Raynaud’s disease

موضوع: Raynaud’s disease الأربعاء مارس 20, 2019 4:25 pm

Raynaud’s disease
Raynaud’s disease (also Raynaud, or Raynaud’s phenomenon) was first described in 1862 by Auguste Raynaud. Even at a first look, the disorder is apparent, owing to the fact that the patient notices the change in the color of their skin, particularly in their digits.
At low temperatures, the skin becomes cold and pale due to abnormal vasoconstriction of the digital arterioles leading to diminished blood supply. This may result in the coldness and cyanosis of the digits as the distension of veins occurs, followed by the bluish skin coloration . With the rise of temperature, the skin turns warm and red as a result of the arterioles’ response to vasodilatation.
It took quite a few years of study for complete comprehension of the pathology and physiology of Raynaud, which continues to be explained today. In 2005, Hendrik classified the causes and mechanisms of Raynaud’s phenomenon into three groups, namely:

Vascular
Intravascular
Neural abnormalities

Definition of Raynaud’s Disease

Raynaud’s disease is an exaggerated vascular response to cold temperature or emotional stress. The phenomenon is manifested clinically by sharply demarcated color changes of the skin of the digits. Reduction in the size of the blood vessels, most commonly in the fingers and toes, results in change of color from white to blue. As blood flow returns to the digits, the skin turns red.
Its affection is symmetrical, usually attacks in episodes, a clear demarcated line exists between ischemic part and unaffected part.

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Image: “Raynaud’s Phenomenon in my right ring finger, viewed from the front of my hand. This occurs in cold weather on my fingers and my toes.” by Thomas Galvin. License: CC BY-SA 4.0

Raynaud’s phenomenon is an ailment causing a specific sequence of color changes in the extremities in response to stress or weather fluctuations, either hot or cold. A great number of people with Raynaud’s disease have extreme sensitivity to cold temperature.

Classification of Raynaud’s Disease

Raynaud’s disease is classified into:

Primary Raynaud phenomenon, also known as idiopathic Raynaud disease
Secondary Raynaud phenomenon

Primary Raynaud phenomenon

Primary Raynaud phenomenon (RP) or idiopathic Raynaud disease are terms to describe those patients without a definable cause for their vascular events. In this setting, RP is considered to be an exaggeration of normal vasoconstriction to cold exposure.

Secondary Raynaud phenomenon

Secondary RP refers to those patients with RP in whom an associated disease or cause may underlie the attacks. Possible associations include atherosclerosis, scleroderma, lupus and rheumatoid arthritis. It can also be a result of taking assured medications, having frostbite, smoking or using vibrating power tools for numerous years. Therefore, it is sometimes referred to as secondary Raynaud’s.
Secondary Raynaud’s is more frequent than idiopathic Raynaud disease. Also, it is more likely to occur in both genders with an almost equal distribution.
The disorder progresses proportionally, affecting digits from both hands or feet in symmetrical manner. Over a period of time, recurrent and sustained spasms become more common.

“Raynaud´s disease” Image created by Lecturio

Symptoms of Raynaud’s Disease

Raynaud’s disease symptoms include:

Cold fingers or toes.
Color changes in your skin from white, blue to red, in response to cold or stress.
Events are episodic. Swelling, numb feeling or stinging pain upon warming or stress relief.

The disorder progresses proportionally, affecting digits from both hands or feet in symmetrical manner. Over a period of time, recurrent and sustained spasms become more common.

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Image: “A good illustration of what Raynaud’s looks like” by Jmesy. License: CC BY-SA 4.0

It can occur both in our lower and upper limbs as well as the chin, nose, lips and ears. In occasional situations, low oxygen supply can result in the occurrence of necrosis.
The impacts are reversible, and they should be distinguished from the non-reversible origins of low blood supply, including thrombosis or vasculitis.

Pathophysiology of Raynaud’s Disease

Vascular abnormalities

A deficit of vasodilators, mainly nitric oxide, has been suggested to be one of the cause of Raynaud’s phenomenon. Moreover, effective vasoconstrictors generally present in our endothelium (known as endothelin-1) have been observed to be flowing in elevated levels in people suffering from secondary Raynaud’s disease.
Discharge of the vasoconstrictor endothelin-1 is elicited by stimuli of vasoactivation, including transforming growth factor beta (TGF-beta), vasopressin and angiotensin.
Angiotensin has both pro-fibrotic and vasoconstrictive impacts.

In people suffering from systemic sclerosis, fundamental anomalies allied with vasculature fibrotic proliferation result in decreased blood flow toward the digits. This is a usual characteristic difference from the primary Raynaud’s disease.
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