在自身免疫神经肌肉疾病中的静脉免疫球蛋白
1Neuromuscular Diseases Section, National Institutes of Health, National Institute of Neurological Disorders and Stroke, Bethesda, Md 20892, USA. dalakasm@ninds.nih.gov
JAMA
|May 20, 2004
概括
静脉注射免疫球蛋白 (IVIG) 对许多自身免疫神经肌肉疾病是有效的,特别是作为吉林巴雷综合征和CIDP的第一线治疗. 需要进一步的研究来确定最佳剂量和长期疗效.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
背景情况:
- 静脉注射免疫球蛋白 (IVIG) 调节免疫反应,包括Fc受体功能,补体激活,细胞因子产生和T/B细胞活性.
- 这些免疫调节机制被认为是IVIG在自身免疫神经肌肉疾病中的治疗效果的基础.
研究的目的:
- 系统地审查IVIG在治疗自身免疫神经肌肉疾病中的疗效.
- 重点是分析来自受控临床试验的数据.
主要方法:
- 使用MEDLINE和EMBASE数据库进行了全面的文献搜索.
- 包括英语同行评审的出版物,重点关注与临床实践相关的受控研究.
主要成果:
- IVIG (2g/kg) 是作为第一线治疗吉兰-巴雷综合征,慢性炎症性脱髓性多神经病变和多焦点运动神经病变的有效药物.
- 它显示了作为第二线治疗硬人综合征,皮肤肌炎,肌痛性肌痛症和兰伯特-伊顿肌痛性肌痛综合征的有效性.
- 在包括体肌肉炎,某些脱髓化多神经病变和已确定的多发性硬化症中,IVIG的益处有限或没有显著的益处.
结论:
- IVIG是许多自身免疫神经系统疾病的宝贵治疗方法.
- 需要进一步进行受控试验,以确定其完整的疗效范围,维持治疗的最佳剂量以及对生活质量的影响.
相关概念视频
Chemical Synapses
Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
Myasthenia Gravis: Overview and Treatment
Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Myasthenia Gravis: Diagnostic Tests
Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin
Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...
The binding of dantrolene to the RYR1...
Autoimmune Disorders
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 system...
Concept and Mechanism of Autoimmune Diseases
The immune system...
Myasthenia Gravis ll: Pathophysiology
The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...


