结节性硬皮症:一个独特的临床表型的特征
Anupam Somashekar1, Stephen Squires2, Anthony V Benedetto3
1Division of Rheumatology, Department of Medicine, Thomas Jefferson University, 211 S 9th St Suite 210, Philadelphia, PA 19107, United States.
The American journal of the medical sciences
|September 26, 2024
概括
结节性硬化症是一种罕见的全身性硬化症变体,呈现出特征性的皮肤结节. 这些结节通常不耐治疗,尽管相关器官参与.
科学领域:
- 类风湿病学 类风湿病学
- 皮肤病学 皮肤病学
- 系统性硬化症研究研究
背景情况:
- 结节性硬化症是一种罕见的系统性硬化症 (SSc) 变体.
- 它的特点是肢体和干部的硬化皮肤结节.
- 最近与 keloid 形式区分开来,它经常与其他 SSc 表现共同发生.
研究的目的:
- 综述和描述结节性硬皮症的临床特征.
- 了解这种SSc表型的表现和关联.
- 提供第一个关于结节性硬化症的综合性综述.
主要方法:
- 对所有报告的结节性硬化症病例的系统文献综述.
- 临床特征,血清学标志物和器官参与的分析.
- 治疗反应的表征.治疗反应的表征.
主要成果:
- 结节性硬化症通常与扩散性SSc和早期渐进性皮肤参与有关.
- 患者表现出斑点/核状反核抗体和SSc多器官参与.
- 肺高血压很少发生,结节是不耐治疗的.
结论:
- 结节性硬化症是一种独特的SSc表型,具有特定的临床和血清学特征.
- 典型的结节对当前的免疫抑制和抗纤维菌疗法表现出耐药性.
- 可能需要进一步的研究,以确定结节性硬性皮质炎的有效治疗方法.
相关概念视频
Atherosclerosis I: Introduction
2
Atherosclerosis is a progressive disorder characterized by the buildup of plaques on the arterial inner wall, causing them to narrow and harden over time. These plaques comprise lipids, calcium, blood components, carbohydrates, and fibrous tissue. The process primarily affects the intima of large and medium-sized arteries, reducing blood flow in any artery.Etiology and risk factorsThe cause of atherosclerosis is multifactorial, involving a complex interplay among endothelial injury, lipid...
2
Skin Cancer
4.0K
Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
4.0K
Atherosclerosis II: Clinical manifestations and prevention
2
Atherosclerosis is a progressive disorder that leads to the thickening and narrowing of arterial walls due to plaque buildup. This condition can cause various symptoms depending on the arteries affected:Coronary Artery Disease (CAD): This condition affects the coronary arteries and may lead to chest pain (angina), shortness of breath (dyspnea), heart attacks, and other heart disease symptoms.Cerebrovascular Disease: This affects blood flow to the brain, causing transient ischemic attacks (TIAs)...
2
Cardiomyopathy IV: Restrictive Cardiomyopathy
2
Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
2
COPD: Pathogenesis and Clinical Features
243
Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
243
Cardiomyopathy III: Hypertrophic Cardiomyopathy
1
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
1


