Raynaud's Disease: Case, Definition & Pathogenesis

by Joseph Alpert, MD

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    00:01 Let's talk about Raynaud's phenomenon, because that's a related thing in which there is spasm in the artery. So let's look at the definition, and we'll start with a case, as we often do. Here's an 18-year-old man, and he complains of severe pain in the tips of his fingers and toes, especially when dealing with cold materials. You'll notice that that's similar to what is seen also in the Buerger's patients.

    00:27 He states that after working in the cold, he develops discolorations of his fingers, progressing from white to blue. And you can see that in the picture on the left and the right. And then when he puts his hands in warm water or warms them up, then color is restored. Usually, the fingertips are bright red for a while and then go back to normal color. Now, the definition of Raynaud's actually falls into three different forms of Raynaud's. Some people have Raynaud's as a disease. In the… You see there in the middle area… in the middle box. This actually… The reason for it is unknown. It occurs in some people where they are just born very sensitive to cold, and they repeatedly get this symptom. And in fact, in Arizona, where I live, I've seen a number of patients who have so-called primary Raynaud's, and they'd moved to Arizona because they couldn't stand living in the northern United States. When the cold weather came on, they were constantly in pain in their hands. On the other hand, you can see it as a phenomenon—a transient phenomenon—that occurs often in the setting of systemic disease like systemic lupus erythematosus or rheumatoid arthritis. Any autoimmune disease can lead to some secondary occasional Raynaud's that's usually not quite so bad, although again, sometimes it can be severe enough that people have to move to a warm climate. And finally, there is the syndrome which occurs in people who know that a certain set of circumstances—for example, emotional stress, will bring on Raynaud's. So the two on either side are secondary to another condition, whereas the primary one can occur and can be inherited and actually is not treatable. Let's talk about the pathogenesis. It's due to a vascular abnormality. The endothelium (remember, that little tiny layer on the inside of the blood vessel?): It produces a number of substances that keep the vessels dilated.

    02:43 And often, in the setting of Raynaud's phenomena or disease, there's a deficiency of these vasodilating substances. One of the most common is called nitric oxide. When that happens (that there's a deficiency), the blood vessel can spasm and close down and cause the white area that you saw from lack of blood flow. And then when the hand warms, there's a redness as the blood rushes back into the area where the vasospasm had occurred.

    03:12 There are often abnormalities in the autonomic nervous system, as we talked about. There's often repeated periods during stress where the noradrenaline or norepinephrine that's released at the nerve endings causes spasm of the blood vessels. And you can even have little clots forming because of the spasm. And also, there's increased activation of the clotting system; the platelets can aggregate. And with the production of increased platelet thromboxane, which is a vasoconstricting compound, you can also make this worse. So there's a whole cascade of things that can happen, starting with a vascular abnormality, going on to a neural abnormality, and then eventually adding in with some floating substances in the bloodstream from the platelets that make things even worse.

    About the Lecture

    The lecture Raynaud's Disease: Case, Definition & Pathogenesis by Joseph Alpert, MD is from the course Diseases of the Lymphatic System.

    Included Quiz Questions

    1. Idiopathic.
    2. Autoimmunity.
    3. Arterial occlusive disease.
    4. Exposure to cold.
    5. Exposure to heat.
    1. Systemic lupus.
    2. Common cold.
    3. Tuberculosis.
    4. Diabetes.
    5. Hypertension.
    1. Emotional stress.
    2. Working out.
    3. Hot climate.
    4. Exposure to sunlight.
    5. Excessive humidity.

    Author of lecture Raynaud's Disease: Case, Definition & Pathogenesis

     Joseph Alpert, MD

    Joseph Alpert, MD

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