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Monday, February 15, 2016

Raynaud's Disease

By: TheGreenLabCoat

Working in Family Medicine, you see several chronic diseases on a daily basis: diabetes mellitus 2, hypertension, and osteoporosis - Or as one of our elderly patients jokingly refers to them, “all these old people diseases.” Recently during a new patient visit, I noticed that this patient’s fingers were a different color from the rest of her hand; fleshy, pink hands but with almost paper white fingers. At first I thought it might have been a skin discoloration that naturally occurs in some people, but through the course of the visit her hand gradually turned blue, and then a bright shade of red. I couldn’t help but to ask the patient, “Are you aware that your fingers have been changing colors since you sat down in here?” The patient waved her unusually colorful hand in the air, stating “Oh honey, I have Raynaud’s, it's fine. I was just holding my soda.” Not knowing exactly what she meant at the time, I decided to educate myself on the subject: What is Raynaud’s?


Raynaud’s Disease, named after Maurice Raynaud in 1862, is a disorder of the blood vessels that causes them to overreact, predominantly in the hands and feet. Our body has an internal thermostat with many locations of thermometers. When we are cold, the sensors in our body tell us “Hey, we need to keep the heat up so that all of our organs continue to function.” It sends out a signal that binds to receptors on our blood vessels, instructing them to secrete vasoconstriction hormones. When this happens, our blood vessels constrict, kind of like shutting all the windows around the house to keep the heat in. In this situation there is less blood going to our extremities, keeping the core of our bodies warmer. People who have Raynaud’s disease are unable to regulate the appropriate blood flow when exposed to cold temperatures. Their blood vessels spasm, which can cause pain, numbness, and tingling. Color changes in fingers are the most observable symptoms when an attack is happening. Emotional distress may also be a trigger of this phenomenon.

Though the exact cause of Raynaud’s is still unknown, scientists have been able to separate this disease into two major classifications: Primary Raynaud’s Phenomenon (PRP) and Secondary Raynaud’s (SR). PRP is the most common type in most people. Development can occur at any age but happens most frequently between the ages of 15 - 30, and it typically affects women more often than men. If a family member has PRP, there is a 33% chance that their offspring will develop the disease as well.

Secondary Raynaud’s (SR) has been associated with rheumatic disorders such as Lupus and scleroderma. Depending on severity of SR, it could be life threatening due to the hardening of blood vessels which down the road could be a contributing factor of heart issues such as ischemia or plaque buildup in the arteries.

Neural abnormalities, hormonal and genetic factors, and smoking all have been noted as potential causes of this disease. A few blood tests and observing capillaries of the fingernails under a microscope are the common methods of diagnosing Raynaud’s Disease. There currently are no medications to cure Raynaud’s. Outcomes for patients with PRP are usually very good. No reported mortality and little morbidity. In very rare cases, ischemia of the affected body part can result in necrosis.

Following the guidelines below can help prevent an attack:
        Keep warm - Manually regulating body temperature can help to prevent an attack. For instance, avoid moving from a hot summer day to a cold air conditioned room. Wearing mittens and socks to bed during winter, or changing out of wet clothing items a rainy day are both good preventative measures.
        Do not smoke - Nicotine in cigarettes causes the body temperature to lower, which may trigger an attack.
        Control stress - Emotional well being can prevent an attack. Relaxation methods such as meditation has been reported useful.
        Avoid stimulating medications, foods - Vasoconstrictor medications such as narcotics, beta blockers (blood pressure medication) OTC cold medication, and caffeine.

As for SR, there is no FDA-approved medication specifically, but this type of Raynaud’s is usually treated with calcium channel blockers such as amlodipine (norvasc) and felodipine which work by relaxing the smooth muscle and dilating the small blood vessels.
When my patient returns to the clinic, hopefully just for a routine check up, I will be able to have a conversation with her about this disease and not look like a deer caught in headlights. I might be able to learn a few pointers from her to potentially offer future patients with this disease some information.

References

Peer-Reviewed
Herrick, A. L. (2005, May 01). Pathogenesis of Raynaud’s phenomenon. Retrieved February 12, 2016, from http://rheumatology.oxfordjournals.org/content/44/5/587.full.pdf html
Wigley, F. M., MD. (2002, September 26). Raynaud's Phenomenon. Retrieved February 12, 2016, from http://www.nejm.org/doi/full/10.1056/nejmcp013013

Non-Peer-Reviewed
Hansen-Dispenza, H., MD, & Lisse, J. R., MD,FACP. (2015, November 17). Raynaud Phenomenon. Retrieved February 12, 2016, from http://emedicine.medscape.com/article/331197-overview
Mayo Clinic Staff. (2015, March 04). Diseases and Conditions Raynaud's disease. Retrieved February 12, 2016, from http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/definition/con-20022916


Raynaud’s Phenomenon by Dr. Nicolas Gumpert is licensed under CC BY 2.0

10 comments:

  1. From: Jana Andersson

    To: TheGreenLabCoat

    There are a number of aspects about this article that I wish to commend you on. Despite being overall interesting and informative, your opening paragraph – a key element in initiating reader interest – was spot on. The retelling of your personalized workplace experience was perfect and just like a good “who done it movie” I too was compelled to follow you on this journey to learn more about Raynaud's disease. The photo was essential and appropriately placed after your intro which further punctuated the lead-in. Well done!

    As far as one improvement we readers are to required to post I would have to suggest a clearer distinction, visually or rather in flow, between the two classifications PRD and SR, their causes and preventions. (And indeed this is even more tricky when there are areas of overlap.) For instance it wasn't clear on the first reading (to me at least) if the neurological abnormalities and the guidelines were cited for SR or PRD. These two entries followed an SR paragraph but then an SR entry followed these entries. I do realize that you mentioned that SR was associated with Lupus and sclerodoma but as a lay reader to new information (and for diseases that I don't know really about) what seems obvious to a writer may be well “cemented” for the reader – so for me I wasn't able to distinguish between the two without rereading. The info is definitely in there though so again, good job!

    Lastly, one aspect that I am super curious about: why in SR is there is a hardening of the arteries? What's going on there and why?

    Thanks for your personally inspired post. This was such an interesting read and I'm glad we learned more about Raynaud's and that you and your patient can be on the same page about her ongoing condition.

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  2. The patient

    1. I really like the introduction in first paragraph. It gave me an idea and excitement to continue reading and finding out more about this disease that I didn't know anything about.
    2. The author could of explained more in depth why PRP is more common in women than men.
    3. I would like to know more how hormonal and genetic factors are cause of this disease.

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    Replies
    1. From: TheGreenLabCoat

      In depth, I would not know the exact reason. But based on my research, due to the nature of women whom have a higher percentage of lupus and rheumatic issues compared to men, I will assume that this is somewhat linked. Of course this is including Secondary Raynaud’s Phenomenon into the statistic as well.
      Genetically, there is some association as mentioned in the post. As of how and why, here is a link to explain the genetic portion of it.
      http://www.ncbi.nlm.nih.gov/pubmed/16609626

      Hormonally, Estrogen does play a role in a complex situation. I have posted the link that sums it up pretty well if you are interested.

      http://www.lifeextension.com/protocols/heart-circulatory/raynauds-phenomenon/page-02

      Delete
  3. BigFudge

    1) Awesome sources, and as a visual learner I appreciated the image. Good balance of depth and complexity of explanation.

    2) It was unclear if Raynaud's Disease was limited to only vasoconstriction, or if there were any connections vasodilation. You mentioned that Raynaud’s is a “disorder of the blood vessels that causes them to overreact”, which lead me to think both vasoconstriction and vasodilation. However, you followed it with “People who have Raynaud’s disease are unable to regulate the appropriate blood flow when exposed to cold temperatures.” So does this mean that in a perpetually warm climate, they would have little to no problems anymore? Just a bit confused.

    3) I am curious how much pathophysiologic similarity there is between Raynaud’s Disease and urticaria, especially since you mentioned to avoid OTC cold medications.

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    Replies
    1. from: TheGreenLabCoat

      Thank you for the feedback.
      So it is a disorder of both constriction and dilation. When the extremities are exposed to cold temperature (usually in cold environments, sometimes emotional stress) the blood vessels are constricted to help with heat conservation. In normal people, the blood vessels should relax after removing themselves from the cold source. In people with Raynaud’s, this action does not happen as fast or unless there is an outer heat source to help with blood flow to return to hands and feet, this is considered an attack. The vessels are not dilating, instead, they are spasming. So to answer your question: Yes, in theory, there should be very few attacks. But if you throw in the emotional trigger part, that cannot be guaranteed.
      OTC cold meds sometimes have pseudoephedrine, it is considered a stimulant that causes vasoconstriction. Taking medication that has this ingredient may cause symptoms to worsen.

      Delete
  4. OMGmbz

    1) I really liked how you added a little section on ways to prevent an attack. I feel that a person who has this disease would really appreciate this. I also liked how entertaining you made it. Definitely is appealing to those who wish to know more about the disease, but don't have the background in this field.

    2) It was unclear to me about the physiological functions behind this disease. What makes their blood vessels spasm? Is it a too much chemical/electrical stimulus?

    3) Im curious to find out more about how smoking could cause this disease. I also am curious to know why it is more common in women than men. Hopefully future studies could address that further. Maybe it has something to do with our hormone levels.

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    Replies
    1. From: TheGreenLabCoat
      Thank you for the feedback. I hope it may show a tiny bit of insight to people that would like to know about this disease.

      There are multiple reason why people with PR has vasodilation/constriction issues. Hormones play a huge role in regards to blood vessel control. To answer your question, it is more chemical related then electrical.

      Based on what I have gathered. Smoking causes hardening of blood vessels, which indirectly could add to this already complicated situation of a person with PR.

      Delete
  5. OCR-Mama

    Great post! This was already a subject I was interested in, but your intro made me even more interested. I really like how you have laid out the two types of Raynaud’s. I had no idea it affects women more than men.

    My husband has Raynaud’s and I feel so bad for him because he bike commutes which is miserable for him and we have sports events to attend with our children which also triggers it. He also has several family members that have it as well. It make sense that coffee would make it worse and his family drinks a lot of coffee. I don’t think they would listen, but perhaps I can mention the link to them.

    I can attest to the hormonal influence of this. When I was breastfeeding my first child I had this happen frequently with my nipples. It was terribly painful and I dreaded the end of the feed. So my question would be did you learn anything to indicate that it can occur acutely or did it seem like this was a chronic condition?

    Beyond the guidelines of preventing an attack, are there any other lifestyle changes that can improve the condition? You mention blood test and observation are the ways to diagnosis. I would have like to learn more about how Raynaud’s is diagnosed. What are the blood tests looking for? What are the observational signs of Raynaud’s?

    Thanks for writing this post!

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    Replies
    1. Thank you for your feedback. I’m glad it was something you could relate to. Sorry that your husband has to suffer this disease though.
      I do believe this is a chronic situation. As for breastfeeding ( yay! ), I would assume some hormonal components would give similar sensations due to the triggering of vasoconstriction/dilation.
      The below for testing was copied from
      http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/tests-diagnosis/con-20022916
      • Antinuclear antibodies test. A positive test for the presence of these antibodies — produced by your immune system — may indicate a stimulated immune system and is common in people who have connective tissue diseases or other autoimmune disorders.
      • Erythrocyte sedimentation rate. This test determines the rate at which red blood cells settle to the bottom of a tube. A faster than normal rate may signal an underlying inflammatory or autoimmune disease.
      These are some tests to rule out other possible disease to help narrow down the certainty of Raynaud’s

      This website does a great job at summarizing what to do for Pt’s.

      Delete
  6. Thank you for your feedback. I’m glad it was something you could relate to. Sorry that your husband has to suffer this disease though.
    I do believe this is a chronic situation. As for breastfeeding ( yay! ), I would assume some hormonal components would give similar sensations due to the triggering of vasoconstriction/dilation.
    The below for testing was copied from
    http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/tests-diagnosis/con-20022916
    • Antinuclear antibodies test. A positive test for the presence of these antibodies — produced by your immune system — may indicate a stimulated immune system and is common in people who have connective tissue diseases or other autoimmune disorders.
    • Erythrocyte sedimentation rate. This test determines the rate at which red blood cells settle to the bottom of a tube. A faster than normal rate may signal an underlying inflammatory or autoimmune disease.
    These are some tests to rule out other possible disease to help narrow down the certainty of Raynaud’s

    This website does a great job at summarizing what to do for Pt’s.

    ReplyDelete