Browsing Tag

lupus

Diseases and Conditions Overcoming Inflammation

Can UV light trigger lupus flares?

July 12, 2017
Can UV light trigger lupus flares?

Now that summer is finally in full swing, I’d like to remind everyone to use broad spectrum sunscreen while enjoying the sun!  This is especially important for people living with systemic lupus erythematosus (SLE). Ultraviolet (UV) light is a known trigger of SLE flares BOTH involving the skin and major organs.  Many people also report joint pain, weakness, and headaches.  These flares can be very serious.

Although we know UV light is a trigger for SLE flares, we still don’t fully know how it happens.  This is what we do know.

  • UV light directly damages the DNA of skin cells.
  • The cells release inflammatory cytokines, most notably interleukin-1α and tumor necrosis factor-α.
  • UV light also increases interferon-α signaling. People with high levels of interferon-α signaling often develop fevers, fatigue, and low white cell count (leukopenia).  Interferon-α signaling is thought to be an important part in the development of SLE.

Take home points

So while you’re enjoying the sun remember to:

  1. Avoid the sun when UV light is strongest, between 10 AM and 3 PM. If you use IFTTT, check out this app.  You will get a notification on your phone when the UV index is high… and it’s free!
  2. Use broad spectrum UVA/UVB sunscreen.  Try to aim for a SPF higher than 30.
  3. Try wearing clothing that have vivid colors and a tight weave. The Skin Cancer Foundation has a great article regarding this topic: “What is Sun-Safe Clothing?”
  4. Wear a broad-brimmed hat when spending time in the sun.

Be safe and please leave your comments below!

References

 Fernandez D, Kirou KA. What causes lupus flares?  2016 Mar;18(3):14. doi: 10.1007/s11926-016-0562-3.

Medical Disclaimer

This information is offered to educate the general public. The information posted on this website does not replace professional medical advice, but for general information purposes only. There is no Doctor – Patient relationship established. We strongly advised you to speak with your medical professional if you have questions concerning your symptoms, diagnosis and treatment.

Diseases and Conditions

Will hydroxychloroquine hurt my eyes?

May 24, 2017
Will hydroxychloroquine damage my eyes?

I love hydroxychloroquine.  Honestly, I really do.  It’s simple, easy, it works, and for the most part it’s benign especially when compared to the rest of the medications I prescribe.  Rheumatologists use hydroxychloroquine (Plaquenil) to treat of lupus, mild cases of rheumatoid arthritis, and many other autoimmune diseases.

Now I said benign.  Well I actually said, “for the most part it’s benign”.  Allergic reactions are always a concern but the main concern I have with hydroxychloroquine is the possibility of developing eye toxicity.  More specifically, hydroxychloroquine maculopathy.  But before I continue, I think it’s important to go through some anatomy.

Anatomy of the Eye

The following is a simplified version of the human eye.  The cornea is the clear part of the eye that lets you focus light into your eye.  The iris regulates the amount of light you let into your eye.  The pupil is the dark part of the eye.  This is where light actually goes into the eye.  The lens focuses light so that it hits the retina just right and the retina actually senses light.  This info is then condensed onto the optic disc and then sent to the optic nerve then into your brain.

Where does hydroxychloroquine fit in?

Now this is the important part because this is where hydroxychloroquine can cause problems: the macula.  The macula is a specialized place on your retina that has cells that enable you to see fine details with high acuity.  We call these specialized cells cones and they are found in high density in this area.  The macula is then made up of the fovea, foveal avascular zone, parafovea, and the perifovea.  The following is a real life example courtesy of Danny Hope.

By Photograph: Danny Hope from Brighton & Hove, UK Diagram: User:Zyxwv99 [CC BY 2.0 (http://creativecommons.org/licenses/by/2.0)], via Wikimedia Commons


Now the prospect of losing the ability to see things clearly sounds absolutely terrible. I’ve been wearing glasses since I was 13.  Without them, everything is blurry.  I can’t even imagine the blow to my quality of life, if I didn’t have my glasses.  Hydroxychloroquine is kind of like that.  Your vision becomes blurry, except glasses won’t help, and the vision loss is permanent.

So why would anyone go on this medication and why would your doctor even suggest it?

Well… because it’s actually a pretty good medication.  Just like most medications, you need to take it as safely as possible.  The American Academy of Ophthalmology released a statement last year about monitoring hydroxychloroquine.  Let’s go over these recommendations together!

Recommendations on Screening for Hydroxychloroquine Retinopathy (2016 Revision)

First of all, it’s still unclear how exactly hydroxychloroquine causes eye toxicity.  In a nutshell the outer layer of the retina gets damaged and then it deepens and spreads around the fovea.  People tend not to notice anything at this stage. Over time, if the medication is not stopped, the fovea becomes involved and visual acuity drops.  It’s also important to note that hydroxychloroquine can worsen even after stopping the medication.  If it’s caught early on, it probably won’t affect vision.  If there already was a lot of damage to begin with, then the risk is higher.  So the real question is what is the real risk of developing toxicity, what are the factors that increase that risk, and how often should you get screened by an ophthalmologist?

What is the real risk of developing hydroxychloroquine eye toxicity?

In the past, we thought the risk of developing eye toxicity from hydroxychloroquine was very low.  New data suggests otherwise.  Although the risk is still low, we were probably underestimating the risk.  Researchers following 2,361 people using hydroxychloroquine, found that about 7.5% of those people had eye toxicity.  The most important risk factors included the daily dose of hydroxychloroquine and duration of use.

People who took 4 to 5 mg/kg/day of hydroxychloroquine had a much lower cumulative risk as compared to people you took a higher dose: 1% risk in the first 5 years and less than 2% up to 10 years.  After 20 years the risk dramatically increased to 20%.

When I mean mg/kg/day, I mean the amount of drug for every kilogram of body weight over a 24 hour period.  To calculate the dosage of hydroxychloroquine you need to use your real weight, NOT your ideal weight.  For some medications, it’s the opposite.  Let’s say you were taking hydroxychloroquine 200 mg twice a day and then you started dieting and exercising, and then you shed a lot of weight.  You now may need to decrease your daily dose of hydroxychloroquine because your real weight decreased.  In the study, the researchers found that thin people tended to have more eye toxicity because they tended to get more than 4 – 5 mg/kg/day of hydroxychloroquine.


FYI When calculating your body weight to verify your hydroxychloroquine dose, you need to use metric.  This is not optional.

1 kilogram = 2.2 pounds


Other Significant Risks

Initially we thought hydroxychloroquine gets stored in fat cells.  In actuality, recent lab studies show that the medication is mostly stored in melanotic tissue, liver, and in the kidneys.  Muscle, fat, and other organs not so much.  That being said, people with severe kidney disease are at higher risk of developing eye toxicity.  These people may need more frequent eye testing and they may not need as high of a dose.

Other significant risks includes concurrent use of tamoxifen, which is a medication commonly used to help treat breast cancer.  The researchers found that there was a 5-fold increase of toxicity in people taking tamoxifen and hydroxychloroquine.  Why?  Tamoxifen itself can affect the retina, so maybe having both on board simultaneously isn’t such a great idea.

Finally, people with macular or retinal issues, like having macular degeneration, may also be at higher risk.  There wasn’t enough results to confirm this, but it kind of makes sense.  If he retina isn’t too hot to begin with, it’ll probably be difficult for the ophthalmologist to decide whether future changes are medication-related versus disease-related, in this case macular degeneration.  Do you need to stop hydroxychloroquine?  Or do you need to start ranibizumab (i.e., a medication FDA approved for macular degeneration)?

Screening Schedule

As I mentioned before, hydroxychloroquine eye toxicity is not reversible.  Once it happens, it happens.  So the trick is to catch it early.  Fortunately, the changes occur VERY slowly.  The American Academy of Ophthalmology recommends the following:

  • Obtain a baseline eye exam within the first year of starting hydroxychloroquine to document any complicating eye problem.
  • Annual screening beginning after 5 years of use.
  • Sooner if there are major risk factors.
  • Check the dose of hydroxychloroquine based on your weight at your doctor’s appointment.
  • Inform your doctor if there’s been any significant change in your health: significant weight loss (intentional or unintentional), kidney disease, or if you’ve been prescribed tamoxifen.

Now you may wonder why your rheumatologist insists on annual eye checks even though you’ve been on hydroxychloroquine for less than 5 years.  This is probably a matter of style.  Personally, I’m one of those rheumatologists that insists on annual eye checks.  I wouldn’t feel comfortable NOT seeing my ophthalmologist if I was taking a medication that had retinal toxicity.

Screening Tests

There are many different techniques to screen for toxicity.  I won’t go into specifics because, well, I’m not an ophthalmologist.  However, here is a list of techniques that the American Academy of Ophthalmology approved to screen for hydroxychloroquine eye toxicity.

  • Automated visual fields
  • Spectral-domain optical coherence tomography
  • Multifocal electroretinogram
  • Fundus autofluorescence
  • Microperimetry – newer test, possible value in future
  • Adaptive optics retinal imaging – newer test, possible value in future

These tests are not recommended for screening

  • Fundus examination
  • Time-domain optical coherence tomography
  • Fluorescein angiography
  • Full-field electroretinogram
  • Amsler grid
  • Color testing
  • Electro-oculogram

If you have any questions about these tests, please ask you ophthalmologist.

Conclusion

Hydroxychloroquine is truly wonderful and useful medication for the treatment of multiple different types of autoimmune diseases.  Although eye toxicity is a real danger, the risk is usually small especially in the short-term.  But like I said at the beginning, like medications you need to take it safely and responsibly.  To learn more about medication safety, please read my article regarding the 10 most frequently asked questions when starting methotrexate.

Please leave your comments below!


By Jessica Chapman, M.D.

References

https://commons.wikimedia.org/wiki/File:Schematic_diagram_of_the_human_eye_en.svg

https://commons.wikimedia.org/wiki/File:Macula.svg

Marmor MF, Kellner U, Lai TY, Melles RB, Mieler WF, American Academy of Ophthalmology. Recommendations on screening for chloroquine and hydroxychloroquine retinopathy (2016 revision). Ophthalmology. 2016 Jun;123(6):1386-94.

UpToDate

Diseases and Conditions

How do autoimmune diseases affect pregnancy?

April 5, 2017

By Jessica Chapman, M.D.

This past week I was asked to discuss the topic of pregnancy and rheumatic diseases.  This is a major issue as many people that develop autoimmune disease are young women.  If you so happen to be one of those young women and you thought that pregnancy was out of the question for you because of your illness… think again.

For the most part, most women with autoimmune disease can have successful pregnancies.  That is, with careful plan and coordination between your doctors.

General tips

It’s extremely important to have a frank discussion with your rheumatologist before thinking about conceiving.  If you have a hematologist, nephrologist, pulmonologist, cardiologist, or any other specialist that also plays a role in caring for your autoimmune, then they should be involved as well.  It’ll also me crucial for you to establish care with a high risk OB-GYN. I did say that it is possible to have a successful pregnancy, but it won’t be your average type of pregnancy.  All this should be done between 6 -12 months prior to attempting conception.

The following are typically recommended.

  • For best outcome, conception should be attempted during periods of low disease activity.
  • All high risk or teratogenic medications should be replaced with low risk medications.  This one can be tricky at times.  Remember that most of the medication have very very long washout periods.  So just because you stopped methotrexate a week ago, it’s still there lingering.
  • You should be assessed for high risk autoantibodies.  These include SSA (anti-Ro) and SSB (anti-La) antibodies as well as all the antiphospholipid antibodies.
  • If you plan on breastfeeding, the choice of medication should also factor into the decision.

Antibodies

SSA and SSB antibodies

These antibodies are typically associated with Sjogren’s syndrome but can also be present in a variety of different conditions such as lupus and rheumatoid arthritis.  It’s important to know whether you have these antibodies because they increase the chance of neonatal lupus.  Maternal SSA and SSB antibodies can passively transfer in utero to the baby.  The risk is small, 1-2% of cases but the risk significantly increases in women who have already borne a child with neonatal lupus.

Children that are born with neonatal lupus may have a rash, liver problems, and low blood cell counts but thankfully, these symptoms disappear completely after about six months.  I did not find research finding that have neonatal lupus increases the chance of the child developing systemic lupus erythematosus (SLE).  Research is research and things may change with time.

The most dreaded complication of neonatal lupus is complete heart block, which typically occurs at weeks 20 – 22.  It’s currently not recommended to use prophylactic steroids to prevent this from happening, but rather to get frequent cardiac monitoring.  This is where the high risk OB-GYN comes into play.

Antiphospholipid antibodies

There are three types of antiphospholipids: lupus anticoagulant, anticardiolipin antibodies, and beta-2 glycoproteins.  The presence of these antibodies, particularly at high titers, are associated with something called the antiphospholipid syndrome.  People that have this condition are  higher risk of developing bother arterial and/or venous blood clots.  Other complications include: miscarriage, stillbirth, pre-term delivery, and preeclampsia.  In an effort to try to prevent some of these complications, women that have antiphospholipids typically treated with prophylactic “pregnancy-safe” blood thinners.

High risk medications

Because it would be completely and utterly unethical to conduct a randomized controlled study on pregnancy outcomes and exposure to disease modifying agents (DMARDs), the vast majority of evidence regarding the safety of medications during pregnancy comes from animal models.  There are also a few registries that capture human data.  As we all know, not all pregnancies are necessarily planned and for some women having active disease may be worse for the baby or the mother, so the benefits of the medication may outweigh the risks.  Every situation is unique.

The American College of Rheumatology has a nice table listing medications that typically are used to treat rheumatic conditions.  Which is considered safe in pregnancy and breastfeeding.  In general the antimalarials, like hydroxychloroquine, are safe as well as azathioprine and sometimes sulfasalazine.  Mounting evidence also suggests that the TNF-alpha inhibitors may also be safe, but this isn’t set in stone.  Many rheumatologists are still on the fence regarding this one.  If a TNF-alpha inhibitor is absolutely required, it may be a good idea to use certolizumab as this medication is pegylated and technically should not cross the placenta.  It is VERY important to have a discussion with your rheumatologist regarding your medications!

How does pregnancy affect disease activity?

It all depends on the disease we are talking about.  Typically SLE becomes more active during pregnancy, whereas rheumatoid arthritis and psoriatic arthritis tends to improve.  Typically vasculitis, polymyositis, dermatomyositis, and systemic sclerosis are unaffected by pregnancy.

High risk clinical situations

HOWEVER, there are a few notable circumstances where pregnancy is risky for both the mother and the child.  It’s generally not safe to conceive during a period of very high disease activity.  This includes lupus nephritis.  It’s generally discouraged to conceive when lupus nephritis is active, but when things are controlled it’s okay with very close monitoring.  Patients with lupus nephritis commonly flare, are at higher risk of preeclampsia, and HELLP syndrome during pregnancy but these can be reversed with prompt treatment.

Another very important high risk situation is pulmonary hypertension.  This can occur in variety of disease such as systemic sclerosis, SLE, myositis, mixed connective tissue disease, Sjogren’s syndrome, and rheumatoid arthritis.  When pulmonary hypertension is caused by an autoimmune disease, we call it connective tissue disease-related pulmonary arterial hypertension.  For those who want to get technical, pulmonary hypertension is defined as a mean pulmonary arterial pressure higher than 25 mmHg at rest, associated with a pulmonary capillary wedge pressure lower than 12 mmHg diagnosed by right heart catheterization. Symptoms include:

  • Shortness of breath particularly while exercising.  Like more than what you would expect.  At one point the shortness of breath even when you are at rest.
  • Fatigue
  • Dizziness, passing out.
  • Chest pain
  • Swollen legs or swollen abdomen (ascites)
  • Palpitations
  • Bluish color

So why is this especially important for pregnancy-related matters?

A study by Qian et al. aimed to evaluate the survival of patient with SLE-associated pulmonary arterial hypertension.  This was a systematic review and meta-analysis.  They identified 6 studies which included a total of 323 patients.  They found that 1-, 3-, and 5-year survival rates were 88%, 81%, and 68% respectively.  The more severe the pulmonary hypertension, the worse the outcome.  These are not good odds.

High pulmonary hypertension peripartal mortality is not an isolated incident related to SLE alone.  It is elevated for all connective tissue disease-related caused of pulmonary arterial hypertension.

Conclusion

We’ve come a long way when it comes to rheumatic diseases and pregnancy.  Way back when, it was generally discouraged in practically all circumstances.  But things have changed.  With careful planning and monitoring, many women with autoimmune diseases can now have safe pregnancies.

References

Kelley and Firestein’s Textbook of Rheumatology, tenth edition

American College of Rheumatology

Pasut G. Pegylation of biological molecules and potential benefits: pharmacological properties of certolizumab pegol.BioDrugs. 2014 Apr;28 Suppl 1:S15-23.

Moroni G, et al. Maternal outcome in pregnancy women with lupus nephritis. A prospective multicenter study. J Autoimmun. 2016 Nov;74:194-200.

Thakkar V, Lau EM. Connective tissue disease-related pulmonary arterial hypertension. Best Pract Res Clin Rheumatol. 2016 Feb;30(1):22-38.

Qian J, et al. Survival and prognostic factors of systemic lupus erythematosus-associated pulmonary arterial hypertension: A PRISMA-compliant systematic review and meta-analysis. Autoimmun Rev. 2016 Mar;15(3):250-7.

Diseases and Conditions

What is Raynaud’s Phenomenon?

February 1, 2017
Raynaud's phenomenon: Classic white discoloration of the pinky

What is Raynaud’s?  They do say that a picture is worth a thousand words.

In a previous article about scleroderma, I alluded to something called Raynaud’s phenomenon.  Tis the season of the Raynaud flare, so I thought this post would be especially relevant.

This phenomenon is super common.  Some studies estimate that it occurs in 3 to 4% of the population but it’s probably a lot more than that.  In colder climates its is present in up to 30% of the population.  It’s more common in women, in younger people, and it tends to run in families.

Raynaud’s is a vasospastic disorder.  Basically the blood vessels clamp up when exposed to the cold, when there is a sudden change in temperature, or sometimes when you are extremely stressed out.  No joke!  Cigarette smoking is also a known cause.  Typically, the finger goes white, then dusky, and when the blood comes back, bright red.  If the change in color involves the palm, this is not Raynaud’s.

Raynaud’s can be associated with many conditions.  And they’re not all autoimmune.

  • Autoimmune – scleroderma, lupus, rheumatoid arthritis, Sjogren’s syndrome, myositis
  • Vibration injury – riveters, rock drillers, etc.
  • Frost bite
  • Medications and chemicals – beta blockers, cocaine, chemo
  • Arterial diseases – carpal tunnel, clots, pressure from a crutch
  • Hormonal diseases – low thyroid, pheochromocytoma
  • Hyperviscosity syndromes – paraproteinemia, polycythemia, cryoglobulinemia
  • Infections – Lyme, hepatitis, endocarditis
  • Cancers – ovarian cancer, lymphoma, leukemia

And then there’s the most common cause… just cause.  Idiopathic primary Raynaud’s.  Typically, this occurs in women during their teens or 20’s.  There are a lot more causes but these are some of the common and… not so common ones.

Primary Raynaud’s vs. Secondary Raynaud’s

When Raynaud’s is idiopathic, this means that it is NOT caused my any underlying condition.  This is primary Raynaud’s.  When there IS an underlying condition, we are dealing with secondary Raynaud’s.

In primary Raynaud’s, the blood vessels are structurally normal.  In secondary Raynaud’s, the structure of the blood vessels can deform because of the underlying cause.

Secondary Raynaud’s can cause finger ulcers, pitting, fissuring, and gangrene.  People tend to have an ANA and or antibodies.  In addition sometimes the doctor can actually see that the blood vessels above your nails look distorted using a pocket microscope.    While, you don’t see any of these things with primary Raynaud’s.

Can people with primary Raynaud’s develop secondary Raynaud’s

The simple answer is YES.  About 1% of patients with primary Raynaud’s develop some form of autoimmune disease yearly.  There are a few risk factors that increase that risk.

  • Having any specific antibody like anticentromere antibodies
  • Abnormal blood vessels above your nails
  • Having a ANA with a nucleolar pattern
  • First flare after the age of 40 years
  • Male gender
  • Finger ulcers, pits, gangrene

If you have any or a few of these, it’s important to see a rheumatologist periodically to make sure that you aren’t developing an autoimmune disease.  Early diagnosis is key.

How do you treat Raynaud’s?

Keep your hands and feet warm and, keep your core temperature warm.  Easier said than done.  I know, I grew up in Canada!  The trick is layering, mittens, scarves, and warm socks. Need to remove your gloves to answer your phone?  Don’t.  Either wear touchscreen friendly gloves or convert them.  I recently discovered nanotips.  It does the job.  Note, this is NOT an affiliate marketing link.  Basically, try to avoid triggers like the cold, stress, or any chemicals that could cause it in the first place.  Known chemicals include cigarettes, decongestants, diet pills… stimulant drugs.  Some heart medications may also worsen Raynaud’s.  Before making any changes, talk to your doctor.

Fish oil to treat Raynaud’s

When it comes to conventional medications, you do have a few options.  Provided you are not allergic to fish, omega-3 fatty acid supplementation could be beneficial.  One of my teachers/colleagues, conducted a double-blind, controlled, prospective study looking at fish oil supplementation in patients with both primary and secondary Raynaud’s phenomenon back in the 80’s.  Basically, 3 grams of fish oil daily improves tolerance to cold exposure and delays the onset of vasospasm in patients with primary Raynaud’s.  This effect was not seen in people with secondary Raynaud’s.

Medications for Raynaud’s

Finally, doctors sometimes prescribe medications like calcium channel blockers: amlodipine and nifedipine.  These are generally considered first-line and help about 35% of people.  Calcium channel blockers like verapamil and nicardipine are essentially useless.  Other medications include prazosin, sildenafil, and talalafil.  All of these can decrease your blood pressure.  For those with normal or low blood pressure, fluoxetine is a possibility.  Although this is an anti-depressent, it’s thought that the increase in serotonin dilates the blood vessels thereby alleviating Raynaud’s.  Sometimes topicals are effective: nifedpine and nitroglycerine.  But again, sometimes they can cause a drop in blood pressure.   Again, this does not constitute medical advice.  Please talk to your doctor before making any change.

What about finger or toe threatening situations

Sometimes Raynaud’s is particularly severe and refractory to medications.  This tends to happen with people suffering from scleroderma.  Sometimes, you can see gangrene because the decrease in blood flow is so severe.  Obviously, you don’t want it to get to that point.  In these situations, hospitalization often times is necessary.  Consequently doctors use medications like epoprostenol or iloprost to aggressively open up those blood vessels.  Surgical intervention may also be necessary: sympathectomy.  It can be performed at the cervical, lumbar, wrist, or digital (finger) levels.  These interventions are reserved for very severe cases.

What’s the prognosis

For primary Raynaud’s prognosis is excellent.  Simply a nuisance for the most part.  However, in high risk people, it’s important to see a rheumatologist on an annual basis to see if anything changes.  Thankfully, in about 10% of people the attacks disappear completely with time.

In contrast, things are little more complicated with secondary Raynaud’s.  It really depends on the underlying problem.  Remember this is treatable.  Not curable, but treatable.

I hope this has been informative and enlightening.  Remember, bundle up and stay warm this winter!

References

Rheumatology Secrets, 3rd Edition

DiGiacomb RA, Kremer JM, Shah DM. Fish-oil dietary supplementation in patients with Raynaud’s phenomenon: a double-blind, controlled, prospective study. Am J Med. 1989 Feb;86(2):158-64.

Diseases and Conditions Overcoming Inflammation

Simple and easy ways to hydrate your skin

January 23, 2017

This past Christmas, I received probably one of the most bizarre gifts I have ever received.  A full adult-sized onesie! Let me tell you, it hasn’t really brought out the most flattering bit of my anatomy.  Last year I had lamented that my house was kind of cold in the winter and mentioned that babies had it made with their one-piece pajamas.  That being said, I decided to use my family’s superhuman memory to my advantage.  This year it was all about my dry skin and how wonderful it would be to have a paraffin wax machine.

I have to admit it though, that onesie does keep me nice and toasty at night!

While waiting for the paraffin wax machine that hopefully will be making its apparition Christmas 2017, hint-hint, I thought I’d do a little research about the topic of dry skin: anatomy, immunology, and basically how to keep it as moist as possible without that oily feeling.  People who suffer from autoimmune diseases tend to suffer from dry skin.  This really isn’t surprising.  This skin is the largest organ in the human body.  Because of its large surface area, it needs a large complex immune system.  Autoimmune diseases = inflammation and inflammation involving the skin = dryness, irritation, and itchiness.

Anatomy and immunology

The medical term for dry skin is xerosis and the term dermatitis signifies inflammation of the skin.  The skin is made up of the epidermis which is the most superficial layer followed by the dermis and then the subcutis.  These layers are made up of blood vessels, hair follicles, as well as glands.  One of the main functions of the skin is to protect your body from the outside world.  That being said, the skin contains many immune cells such as Langerhans cells, neutrophils, eosinophils, and lymphocytes.  When skin is irritated by bacteria, viruses, chemicals, the immune system kicks in to fight off the foreign invader.  With autoimmune diseases, it’s the immune system that starts to attack the skin itself.  In either case, when the immune system is activated it causes inflammation which = irritation + dryness.

There are many autoimmune diseases that affect the skin.  Some of these include scleroderma, Sjögren’s syndrome, rheumatoid arthritis, lupus, sarcoidosis, and psoriasis.

So what can you do to keep your skin moist and healthy? Drink plenty of water, limit the amount of exposure of your skin to water, protect your skin from the sun, avoid allergens, and moisturize regularly.

Avoiding irritation and dehydration

You would think that more water equals more hydration.  That may be true for ingested water, but it’s the opposite for water that makes direct contact with your skin.  For example, every healthcare worker soon learns that there is a positive correlation between the dryness of their hands with the amount of times they wash their hands per day.  Don’t get me wrong washing your hands is very important to prevent the spread of infection, but it certainly does a number to your skin.  Some things simply cannot be avoided.  That being said, if you suffer from dry skin, try to limit the amount of time you spend washing in the shower.  Make it a quick 5 min instead of a long 30 min shower.  Another tip is to use lukewarm water instead of hot water.

For people who shave, try to shave immediately after you shower.  The hair will be much more malleable at this time.  Always use a sharp razor and always shave with the line the hair grows.  These will lessen the amount of irritation caused by shaving.

This may sound obvious, but it’s really important to prevent your skin from burning.  Burnt skin = dehydration.  If you plan on being in the sun, try to stay in the shade between 11 AM and 3 PM.  The UV is at its highest during these hours.  Aim to cover up with clothing made of light-colored cotton.  Light colored clothing adds a few extra SPF points.  Broad-brimmed hats and sunglasses are your friends.  Use sunscreen liberally.  At least 15 SPF.  For people suffering from lupus, plan for a much high SPF as UV can actually trigger a full-fledged systemic flare.

What I mean by avoiding allergens means, try to avoid anything that may cause some form of allergic reaction.  Again allergic reaction = inflammation = deterioration of skin barrier function = dehydration.  Obviously, it’s impossible to avoid everything but it may be a good idea to swap perfumed household products for hypoallergenic ones: laundry detergent, softener, soap, shampoo, moisturizer etc.  Anything that directly or indirectly makes contact with your skin.  It’s important to note that even, “all natural” products can potentially contain allergens.  For example, most people aren’t allergic to Shea butter, but some are.  Learn to know your skin.

Hydrate you skin

Are you as confused as I am when it comes to moisturizers?  Which ones are good?  Which ones are bad?  What’s the difference between a lotion, cream, and an ointment?  What goes where?  How much should I apply?  How often?

It’s essential to moisturize daily particularly those suffering from dry skin conditions.  Simply put, dry skin is determined by the amount of transepidermal water loss and this in term is determined on the integrity of the skin barrier function.  The composition of the moisturizer determines whether the treatment helps skin barrier function or not.  I can’t tell you which moisturizer is better than the other, because I haven’t found any blinded head-to-head evidence-based studies addressing this topic.  If you do find one, let me know.  I’m all ears!

Moisturizers come in various forms: lotions, creams, and ointments.  Lotions are the least greasy and ointments are the greasiest.  Typically, the greasier the moisturizer, the longer it lasts.  The questions what goes where and how often and how much to apply, may actually be over-complicating the matter.  The goal is to keep the skin nice and hydrated.  When you really think about it, consistency is key.  Would you wear a thick greasy ointment on your hands all day long?  I wouldn’t because it’s uncomfortable and quite frankly not practical.  Due to my job description, I wash my hands nearing 100 times a day.  I’d rather use a cream or lotion and simply apply it more often.  The ointment might be better tolerated at night before going to bed?  If I’m comfortable, I’m more likely to wear the moisturize regularly.  How much to apply?  Apply enough so that the skin feels moist.

Like I said, there’s no need to complicate things.

One word of advice, when applying a moisturizer, try to stoke it onto the skin in the direction that the hair naturally falls.  This can prevent folliculitis.

Caution

A little bit about miracle cures.  They don’t exist.  Any product marketing itself to be a “cure for psoriasis”, is probably a product to be avoided.   A lot of these products have high doses of corticosteroids, which may initially make the skin look more hydrated and look “healthier”.  If you suffer from psoriasis, it may even clear it up.  But in the long run, regular application will cause permanent skin thinning, aging, and atrophy.  Just like food, read the ingredients on the packaging of your moisturizer.

When it comes to diet, in general beware of any diet advocating cutting out lists of foods.  For the most part, these are not founded in evidence and you actually may be doing more harm than good.  Nothing beats a clean diet and plenty of water.  What that actually means, is a matter up for debate.  For more information regarding clean eating, I recommend visiting the Blue Zone Project by Healthways.  The Blue Zone principles were derived from a National Geographic study identifying practices in cultures where people tend to live longer, i.e., greater than 100 years of age, and healthier as compared to the normal population.  When you have entire populations of people living longer and healthier over  thousands upon thousands of patient years, it makes me think they’re onto something.

Parting words

I hope you’ve found this information useful.  If you would like more information, please contact your local physician.  Love your skin, keep it nice and hydrated!

References

Dermatology Secrets Plus, 5th edition copyright 2016 by Elsevier

American Academy of Dermatology

Loden M. Effect on moisturizers on epidermal barrier function. Clin Dermatol. 2012 May-Jun;30(3):286-96.

Penzer R. Providing patients with information on caring for skin. Nurs Stand. 2008 Nov 5;23(9):49-56.

Diseases and Conditions Featured

What is an ANA and what does it mean?

December 12, 2016

The antinuclear antibody is one of the worst tests in the history of all humanity.  It is my constant companion.  Why… I’ll get to that.

First, a little background.  What exactly is an antinuclear antibody, also known as an ANA?  In simple terms, an ANA is an antibody that is directed towards or attacks the nucleus of a cell.

How is an ANA measured?

The ANA is calculated by taking a standardized cell from the lab and mixing it with a person’s blood.  If a person has antinuclear antibodies, these will stick to the standardized cells’ nuclei.  At this point, there’s no way for us to know whether this has happened, so the lab tech adds fluoresceinated antibodies to the mix.  These antibodies bind to ANAs that stuck to a nucleus.  With the help of a specialized microscope, the lab tech can now visualize the ANA because the fluoresceinated antibodies make them light up like a Christmas tree.

My doctor told me my ANA was high.  What does that mean?

Unfortunately, the tech cannot count how many ANAs they see.  Instead, they see how much they can dilute the person’s blood and still see the fluoresceinated antibodies.  So when you see and ANA of 1:80, that means the tech really wasn’t able to dilute very much.  This is a low level.  If you see a value of 1:640, that means they were able to dilute way more.  This is a higher level.

What is considered a positive ANA?  That answer really depends on the lab.  Every lab has different cut off values, but in general, an ANA of 1:80 is typically considered positive.  Whether it is clinically significant, is a whole different question.  This is where the art of medicine comes into play.  But before that, let’s talk about patterns because those are important too.

So let’s take an example.  Your doctor runs an ANA and it comes back as 1:320 speckled pattern.  So what does that mean?  When the lab tech was looking at the fluoresceinated antibodies, it basically literally looked speckled.  There are many other kinds of patterns: homogenous, centromere, nucleolar, speckled, rim etc.  Each of these patterns are associated with a set of specific nuclear antibodies.  For example, the speckled pattern is associated with SSA, SSB, RNP, Smith, and Ku antibodies.  These specific nuclear antibodies are themselves associated with specific autoimmune diseases.  It’s important to take ANA patterns with a grain of salt because interpretation highly depends on the experience of the lab tech.

I’m not going to go more into details regarding specific nuclear antibodies because first, there’s about 150 of them and second, they’re all associated with different diseases lupus being one of them.  That’s a lot of material to cover in one article.

When is an ANA clinically significant?

Now that we understand what an ANA actually is, we can now start to approach the subject of clinical significance AND when it is appropriate to test someone for it.

The problem with the ANA is that it can be found in normal healthy people.

  • ANA 1:40 is found in 20 – 30% of healthy people
  • ANA 1:80 is found in 10 – 15% of healthy people
  • ANA 1:160 is found in 5% of healthy people
  • ANA 1:320 is found in 3% of healthy people
  • ANA can be positive in 5 – 25% of healthy people with a family member suffering from lupus
  • ANA can be positive in up to 70% of people aged above 70 years

To complicate things even more, an ANA can be present in someone that is about to develop an autoimmune disease… UP TO 10 YEARS before they actually develop the disease.  It can be positive in people suffering from cancer, people suffering from infections like tuberculosis, HIV, hepatitis C, mononucleosis etc.  It can even be positive when people are taking certain medications.  Not terribly not helpful right?

Bad example

So someone runs an ANA just because and it’s positive.

  1. Does it mean anything?
  2. Is the person one of those healthy normal individuals that has a positive ANA?
  3. Is the person going to develop an autoimmune disease in the future?

In this scenario, I would say that this test is of low clinical significance because it was obtained without purpose.  Because so many people that are completely healthy have an ANA, the test should only be run if a person is manifesting a symptom or better yet, multiple symptoms that are suggestive of an autoimmune disease like lupus, Sjögren’s syndrome, systemic sclerosis, mixed connective tissue disease, etc.  In that situation, it is helping answer a question.

Good example

If you’ve read my previous post, 8 important warning signs of scleroderma, you’ll remember that Raynaud’s phenomenon is an important red flag for scleroderma.  The vast majority of people suffering from Raynaud’s has no underlying autoimmune disease.  A small proportion does.  This is the perfect scenario, where an ANA would be useful.  If the ANA is negative, the person likely will NOT develop an autoimmune disease.  If the ANA is positive, then the person it at high risk of developing an autoimmune disease like scleroderma.  This person should be monitored more closely and probably have some follow-up blood tests.

Let’s wrap things up

Ultimately it all boils down to this simple fact: doctors treat people not numbers.

As a physician I care about symptoms and signs way more than unreliable lab tests.  Don’t get me wrong, these tests are important.  For example, over 99% of people suffering from systemic lupus erythematosus have a positive ANA.  It’s pretty much safe to say that if someone tests negative for ANA, they likely don’t have lupus.  FYI that other less than 1% usually has a positive SSA, they have a problem with their complement system, or they have a lot of protein in their urine (nephrotic syndrome).

I hope I’ve helped you better understand the elusive and mysterious ANA.  Again, this information does not constitute medical advice.  If you’ve tested positive for an ANA and have more questions, I highly encourage you to speak with your physician or local rheumatologist.  And remember, symptoms, symptoms, symptoms.

References

Rheumatology Secrets 3rd edition

 

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