble TNF and membrane-bound TNF, with actions mediated through TNF receptors (TNFR). TNFR activation leads to the release of proinflammatory cytokines, T-cell activation, collagen production, and leukocyte migration, all contributing to inflammation in Crohn's disease. Anti-TNF monoclonal antibodies, namely infliximab (Remicade), adalimumab...
470

