临床病理学珍珠和IgG4相关疾病的诊断陷:具有挑战性的病例系列和文献审查
Sokol Sina1, Giulio Luigi Bonisoli2, Sofia Vitale2
1Section of Pathology, Department of Diagnostics and Public Health, University of Verona, Piazzale L.A. Scuro 10, 37134 Verona, Italy.
Diagnostics (Basel, Switzerland)
|September 27, 2025
概括
与免疫球蛋白G4相关的疾病 (IgG4-RD) 由于模仿其他疾病的非典型症状而带来挑战. 早期诊断和多学科护理对于有效的管理和避免误诊至关重要.
科学领域:
- 风湿病学和免疫学
- 胃肠病学 胃肠病学
- 在瘤学瘤学.
背景情况:
- 免疫球蛋白G4相关疾病 (IgG4-RD) 是一种纤维炎症疾病,具有特征性的IgG4阳性血细胞透.
- 非典型的IgG4-RD表现可以模仿恶性病或其他炎症状况,导致诊断延迟和不适当的治疗.
研究的目的:
- 突出IgG4相关疾病 (IgG4-RD) 的非典型表现所带来的诊断挑战.
- 强调早期识别,多学科合作和IgG4-RD管理的综合诊断方法的重要性.
主要方法:
- 介绍和管理三个不同的IgG4相关疾病 (IgG4-RD) 病例与异常表现.
- 审查现有文献,以确定IgG4-RD的诊断挑战和治疗策略.
- 分析临床特征,组织病理学,血清学 (血清IgG4水平和IgG4/IgG比率),以及治疗反应.
主要成果:
- 案例1:一个30岁的男性患有胃肠道,神经和血清干扰的IgG4-RD,在类固醇失败后,与rituximab持续改善.
- 案例2:一名35岁的女性患有胆道IgG4-RD模仿胆管癌,对rituximab治疗反应良好.
- 案例3:一个64岁的女性下下IgG4-RD在没有全身治疗的情况下,在切除术后仍然无症状.
- 文献审查证实了诊断困难,特别是对于胃肠道和胆道IgG4-RD,并强调了组织病理学不可或缺的作用.
结论:
- 在患有不明原因,复发或对类固醇有反应的患者中,即使有异常表现,也应该怀疑IgG4-RD.
- 与临床发现相结合的组织病理学确认对于准确的诊断和排除恶性瘤至关重要.
- 虽然类固醇通常是第一线治疗,但对于IgG4-RD的复发或耐火病例,可能需要Rituximab等B细胞导向疗法.
相关概念视频
01:30Inflammatory Bowel Disease II: Crohn's Disease
960
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...
960
01:15Transcytosis of IgG
4.0K
Transcytosis is the process in which molecules are internalized by endocytosis, transported across the cell, and released through exocytosis from the opposite end of the cell. Molecules such as insulin, immunoglobulins, and certain nutrients are transferred through the recycling endosomes by recycling and transcytosis.
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...
4.0K
01:17Chronic Bowel Disorders: Introduction
747
Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
747
01:27Inflammatory Bowel Disease I: Ulcerative Colitis
903
Introduction
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
903
01:19Pericarditis II: Clinical Features and Diagnostic Tests
287
Pericarditis is distinguished by inflammation of the pericardium, the fibrous sac that encases the heart. It can be acute, lasting less than six weeks, or chronic, persisting for over three months. Understanding its clinical manifestations and diagnostic findings is crucial for timely and effective management.Clinical ManifestationsWhile pericarditis can be asymptomatic, it usually presents with characteristic symptoms such as:Chest Pain: The most characteristic symptom of pericarditis is chest...
287
01:30Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation
732
Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation
Irritable Bowel Syndrome (IBS) is classified into subtypes based on the predominant bowel habits as determined by the Bristol Stool Form Scale (BSFS). The subtypes are:
Irritable Bowel Syndrome (IBS) is classified into subtypes based on the predominant bowel habits as determined by the Bristol Stool Form Scale (BSFS). The subtypes are:
732