与IgG4相关的脏疾病:临床病理学特征,差异诊断和模仿
Sarwat I Gilani1, Alessia Buglioni2, Lynn D Cornell2
1Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at San Antonio, TX, USA.
Seminars in diagnostic pathology
|January 21, 2024
概括
与IgG4相关的脏疾病 (IgG4-RKD) 涉及与IgG4相关的疾病 (IgG4-RD) 相关的各种脏疾病. 这篇评论详细介绍了其多样化的表现,诊断标记和模仿,如ANCA相关疾病.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 与IgG4相关的脏疾病 (IgG4-RKD) 是IgG4相关疾病 (IgG4-RD) 中脏表现的不断变化的谱.
- 最初被确定为IgG4相关的管介质性炎 (IgG4-TIN) 和IgG4相关的膜状质炎 (IgG4-MGN),现在包括多种介质,质和血管病理.
- 虽然IgG4-RKD通常是全身性,但在约20%的病例中,IgG4-RKD可以表现为限性疾病.
研究的目的:
- 为提供IgG4相关病 (IgG4-RKD) 的全面概述.
- 讨论IgG4-RKD的各种临床表现,诊断特征和差异诊断.
- 为了突出IgG4相关的管介质炎的模仿,特别是ANCA相关的疾病.
主要方法:
- 关于IgG4相关病 (IgG4-RKD) 和相关疾病的文献综述.
- 对IgG4-RKD的临床表现,病理模式和血清学发现的分析.
- 将IgG4-RKD与其他间歇性炎和球细胞疾病进行比较.
主要成果:
- IgG4-RKD可变地表现为急性/慢性损伤,蛋白尿,性综合征,质或阻塞.
- 与一般IgG4-RKD相比,IgG4-RKD患者经常表现出血清IgG4/IgG的升高和低补充血.
- 差异诊断包括其他自身免疫和富含血细胞的间歇性神经,炎症性质量病变以及与ANCA相关的疾病.
结论:
- IgG4-RKD是一种复杂的病,具有多种表现和诊断考虑.
- 识别IgG4-RKD的广泛范围对于准确的诊断和管理至关重要.
- 区分IgG4-RKD与模仿者,如ANCA相关疾病,对于适当的治疗策略至关重要.
相关概念视频
Nephrons
2.5K
The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
2.5K
Renal Corpuscle
2.2K
The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
2.2K
Internal Anatomy of the Kidney
1.7K
The kidneys are essential organs in the human body, performing a myriad of tasks that maintain homeostasis and overall health.
Anatomical Position and Dimensions
The kidneys are retroperitoneal organs positioned against the posterior abdominal wall on either side of the spine, roughly between the twelfth thoracic and third lumbar vertebrae. Each kidney is typically 10-12 cm long, 5-6 cm wide, and 3-4 cm thick, weighing about 150 grams.
Renal Cortex
The outermost region of the kidney is the...
Anatomical Position and Dimensions
The kidneys are retroperitoneal organs positioned against the posterior abdominal wall on either side of the spine, roughly between the twelfth thoracic and third lumbar vertebrae. Each kidney is typically 10-12 cm long, 5-6 cm wide, and 3-4 cm thick, weighing about 150 grams.
Renal Cortex
The outermost region of the kidney is the...
1.7K
Renal Tubule and Collecting Duct
995
The renal tubule is divided into three parts: the proximal convoluted tubule (PCT), the Loop of Henle (LOH), and the distal convoluted tubule (DCT).
Proximal Convoluted Tubule (PCT):
The PCT is the initial segment of the renal tubule, extending from the Bowman's capsule that encloses the glomerulus. Its convoluted structure and microvilli-lined cells increase the surface area for reabsorption. The PCT reabsorbs glucose, amino acids, sodium, and water from the filtrate, ensuring essential...
Proximal Convoluted Tubule (PCT):
The PCT is the initial segment of the renal tubule, extending from the Bowman's capsule that encloses the glomerulus. Its convoluted structure and microvilli-lined cells increase the surface area for reabsorption. The PCT reabsorbs glucose, amino acids, sodium, and water from the filtrate, ensuring essential...
995
External Anatomy of the Kidney
1.1K
The kidneys are a pair of bean-shaped organs in the human body that play a critical role in maintaining overall health. They filter out waste products from the blood, regulate blood pressure, maintain electrolyte balance, and stimulate the production of red blood cells.
The kidneys are located in the retroperitoneal space on either side of the vertebral column, protected posteriorly by the 11th and 12th ribs. The right kidney sits slightly lower than the left owing to the presence of the liver...
The kidneys are located in the retroperitoneal space on either side of the vertebral column, protected posteriorly by the 11th and 12th ribs. The right kidney sits slightly lower than the left owing to the presence of the liver...
1.1K
Factors Affecting Renal Clearance: Renal Impairment
95
Renal dysfunction significantly impairs the renal clearance of drugs, leading to potential complications in drug therapy. Renal failure, which can be caused by various factors, poses a significant challenge in the elimination of drugs from the body.
One condition associated with renal failure is uremia. Uremia is characterized by impaired glomerular filtration and fluid accumulation in the body. This condition hinders the renal clearance of drugs, resulting in drug accumulation and potential...
One condition associated with renal failure is uremia. Uremia is characterized by impaired glomerular filtration and fluid accumulation in the body. This condition hinders the renal clearance of drugs, resulting in drug accumulation and potential...
95


