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When to see a rheumatologist

Diseases and Conditions When to see a rheumatologist

10 Warning signs you could have Sjögren’s syndrome

March 1, 2017
Sjögren's syndrome can cause dry eyes and dry mouth as well as many other symptoms. Read on to learn more!

Today is a most bizarre weather-related day.  It’s warm, like you don’t need a coat warm, and there’s a raging thunderstorm.  Did I mention it’s February in upstate New York?  In honor of this most bizarre day, I’d thought I’d write a few words on a somewhat bizarre and illusive autoimmune disease called Sjögren’s syndrome.

Henrich Sjögren gave Sjögren’s syndrome its name.  He was a Swedish physician who first described the disease in 1933.  Sjögren’s syndrome is a common autoimmune disease that primarily causes dryness.  But it’s a lot more complicated than that because Sjögren’s syndrome can involve almost any organ so can present with a myriad of symptoms.  The symptoms arise from infiltration of lymphocytes into glands and affected organs.  Simply put, Sjögren’s syndrome is on the differential diagnosis in any person who has a positive ANA presenting with unexplained symptoms.

10 Warning signs you may be suffering from Sjögren’s syndrome

The following are some of the common manifestations of Sjögren’s syndrome.  Believe me, there are A LOT more but these are some of the common ones.

  1. Dry eyes
  2. Dry mouth
  3. Swollen cheek(s) i.e., parotid gland enlargement
  4. Profound tiredness
  5. Joint pain, sometimes with swelling
  6. Swollen glands
  7. Numbness, tingling, burning of the skin
  8. Raynaud’s
  9. Shortness of breath with minimal work
  10. Having a child that suffered from congenital heartblock

Dry mouth symptoms

The following are some common symptoms of dry mouth.

  1. Difficulty swallowing dry foods
  2. Inability to talk continuously
  3. Change in taste
  4. Burning sensation
  5. Large dentist bill! – Cavities, cracked teeth, loose fillings
  6. Problems with your dentures
  7. Worsening heartburn
  8. Thrush

As you can see the symptoms are a little all over the place and quite frankly are kind of vague.  Furthermore, many different conditions can mimic some of these symptoms: dehydration, depression, various medications, uncontrolled diabetes, multiple sclerosis, hepatitis C, sarcoidosis, etc etc.  Literally.

Classification criteria

Now it’s important to note that the following classification criteria are used for research purposes, and not necessarily for the day-to-day clinic.  Although they are important, there is such a thing called the art of medicine.

As we all know, not everyone fits into a neat little box.

Recently the American College of Rheumatology and the European League Against Rheumatism came up with a new system to classify Sjögren’s.  Basically, a group of hot-shot Sjögren’s specialists got together, looked at the literature, probably had more than one heated discussion, and came up with the following.

To test positive you need to have a score ≥4.  There are five items but they are weighted differently.

  • 3 Points – Anti-SSA/Ro antibody positivity
  • 3 Points – Focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2
  • 1 Point – Abnormal Ocular Staining Score of ≥5 (or van Bijsterveld score of ≥4)
  • 1 Point – Schirmer’s test result of ≤5 mm/5 mi
  • 1 Point – Unstimulated salivary flow rate of ≤0.1 mL/min, each scoring = 1

The sensitivity of this score is 96% and the specificity is 95%.  The sensitivity tells you how likely you are to detect all cases of Sjögren’s syndrome and the specificity tells you how accurate you are with the diagnosis using these set of diagnostic criteria.  These are pretty good figures.

What does this mean?

As you can see, the diagnosis favors objective findings, NOT symptoms.  This is a huge change from the previous set of diagnostic criteria.  You’ll also note that positive ANA, rheumatoid factor, and positive anti-SSB/La antibody positivity are not included in the new classification criteria.

Now I don’t want people thinking that I think symptoms are unimportant.  They are VERY important.  It’s just that symptoms should prompt a workup looking for objective features of the disease.

Now, try to remember the 10 warning signs.  If you find yourself checking a few of these items, check-in to your local rheumatologist.

References

Rheumatology Secrets 3rd edition

Shiboski CH, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts.Ann Rheum Dis. 2017 Jan;76(1):9-16.

Diseases and Conditions When to see a rheumatologist

What is autoimmune back pain?

December 26, 2016
What is autoimmune back pain

Let’s face it, back pain is terrible and unfortunately for us, very common.  According to the 2016 Global Burden of Disease Study, low back pain is the number one cause for disability globally.

So we know back pain is common and debilitating, but the question is, what is the difference between regular and autoimmune back pain?

There are two broad categories: mechanical back pain and inflammatory or autoimmune back pain.  Many but not all autoimmune diseases cause back pain.  For example, rheumatoid arthritis usually does not cause back pain.  Sometimes these disease are very difficult to diagnosis because they progress very slowly.  Often times it takes years to decades to diagnose.  Yes you heard me, DECADES.  Unfortunately, if left untreated, it can cause irreversible spinal damage: joint erosions and spinal fusion also known as ankylosis.  Prompt treatment with a disease modifying anti-rheumatic agent (DMARD) often can slowdown or prevent joint damage.

Autoimmune back pain

As I was mentioning before, mechanical and autoimmune back pain are completely different.  Mechanical back usually improves with rest, worsens with strenuous activity, and usually begins later in life.  People that have autoimmune back pain experience the opposite.  Here are some of the key features.

  • Usually pain starts during the 2nd or 3rd decade of life.  People usually experience symptoms before the age of 45
  • Onset is gradual for the most part
  • Symptoms have been ongoing for more than 3 months
  • Exercise improves the pain
  • Rest really doesn’t help.  The pain usually worsens with rest
  • Anti-inflammatory medications like naproxen or ibuprofen helps the pain
  • Pain wakes you up during the second half of the night
  • Pain and prolonged stiffness in the morning. Typically, stiffness lasts more than one hour
  • Alternating deep buttock pain

Risk factors

Autoimmune disease rarely occur in isolation.  The following are some of the risk factors pointing towards a diagnosis of autoimmune back pain.

  • Personal history of uveitis. This is a type of inflammatory condition that affects the eye.
  • A personal history of psoriasis.
  • Having a history of dactylitis in your finger or your toes, aka “sausage digitis”.
  • History of inflammatory arthritis: redness, swelling, and stiffness in any joint
  • Any first degree relative with any spondyloarthritis (SpA)-associated condition? This means mom, dad, siblings, or your children.  SpA-associated conditions include:
    • Axial spondylitis also known as ankylosing spondylitis
    • Psoriasis
    • Psoriatic arthritis
    • Reactive arthritis
    • Crohn’s disease
    • Ulcerative colitis

If you think you could be suffering from autoimmune back pain, please seek your local rheumatologist.  But do not jump to conclusions.  There are many other diseases that can mimic inflammatory back pain that are not autoimmune in nature.

References

Vos T, et al. Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990-2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet. 2016 Oct 8;388(10053):1545-1602.

Sieper J, et al. The Assessment of SpondyloArthritis international Society (ASAS) handbook: a guide to assess spondyloarthritis. Ann Rheum Dis. 2009 Jun; Suppl 2:ii1-44.

http://www.asas-group.org/publications/ASAS-handbook.pdf

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.

When to see a rheumatologist

Signs you may be suffering from autoimmune joint pain

December 5, 2016

 

Joint pain is the price of walking upright.  Pretty much everyone at some point in their lives is going to experience some form of joint pain.  Death, taxes, and joint pain.  But when does joint pain become more than just your typical, “I over did it” joint pain?  When does it become, “I think there is something really wrong” type of joint pain?

I would say that 50% of the consultations I see on a daily basis come from people asking me this exact question.  Is this joint pain caused by an autoimmune disease?

Here are a few typical scenarios.

Scenario #1

Mr. B is a 65 year old man who presents to clinic with bilateral knee pain.  He first noticed the pain a few years ago.  He used to take some over-the-counter ibuprofen when it got real bad and that used to relieve the pain.  Over the past few months he has noticed worsening.  His left knee sometimes swells up.  The ibuprofen isn’t really cutting it anymore.  He tells me that the pain is worse after particularly active days.  When he sits down he feels pretty good but when he stands up, he feels pretty stiff but generally loosens up after a few steps.  He used to work construction and played football in college.

Scenario #2

Mrs. M is a 53 year old woman who comes to the clinic for joint pain.  About 3 months ago, she noticed that she was having a lot of difficulty removing her rings.  She really didn’t pay much attention to it.  She told herself, “I must be eating too much salt”.  Over the course of the next few weeks she began developing pain and swelling involving her knuckles.  The pain is particularly worse first thing in the morning and sometimes wakes her up in the middle of night at times.  She also states, “My hands feel like a claw in the morning”.  The stiffness last well over one hour in the morning and typically, by noon she’s as good as she is going to be for the rest of the day.  She’s tried ibuprofen, naproxen, and acetaminophen but nothing seems to work.  She also remarks that her hot flashes have gone completely out of control recently.

Sound familiar?

Inflammatory vs. Non-inflammatory joint pain

In scenario #1, we have a man presenting with non-inflammatory joint pain.  This is your common wear and tear arthritis or osteoarthritis.  It can involve pretty much any joint you can think of.  What is important to note, is that it tends to progress slowly over time.  The joint pain tends to worsen with increasing activity and it typically responds, although maybe not completely, to over-the-counter anti-inflammatory medications.  Usually there is no joint swelling, but when it comes to the knees, swelling often does occur. A phenomenon called “gelling” can also occur with osteoarthritis.  This occurs when the joint has been in a resting position for a while and then becomes active.  The joint stiffens up or gels, but then loosens up pretty quickly.

In scenario #2, we have a woman presenting with joint pain that develops over the course of 3 months, or what we call a subacute presentation.  She’s experiencing joint swelling involving small joints and it’s associated with prolonged morning stiffness. What I mean by prolonged is over one hour.  Her symptoms also are worse in the morning.  She also experiences constitutional symptoms, i.e., hot flashes.  These are all hallmarks of inflammatory joint pain.  There are MANY different autoimmune diseases that present with inflammatory joint pain and they all have their own particular flavor.  Some like the knuckles, some like the ankles, some like the knees, some have a symmetrical distribution, and some are simply just random.  But they all share these specific key characteristics.

Another important note about autoimmune joint pain.  It doesn’t go away.  This is very important. There are A LOT of different things that can cause a joint to swell, but most of them get better with time.  When it doesn’t, then you have to start wondering.

Here’s a table summarizing what we just learned.  I like tables 🙂

The difference between inflammatory versus non-inflammatory joint pain

Why is this important?

So why should you care?  Well, first of all, walking around with swollen joints isn’t exactly the most pleasant thing in the world.  It turns out that it isn’t exactly healthy for your joints either.  Autoimmune joint disease at times can cause permanent joint damage and it can happen over the course of 3 months.  Don’t believe me?  I dare you to do a quick Google image search.  Type in “rheumatoid arthritis hands”.  Rheumatoid arthritis is the prototypical autoimmune disease that affects the joints.

Disturbing right?

Early identification and prompt treatment is essential to prevent joint damage.  I mean look at those hands!

So if you think that you or someone you know is suffering from autoimmune or inflammatory joint pain, give your local rheumatologist a buzz.

References

Rheumatology Secrets 3rd edition

Diseases and Conditions When to see a rheumatologist

8 important warning signs of scleroderma

November 28, 2016
Scleroderma is an autoimmune disease that can cause fibrosis of skin and internal organs. Early diagnosis is very important. Read on to learn more!

If I had to pick one autoimmune condition, which causes chills to run down one’s spine, I pick scleroderma.  The medical name for this condition is systemic sclerosis.  In a nutshell, systemic sclerosis is an autoimmune disease which causes inflammation in small blood vessels, which eventually can cause hardening of the skin and other major organs.

I quick Google search of scleroderma will inevitably present you with a horror show of images and stories from sufferers of this condition.

Yes, scleroderma is a terrible disease, but it’s also a terribly heterogeneous disease meaning that no two cases of scleroderma are the same.  There are mild cases and there are life-threatening cases.

Identification of early disease is probably one of the most important determinants of prognosis.  Well actually, it’s a little more complicated than that, but it is the one aspect that you can control:  The knowledge to know when to ask.

Types of Scleroderma

This is how rheumatologists breakdown scleroderma:

Classification of scleroderma

 

Basically, systemic sclerosis is when the disease affects the skin and internal organs and localized scleroderma is when it only involves the skin.  A lot of rheumatologists, included yours truly, believe that localized scleroderma and systemic sclerosis are actually two completely different diseases.

I’m going to concentrate on systemic sclerosis going forward.

Now we’re left with scleroderma sine scleroderma, limited systemic sclerosis, and diffuse systemic sclerosis.  Scleroderma sine scleroderma is systemic sclerosis involving the organs but not involving the skin.  This is super rare.

People that have limited systemic sclerosis have hardening of the skin that does not go past the elbows or knees.  They also tend to NOT have kidney involvement or inflammation of the lungs.  The disease course usually is insidious and sometimes is very difficult to detect, particularly during a 15 minute doctor’s appointment.

The last type is called diffuse systemic sclerosis.  It’s important to detect REALLY fast because it can progress quickly, because it can be deadly, and people simply do better when they’re treated early. People suffering from this type of scleroderma can have whole body hardening and are more prone to develop lung inflammation and kidney involvement.

Raynaud’s phenomenon

Almost everyone with any form of systemic sclerosis has something called Raynaud’s and esophageal reflux.  They also tend to appear early during the disease process, so these two symptoms are important to watch out for.

They say a picture is worth a thousand words.  Here’s an example of Raynaud’s… not the most dramatic example but that’s kind of the point.

This is a nice example of Raynaud's phenomenon. Sometimes the finger can be white, like the pinky, and sometimes it can look dusky

 

Raynaud’s can involve your fingers and your toes.  Exposure to cold can cause it as well as a rapid change in temperature, and stress.  Raynaud’s is very common in women and for the most part is annoying but nothing serious.  The picture above is actually my hand.  This has been going on since my teens.  Let me tell you, I grew up in Canada.  Shoveling snow was kind of brutal.

About 15% of women have primary Raynaud’s (i.e., Raynaud’s without any underlying autoimmune condition).  Primary Raynaud’s typically first appears during one’s teens or early adulthood.  People with scleroderma tend to develop it later in life and  the intensity is A LOT more severe.  Sometimes the skin can become so ischemic (poor circulation) that it can cause an ulcer.  I have a lot of patients whom I’ve diagnosed with systemic sclerosis that told me that they thought that they had purple fingers because they were getting old…  This is NOT part of the normal aging process.

  1. Onset of Raynaud’s at a later stage in life.
  2. Raynaud’s complicated by ulcers.

Heartburn

Another symptom found in almost all people suffering from systemic sclerosis is heartburn.  This can present as chest pain, burning pain up your esophagus, nighttime cough, an acid taste in your mouth in the morning.  Sometimes people may have difficulty swallowing food and water.  Now, a lot of people have heartburn.  Just because you have heartburn, does not mean you have scleroderma.  You have to look at the entire picture.

  1. Heartburn
  2. Difficulty swallowing

Other common symptoms

Here is a list of other symptoms to watch out for:

  1. Puffy fingers, VERY high yield symptom
  2. Red spots all over your face, chest, and/or palms
  3. Hardening of the skin
  4. Shortness of breath with exercise

 

Conclusion

There are a lot more symptoms that I haven’t gone over.  But sometimes less is more.  If you can remember these 8 warning signs, you have a really good chance of detecting early disease on your own, and maybe even save your life or the life of a loved one.  Having more than one of these symptoms should prompt you to consult a rheumatologist for further evaluation and testing.

For more information, I highly urge you to visit the Scleroderma Foundation website. They provide useful information about scleroderma for both the newly diagnosed and those that have had the disease for a long time.  They’re also involved research and patient advocacy.  Needless to say, I highly recommend them!

If you want to learn more about Raynaud’s phenomenon and ways how to treat it, read on.

References

Rheumatology Secrets 3rd edition

van den Hoogen F, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55.

Minier T, et al. Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicenter study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis. 2014 Dec;73(12):2087-93.

Scleroderma Foundation: http://www.scleroderma.org/site/PageServer#.WTC56WjytPY

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