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.

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

Dry mouth symptoms

The following are some common symptoms of dry mouth.

Difficulty swallowing dry foods
Inability to talk continuously
Change in taste
Burning sensation
Large dentist bill! – Cavities, cracked teeth, loose fillings
Problems with your dentures
Worsening heartburn
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.

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Diseases and Conditions

Does acupuncture help neuropathy?

February 8, 2017

Does acupuncture help neuropathy? Good question. But before trying to answer that question, why does a rheumatologist care about neuropathy anyways? Isn’t neuropathy more of a neurology thing? The answer to that question is yes and no. If you’ve read a few RheumDoctor articles, you’ve probably realized that rheumatology is not just about joints. It’s about the immune system, and the immune system affects ALL organs including the nervous system. That being said, innumerable autoimmune diseases affect nerves including: lupus, rheumatoid arthritis, Sjogren’s syndrome, vasculitis, ankylosing spondylitis/axial spondylitis, scleroderma, myositis, sarcoidosis, etc. I could go on, but I think you get the point?

So what is neuropathy anyway?

In medical terms, neuropathy, more specifically peripheral neuropathy, occurs when peripheral nerves are malfunctioning for any number of reasons. Peripheral nerves are those that DO NOT involve the brain or spinal cord. When it involves multiple nerves, we call it polyneuropathy. When it involves one nerve, we call it mononeuropathy.

Symptoms that are suggestive of neuropathy include:

Numbness
Tingling
Burning
Pain out of proportion to stimulation (e.g., touching intact skin should not cause pain)
Weakness
Cramping
Itching
Temperature dysregulation

Some people describe neuropathy as invisible pain. Someone can be in terrible agonizing pain, but physically they look perfectly normal.

How do you diagnosis neuropathy?

Peripheral neuropathy can be diagnosed clinically, with nerve conduction tests, and with a special type of skin biopsy called an epidermal nerve fiber density test. Don’t get me wrong, I’m all about history and physical exam, but when it comes to diagnostics, I like to have some hard data supporting my clinical diagnosis. This is why a suspicion of peripheral neuropathy typically should be supported by either a nerve conduction test and/or a skin biopsy.

Nerve conduction tests are pretty much the go to test when it comes to neuropathy. The doctor measures the conduction of electrical impulses that go down the nerves. This type of test is good to diagnose problems with large myelinated nerves. Not so much for small, unmyelinated nerve fibers like the ones found on the skin. When the doctor suspects the problem is coming from small fibers, a skin biopsy is the way to go. In very special and unique cases, an actual biopsy of a large nerve may be required. In the world of rheumatology, this can happen if there is a suspicion for systemic vasculitis. This includes really rare diseases like Wegener’s (although we don’t it that anymore)¹, Churg-Strauss (we don’t call it that anymore either)², and microscopic polyangiitis.

What causes neuropathy?

The most common diseases in general include diabetes, chemotherapy, carpal tunnel syndrome, Lyme disease, alcohol abuse, vitamin deficiencies, low thyroid, HIV, and hepatitis C infection. At that point, you start getting into the weird and rare diseases. This is where rheumatic autoimmune diseases feature. Unfortunately, despite exhaustive workups, in about one-third of cases, a specific cause is never found. This is called idiopathic neuropathy.

Treatment of neuropathy

First, treat the underlying disease. Unfortunately, in most situations this is not enough. Medications that are often used include: gabapentin, pregabalin, duloxetine, amitriptyline, topiramate, and carbamazepine. In extreme situations, infusions with immunoglobulin are used particularly with demyelinating autoimmune conditions. When all else has failed, and I mean, ALL else has failed narcotic medications for breakthrough pain be necessary. Every person and situation is unique, so it’s important to work closely with your physician to figure out the best plan of action.

It’s important to note that all these medications have potentially serious or undesirable side effects. Increasingly, people are searching for alternatives treatments. Long a staple of Chinese traditional medicine, there’s recently been an increased interest in using acupuncture to treat neuropathy.

But is there any evidence and is it safe?

Acupuncture for the treatment of peripheral neuropathy: a systematic review and meta-analysis

Researchers recently sought to determine whether acupuncture was safe and efficacious for the treatment of peripheral neuropathy. They searched multiple medical databases including Medline, AMED, Cochrane, Scopus, CINAHL, and clinitrials.gov looking for studies that matched stringent entry criteria. About one thousand studies were identified, but only 13 made the cut.

They selected randomized controlled trials studied acupuncture for neuropathy caused by diabetes, Bell’s palsy, carpal tunnel syndrome, HIV, and idiopathic conditions. What they found was that acupuncture generally was effective for diabetic neuropathy, Bell’s palsy, and carpal tunnel syndrome. In these cases, they even found improvement in nerve conduction study parameters in both sensory and motor nerves. More data was necessary to determine whether acupuncture was effective in cases of HIV-associated neuropathy but there was a positive trend. With regards to idiopathic neuropathy, there was insufficient evidence. Then again, there were hardly any trials that looked into this type of neuropathy.

Does this mean that everyone with diabetic neuropathy should start acupuncture ASAP? Hold your horses. The researchers also noted that there was A LOT of variability between studies and A LOT of bias. Evidence-based medicine is all about comparing oranges with oranges and apples with apples in an unbiased way. If these conditions are not met, then it really becomes difficult to determine what caused what.

The following are some of the problems that the researchers identified.

Methodological problems with standardization

Point selection
Number of needles used
Needle retention time
Needling depth
Needle manipulation
Use of moxibustion
Use of electroacupuncture vs. manual acupuncture

Methodological problems with sample size

Only two of the studies stated sample size
Many trials were likely underpowered (You cannot make generalizations from a small group of people)

Methodological problems with improper control and blinding

In many cases researchers were not blinded
In many cases participants were not blinded

Methodological problems with outcome measures

Most trials assessed improvement with subjective measures of improvement instead of hard evidence.
Only 4 out of 13 trials actually did nerve conduction tests before and after, to see whether anything changed.

On the up side, acupuncture was found to be safe. Serious side effects only occurred in people suffering from HIV-associated neuropathy. Otherwise, acupuncture seems pretty safe.

Conclusion

Basically, the meta-analysis demonstrated that acupuncture is generally safe and that it may help in people suffering from diabetic neuropathy, Bell’s palsy, and carpal tunnel syndrome. Autoimmune disease-associated neuropathy is a big unknown. However, these results should be taken with a grain of salt. Although the meta-analysis in itself used good methodology, the researchers were not working with the studies with the best methodological practices in the world.

This brings me back to my first question. Does acupuncture help neuropathy? My answer to that questions is as follows.

As always, think for yourself! You now have the tools to make an educated, unbiased, objective, informed decision regarding health.

References

UpToDate

Dimitrova A, Murchison C, Oken B. Acupuncture for the treatment of peripheral neuropathy: a systematic review and meta-analysis. J Altern Complement Med. 2017 Jan 23. doi: 10.1089/acm.2016.0155.

Notes

Wegener’s = granulomatosis with polyangiitis. And they wonder why people still call it Wegener’s?

Churg-Strauss = eosinophilic granulomatosis with polyangiitis

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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, 3^rd 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.

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Diseases and Conditions Featured

What is a positive ANA and what does it mean?

December 12, 2016

What does it mean to have a positive ANA also known as an antinuclear antibody? This is a loaded question and the answer is complex. The answer is usually quite personalized to the person and their symptoms. The answer also usually entails follow-up bloodwork and evaluation by a rheumatologist. But in simple terms, an ANA is an antibody directed towards 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.

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 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 a lot more. This is a higher level.

So how much dilution is enough to consider an ANA as positive? 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’s 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.

Positive ANA patterns

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 possibly indicate the presence of specific nuclear antibodies. For example, the presence of a speckled positive ANA indicates the presence of these specific autoantibodies, 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 experience.

I’m not going to go more into details about 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 a positive 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 you should be tested.

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
5 – 25% of healthy people with a family member suffering from lupus have a positive ANA
Up to 70% of people aged above 70 years have a positive ANA

To complicate things even more, someone who is about to have and autoimmune disease can have a positive ANA… UP TO 10 YEARS before they actually develop the disease. Cancer and infections can also cause someone to have a positive ANA. It can even be positive when people are taking certain medications. Not terribly helpful right?

Bad example

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

Does it mean anything?
Is the person one of those healthy people that has a positive ANA?
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 that person did not have any symptoms. Because so many people who are completely healthy have an ANA, the test should only be run if a person has a symptom or better yet, multiple symptoms that potentially indicate the presence of an autoimmune disease like lupus, Sjögren’s syndrome, systemic sclerosis, mixed connective tissue disease, etc. In that situation, it is helping rule in or rule out certain diagnoses.

Good example

If you’ve read my earlier post, 8 important warning signs of scleroderma, you’ll remember that Raynaud’s phenomenon is an important red flag for scleroderma. The majority of people suffering from Raynaud’s have no underlying autoimmune disease but 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 has a high risk of developing an autoimmune disease like lupus, scleroderma or Sjogren’s syndrome.

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 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 have 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 positive ANA. If you’ve tested positive for an ANA and have more questions, I highly urge you to speak with your physician or local rheumatologist. And remember, doctors treat people not numbers.

References

Rheumatology Secrets 3^rd edition

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.

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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:

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.

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.

Onset of Raynaud’s at a later stage in life.
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.

Heartburn
Difficulty swallowing

Other common symptoms

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

Puffy fingers, VERY high yield symptom
Red spots all over your face, chest, and/or palms
Hardening of the skin
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 3^rd 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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