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Diseases and Conditions

Diseases and Conditions When to see a rheumatologist

I have a positive HLA-B27 what does it mean?

June 5, 2018
I have a positive HLA-B27 what does it mean?

During the course of a workup for possible autoimmune arthritis or autoimmune back disease, your rheumatologist may order something called a HLA-B27 test.  So what does a positive HLA-B27 mean?

HLA-B27 antigen

HLA-B27 is a genetic test. The majority of people who have a positive HLA-B27 are perfectly healthy. HOWEVER, having a positive HLA-B7 can put you at increased risk of developing what we call spondyloarthritis-associated diseases. This is a family of autoimmune diseases.

Spondyloarthritis-associated diseases

  • Ankylosing spondylitis (AS), now called axial spondylitis
  • Peripheral spondyloarthritis
  • Reactive arthritis
  • Psoriasis
  • Psoriatic arthritis
  • Uveitis
  • Crohn’s disease
  • Ulcerative colitis

I have a positive HLA-B27, what does it mean?

The answer to this question highly depends on your situation.  As I mentioned earlier, many people who have a positive HLA-B27 are perfectly healthy.  More specifically, the majority of these people do NOT have an autoimmune disease.  Here are a few stats to put things into perspective.

  • About 6 to 9 % of Caucasians and 3 % of African-Americans have a positive HLA-B27.
  • However, having a positive HLA-B27 increases a person’s risk of ankylosing spondylitis by 50 to 100 times.
  • But only 2 % of people who have a positive HLA-B27 go on to develop ankylosing spondylitis.
  • About 50% of people with psoriatic arthritis that involves the spine have a positive HLA-B27.
  • 60% of people diagnosed with reactive arthritis have a positive HLA-B27. These people tend to have more severe symptoms, non-joint symptoms (e.g., uveitis), and it tends to last longer.
  • In the West, 50% of cases of anterior uveitis is associated with a positive HLA-B27 and about half of these people will develop spondyloarthritis.

I was diagnosed with AS, should I have my kids tested?

Technically not unless a family member is experiencing symptoms suggestive of ankylosing spondylitis, psoriatic arthritis, inflammatory bowel disease, or any other spondyloarthritis-associated disease.  Remember, only 2% of people with a positive HLA-B27 go on to develop ankylosing spondylitis, which is the main autoimmune disease associated with this test.

However, people who have a positive HLA-B27 AND have a first-degree relative with ankylosing spondylitis have a 15% to 20% risk of developing the disease at some point during their lifetime.

I guess the answer whether to or whether not to test your kids really is a matter of choice.  My opinion is, if it’s going to happen it’s going to happen. All we really can do is try to make healthy life choices like eating clean, staying active, reduce stress, and making sure we sleep enough.  Remember the Blue Zones Principles?

For some people, knowing they tested positive for HLA-B27 may increase their stress level and for others not knowing whether they test positive or not may be more harmful. Whatever decision you make is highly personal.

In summary

Why having a HLA-B27 increases risk of having ankylosing spondylitis as well as a host of different autoimmune diseases is still uncertain: arthrogenic peptide hypothesis, molecular mimicry, free heavy chain hypothesis, and unfolded protein hypothesis.  These are very complex concepts, which I will hold off on discussing further.

If you want to learn more about HLA-B27 and spondyloarthritis, I invite you to read the following articles: What is autoimmune back pain? Guide to living with psoriatic arthritis: part 1, and What is autoimmune joint pain?

If you think you may be suffering from a spondyloarthritis-associated condition, I encourage you to get in touch with your local rheumatologist.  Here is a link to the American College of Rheumatology physician directory.

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.

References

Rheumatology Secrets, 3rd edition

Diseases and Conditions When to see a rheumatologist

Signs and symptoms of polymyalgia rheumatica

May 22, 2018
Signs and symptoms of polymyalgia rheumatica

Polymyalgia rheumatica is a systemic inflammatory disease that almost exclusively affects people aged 55 and above.  How this disease occurs and why it more-or-less exclusively affects the elderly is poorly understood.  Like most autoimmune diseases, it’s thought that something in the environment be it a virus, bacteria, or in the case of polymyalgia rheumatica seasonal variation, triggers autoimmunity in a genetically susceptible person.

The hallmark symptoms of polymyalgia rheumatica include severe pain and stiffness involving the shoulder and or pelvic muscles.

However, it’s very important to understand that polymyalgia rheumatica is a diagnosis of exclusion.  It’s important to rule out other conditions before making this diagnosis.

Polymyalgia rheumatica statistics

Polymyalgia rheumatica is the second most common autoimmune condition.  It affects 2.4% of women and 1.7% of men.  It can affect people as young as 50 years, but mainly affects people aged 60 years and above. Finally, Caucasians are mainly affected.  It very rarely affects African Americans, Asians, or people of Hispanic descent.

What are the signs and symptoms of polymyalgia rheumatica?

People suffering from polymyalgia rheumatica typically describe a slow and insidious process of worsening stiffness and pain involving their shoulders and their pelvis.  Sometimes the process is quick but typically it progresses over 3 months.  Usually there is a dramatic loss of function.  This includes difficulty getting up from a seated position, difficulty getting out of bed, difficulty dressing.

“I feel 20 years older than I am”.

What are some other symptoms of polymyalgia rheumatica?

Some other symptoms include fevers, chills, drenching night sweats, and unintentional weight loss. Importantly, giant cell arteritis, a type of vasculitis, can occur in about 15% of people suffering from polymyalgia rheumatica.

People suffering from giant cell arteritis typically report very similar symptoms, as well as the following:

  • Recent onset headache
  • A cramping sensation while chewing
  • Change in vision
  • A recent change in hearing, taste, as well as smell
  • Tenderness of the scalp

I’ll go into this later, but people suffering with giant cell arteritis usually do NOT improve with 20 mg of prednisone, whereas, people with pure polymyalgia rheumatic do.

Do people with polymyalgia rheumatica get arthritis?

The simple answer is yes they can.  About 30% of people with polymyalgia rheumatica develop inflammatory arthritis that can look a lot like rheumatoid arthritis.  However, unlike rheumatoid arthritis, this type of arthritis does not cause permanent damage.

How do you differentiate polymyalgia rheumatica from rheumatoid arthritis?

This can get very tricky and sometimes can take months of observation to finally figure it out.  However, here are some distinguishing features that can help:

  • Absence of rheumatoid factor and other rheumatoid arthritis antibodies
  • Lack of involvement of small joints
  • Lack of joint damage

What are some differential diagnoses in polymyalgia rheumatica?

Diagnosis Features
Fibromyalgia Tender points and normal inflammation
Underactive thyroid (hypothyroidism) Normal thyroid tests, normal inflammation
Depression Normal inflammation
Osteoarthritis, rotator cuff, frozen shoulder Abnormal x-rays and MRI, normal inflammation
Myositis Mainly weakness, elevated muscle enzymes, abnormal nerve conduction test
Infection Clinical suspicion and positive cultures
Cancer Clinical suspicion with positive workup
Rheumatoid arthritis Positive rheumatoid factor, small joints involved
Spondyloarthritis History of psoriasis or inflammatory bowel disease[1]

How is polymyalgia rheumatica diagnosed?

The diagnosis of polymyalgia rheumatica is predominantly clinical.  It’s very important to exclude other potential diagnoses as listed above.  Levels of inflammation are typically very high but can be normal in select cases.

The American College of Rheumatology and EULAR have established criteria, but these are not the greatest in the world.  They have a sensitivity of 68% and specificity of 78%.  Finally, sometimes ultrasound studies and a PET scan can help as well.

How is polymyalgia rheumatica treated?

Steroids.  People suffering from polymyalgia rheumatica often describe a dramatic resolution or improvement of the symptoms on 15 to 20 mg of prednisone a day.  By dramatic I mean it typically take less than 24 hours for symptoms to resolve.  In about 25% of cases, the response will not be dramatic and will take a few more days or a higher dose of prednisone.

Unlike other rheumatic conditions, prednisone must be tapered very slowly in polymyalgia rheumatica.  It’s not uncommon for a person to be on steroids for over two years.  At times, we need to taper by 1 mg every month or two!

My best friend, my worst enemy

Prednisone is both your best friend and worst enemy.  Unfortunately, it remains the mainstay of therapy.  While on high doses of steroids for a prolonged period of time, it’s very important to remain active and pay attention to possible side effects such as osteoporosis, diabetes, heartburn/stomach ulcers, and infection.  Not to mention an ever expanding waist line.

Regular weight-bearing exercise, vitamin D supplementation, as well as keeping up-to-date with vaccines (flu shot, pneumonia shot, shingles shot) are essential.

Conclusion

If you think you or one of your loved ones suffers from polymyalgia rheumatica, I encourage you to make an appointment with your local rheumatologist.

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.

References

Rheumatology Secrets, 3rd edition

UpToDate

[1] Adapted from Rheumatology Secrets, 3rd edition

Diseases and Conditions

Shingrix: The new shingles shot

May 8, 2018
Shingrix: The new shingles shot

Benjamin Bergen, Doctor of Pharmacy Candidate Class of 2018, Albany College of Pharmacy and Health Sciences

In Fall 2017, a new inactivated recombinant shingles vaccine, Shingrix, came to the market to help people better protect themselves against the herpes zoster (shingles) virus.1

What is shingles?

Shingles is a painful rash that usually develops on only one side of the body, caused by the chickenpox virus reactivating in the body years after initial infection. Since over 99% of people over 40 have had chickenpox, vaccination is recommended regardless of the person remembering if they had it. Blisters appear that scab over in 7 to 10 days and clear up within 2 to 4 weeks. Some people with shingles have a long-lasting burning sensation that can last for months or years after the rash goes away, called postherpetic neuralgia (PNH). The risk to get both shingles and PHN increases with age.1

Zostavax: The “old” shingles shot

Previously, the only available product for protection against shingles was Zostavax, Merck’s live vaccine which was approved in 2006. Zostavax was FDA approved for people over 50 years of age, but the Centers for Disease Control and Prevention (CDC) only recommended it for people 60 years of age and older. Zostavax should not be given to any person that has:1,2

  • An anaphylactic allergy to gelatin, neomycin, or any other component of the vaccine
  • Immunosuppression for any reason, including:
    • Primary or acquired immunodeficiency states
    • AIDs or other clinical manifestations of HIV
    • Leukemia or lymphoma, or any other malignant neoplasms affecting the bone marrow or lymphatic system
    • Receiving immunosuppressive therapy
      • Cancer treatment such as radiation or chemotherapy
      • Long-term steroids (ie: prednisone)
      • Biologic therapy such as adalimumab (Humira) or etanercept (Enbrel)
    • Women should not become pregnant for at least 4 weeks after receiving the vaccine

Shingrix: The new shingles shot

The CDC now recommends Shingrix over Zostavax for the prevention of shingles and related complications for all immunocompetent adults aged 50 and older:1,3

  • Regardless of having a history of shingles
  • Regardless of prior Zostavax vaccination. There should be at least 8 weeks between the vaccinations.
  • With chronic medical conditions such as diabetes, chronic kidney disease, or rheumatoid arthritis
  • At the same time as the flu or pneumonia vaccines

There are exceptions

The CDC still recommends Zostavax in certain cases, such as if Shingrix is unavailable or if a person is allergic to Shingrix.1 Shingrix has been shown to be much more effective than Zostavax for preventing shingles in different age groups, illustrated in the following table:2,3

Age Group Zostavax Efficacy Shingrix Efficacy
50-59 70% 96%
60-69 64% 97%
70-79 41% 91%
>80 18% 91%

 

Shingrix should not be given to any person that:1,3

  • Has a history of a severe allergies reaction to any component of the vaccine or after a previous dose of Shingrix.
  • Tested negative for immunity to varicella zoster virus (the chickenpox vaccine should then be given)
  • Currently has shingles
  • Is currently pregnant.

Possible side effects

The most common side effect with Shingrix is pain at the injection site. This is more common in younger people. Other side effects include muscle pain, fatigue, headache, shivering, fever, and gastrointestinal symptoms.

Head-to-head comparison

Shingrix is covered by Medicare Part D, the prescription drug plan, and may have a copay. A full comparison of the two vaccines is shown below:

Zostavax2 Shingrix3
Live attenuated vaccine Recombinant Inactivated Vaccine
Cannot be given to immunosuppressed patients Has not been studied in immunosuppressed patients
No longer CDC recommended CDC recommended for patients aged 50 or older
Single vaccination Two vaccinations, 2-6 months apart
Patients over 60: 39% decrease in postherpetic neuralgia Patients over 70: 88% decrease in postherpetic neuralgia

Call to action

If you are interested in learning more about vaccines and autoimmune disease, I encourage you to read our flu vaccine and pneumonia vaccine articles.

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.

References:

  1. Herpes Zoster Shingrix Vaccine Recommendations. The Centers for Disease Control and Prevention. Cited 1 May 2018.
  2. Zostavax Prescribing Information. Food and Drug Administration 2006. Revised 2018.
  3. Shingrix Prescribing Information. Food and Drug Administration 2017. Revised Oct 2017.
Diseases and Conditions When to see a rheumatologist

8 Warning signs of psoriatic arthritis

April 10, 2018

Most people think psoriasis is an autoimmune disease that only affects the skin, but did you know that about 26% of people also have psoriatic arthritis?  Did you know that psoriatic arthritis is more common than rheumatoid arthritis?[1]  What are the signs of psoriatic arthritis?

8 Warning signs of psoriatic arthritis

1.   Having nail psoriasis

A recent Japanese study tried to find risk factors that predispose people with psoriasis to develop psoriatic arthritis.  First, they found that about 17% of people with psoriasis also had psoriatic arthritis.   Furthermore, they found that people who had psoriasis involving their nails had a higher chance of having psoriatic arthritis: 29% (PsO) versus 62% (PsA).  In conclusion, they found that people who had high uric acid levels also had a higher risk of having psoriatic arthritis 9% (PsO) versus 22% (PsA).[2]

How does nail psoriasis look like?

Nail psoriasis can sometimes mimic nail fungus.  It can also make pits in the nail and can cause nails to break very easily.

Follow the link for examples.

2.   Having autoimmune joint pain

Autoimmune joint pain typically causes swelling and warmth of the joints.  People often describe stiffness that lasts more than one hour.  Symptoms are worse in the morning.  Furthermore, psoriatic arthritis can affect almost any joint: knuckles, toes, wrists, ankles, knees, etc.

3.   Inflammation involving the tips of your fingers

I’m referring to the tips of the fingers, also called the distal interphalangeal joints.  Osteoarthritis (i.e., wear and tear arthritis) in the hands can sometimes cause similar symptoms.  Typically, the symptoms in psoriatic arthritis happen more quickly and typically there is more swelling and redness.

4.   “Sausage fingers”

This happens when the tendons and ligaments that supply fingers get inflamed.  This can happen in a variety of different diseases such as ankylosing spondylitis, reactive arthritis, inflammatory bowel disease, infection, sickle cell anemia, sarcoidosis, and gout to name a few.  Follow the link to look at some examples.

5.   Having inflammation of tendons and ligaments

Enthesitis means inflammation of connective tissue that attaches to bones.  These include tendons, ligaments, and bursae.  Most cases of enthesitis are due to injury or overuse.  Think of a marathon runner with Achilles tendinitis or a tennis player with tennis elbow.  In psoriatic arthritis, the immune system attacks these connection points.  So you can have someone who leads a fairly sedentary life who suddenly develops Achilles tendinitis on both feet, runner’s knee, and plantar fasciitis happening all at once, for no good reason.

6.   Autoimmune back pain

Inflammation involving the back causes symptoms that are very different from your usual mechanical back pain.  Here are some key features:

  • Back pain present for more than three months.
  • Pain improves with exercise.
  • Pain improves with anti-inflammatory medications like naproxen or ibuprofen.
  • Rest usually worsens the pain.
  • Back pain that wakes you up during the second half of the night.
  • Pain and prolonged stiffness in the morning, typically lasting more than an hour.
  • Alternating deep buttock pain.

7.   History of uveitis

Uveitis is a general term used to describe a group of diseases that cause inflammation in the eye: uvea, lens, retina, optic nerve, and the vitreous.  Depending on where the inflammation is happening, your ophthalmologist may describe it as anterior uveitis, intermediate uveitis, posterior uveitis, or panuveitis.  As a result, people can experience blurry vision, eye pain, redness, sensitivity to light, and sometimes headaches.

Uveitis is associated with many diseases including psoriasis and psoriatic arthritis.  Sometimes uveitis is the first manifestation of psoriatic arthritis.  This is why I’ve included this topic here, even though technically it isn’t arthritis.  It’s important to keep this in the back of your mind.[3]  While uveitis is one of the signs of psoriatic arthritis, psoriatic arthritis is not the most common cause of uveitis.

8.   Having a first degree family member with psoriasis or psoriatic arthritis

This means mom, dad, siblings, and children.  Furthermore, other diseases also increase risk.  These include Crohn’s disease, ulcerative colitis, ankylosing spondylitis, reactive arthritis, and uveitis.

Call to action

In conclusion, if you think you may have psoriatic arthritis, I encourage you to contact a rheumatologist to get tested.  Time is of the essence.  Click on “FIND A RHEUMATOLOGIST” above to search the American College of Rheumatology database to find a rheumatologist near you.

Want to learn more?  The Arthritis Foundation and the Arthritis Society are also great starting points.

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.

References

[1] Rheumatology Secrets, 3rd edition

[2] Tsuruta N, Imaguku S, Narisawa Y.  Hyperuricemia is an independent risk factor for psoriatic arthritis in psoriatic patients. J Dermatol. 2017 Jul 10. doi: 10.1111/1346-8138.13968. [Epub ahead of print]

[3] https://nei.nih.gov/health/uveitis/uveitis

Diseases and Conditions When to see a rheumatologist

10 Important warning signs of systemic lupus

February 27, 2018
10 Important warning signs of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease that presents in many ways.  The disease is characterized by the production of autoantibodies that deposit onto tissue and fix complement on almost any organ which causes systemic inflammation.  Typically it affects women (9:1) aged between 15 and 45 years and it tends to affect people of African-American, Asian, and Hispanic descent more so than Caucasians: 3 to 4 times higher.[1]

This is a very complex condition and we’re still trying to understand the underlying cause and trying to find effective treatments.  Even diagnosis remains challenging at times.  Today I’d like to go over 10 important warning signs of systemic lupus.

Criteria for the classification of SLE

The following are the criteria for the diagnosis of SLE. To fulfill criteria you need to have at least 4 criteria with at least one coming from the clinical section and one coming from the immunological section.  An exception to the rule is if someone has a kidney biopsy that shows lupus nephritis in the presence of a positive ANA or other lupus-related autoantibody.

It’s important to note that there are exceptions to the rule.  These criteria are meant to be used in research and we all know that real life sometimes doesn’t fit the mold!  Also, as researchers make more discoveries, classification systems change.  The Autoimmunity Blog has a great pdf showing how these criteria evolved with time.

Systemic Lupus International Collaborating Clinics Classification Criteria for SLE (2012)

Criterion Definition
Clinical Criteria
1.      ACLE (acute cutaneous lupus erythematosus) Malar rash, bullous lupus, TEN variant, maculopapular, photosensitive, subacute cutaneous lupus
2.      CCLE (chronic cutaneous lupus erythematosus) Classic discoid, hypertrophic, lupus panniculitis/profundus, mucosal, lupus erythematosus tumidus, chilblains lupus, discoid/lichen panus overlap
3.      Alopecia Non-scarring, diffuse hair thinning or visible broken hairs
4.      Oral ulcers Oral (palate, buccal, tongue) or nasal ulceration
5.      Synovitis Arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion and morning stiffness > 30 minutes
6.      Serositis Pleuritis: convincing history of pleuritic chest pain for >1 days or pleural rub or evidence of pleural effusion, or Pericarditis documented by EKG or rub, or evidence of a pericardial effusion
7.      Renal disorder Persistent protein ≥ 0.5 grams/day or red blood cell casts
8.      Neurological disorder Seizures, psychosis, myelitis, mononeuritis multiplex, peripheral or cranial neuropathy, acute confusional state
9.      Hemolytic anemia Direct Coombs positive
10.   Leukopenia Leukopenia < 4000/mm³ at least once or lymphopenia < 1000/mm³ at least once
11.   Thrombocytopenia Platelets < 100 000/ mm³
Immunological Criteria
1.      Positive ANA Level above laboratory reference
2.      Anti-dsDNA Level above laboratory reference range (or >2-fold ELISA reference range)
3.      Anti-Sm Presence of antibody to Sm nuclear antigen
4.      Antiphospholipid antibody

–        Positive lupus anticoagulant

–        False positive for rapid plasma regain

–        Medium titer or high titer anticardiolipin antibody level

–        Positive anti-β-glycoprotein I

5.      Low complements Low C3, low C4, or low CH50
6.      Direct Coombs test In the absence of hemolytic anemia

RheumDoctor’s simplified lupus criteria

As you can see these criteria are somewhat complicated and use A LOT of “medicalese”.  Let’s try simplifying things!  Basically, the criteria include clinical features and blood tests that show abnormal changes with the immune system.  The clinical criteria do include some blood tests like white cells, blood cells, platelets, and urine, but these are NOT tests that specifically show problems with the immune system.  They are abnormal BECAUSE the immune system is affecting them.

10 Important warning signs of systemic lupus erythematosus

  1. Rash
  2. Hair loss
  3. Oral and/or nose ulcers
  4. Autoimmune joint pain
  5. Chest pain
  6. Kidney problems
  7. Neurologic or psychiatry changes
  8. Anemia
  9. Frequent infections
  10. Easy bruising or bleeding

Other symptoms that are not included in the criteria include profound fatigue, fevers, unintentional weight loss, and Raynaud’s phenomenon.

Rash

The first thing that pops up in most people’s mind when they think about lupus is the famous “butterfly” rash, more specifically the malar rash.  This type of rash is one of many ways the disease can inflame the skin.  DermNet New Zealand has a great selection of examples.  People with lupus also tend to get a rash when their skin is exposed to the sun (photosensitivity).  Sometimes exposure to the sun can cause a systemic flare, e.g., cause joint pain, swelling, and fatigue.

Hair loss

Unless the hair loss is caused by a rash, e.g., discoid lupus, hair loss tends not to scar.  It can happen in patches or simply be generalized.

Oral and/or nose ulcers

Almost everyone gets a canker sore once in a while.  People with lupus often get multiple ulcers over and over again.  They usually DON’T hurt.

Autoimmune joint pain

Autoimmune joint pain or inflammatory arthritis looks very similar to the joint pain that people have with rheumatoid arthritis.  However, joint inflammation caused by lupus is NON-EROSIVE, meaning that people do not get permanent joint deformities.  Like rheumatoid arthritis, joints get swollen, tender, and they tend to stay stiff for at least 30 minutes to an hour in the morning.

Chest pain

Lupus can cause pleuritic and pericarditis. Basically, the lining of the lungs (pleuritic) or the lining of the heart (pericarditis) get inflamed. This can cause sharp chest pain and sometimes can cause fluid to accumulate around the lungs or heart.  Usually this type of chest pain worsens when you take a deep breath and in the case of pericarditis, improves when you lean forward.

If you are experiencing chest pain, don’t mess around, go to the emergency room.  People with lupus have a higher risk of heart disease.  In fact, cardiovascular disease is the leading cause of death in people with lupus.  So you need to rule out a heart attack.

Kidney problems

Lupus can cause inflammation in the kidneys (lupus nephritis).  There are 6 types.  It’s important to know what type we’re dealing with when making treatment decisions.  Lupus nephritis doesn’t cause “kidney pain”.  The symptoms of kidney malfunction include:

  • Weight gain (water weight)
  • Uncontrolled high blood pressure
  • Dark urine
  • Frothy or foamy urine
  • Leg swelling
  • The need to urinate during the night

Neurologic and/or psychiatric changes

These are probably the most difficult to diagnose features of lupus.  They vary widely are terribly non-specific.  People with lupus who present with neurological symptoms can present with a stroke, limb weakness, small fiber neuropathy, seizures, and even psychiatric changes like schizophrenia or major depression.  Close collaboration with a rheumatologist, neurologist, and psychiatrist is often required.

Anemia

Lupus can cause many different types of anemia: anemia of chronic disease, hemolytic anemia, kidney failure, etc.  Some symptoms of anemia include:

  • Loss of energy
  • Rapid heart beat
  • Shortness of breath
  • Headache
  • Difficulty concentrating
  • Dizziness
  • Pale skin
  • Leg cramps
  • Insomnia
  • Hemolytic anemia can also cause yellowing the eyes/sclera (jaundice)

Frequent infections

Many people with lupus get frequent infections.  For the most part this is caused by medications used to treat lupus but sometimes it can be the main culprit.  Lupus can cause white blood cells and specialized white cells called lymphocytes to decrease in number.  These cells are part of the immune system and help your body fight off infection.  If they are critically low, you can get frequent infections.

Easy bruising and bleeding

Platelets are specialized cells in your blood that prevent bleeding and help stop bleeding.  Lupus can cause a reduction in platelet levels.  Some symptoms of low platelets (thrombocytopenia) include:

I think I may have lupus?

If you think you may have lupus, talk to your primary care physician, your GP, or make a consultation with a licensed rheumatologist. Remember this is not a common disease, so more often than not, it isn’t lupus.  Because the condition can affect many organ systems, it can mimic many diseases.  Most of these are much more common than lupus.

Get involved

Would you like to get involved?  Follow these links!

Lupus Foundation of America

Lupus Research Alliance

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.

References

[1] West, SG. Rheumatology Secrets 3rd edition

Diseases and Conditions

Pneumonia vaccines in people with autoimmune diseases

December 19, 2017
Pneumonia vaccines in people with autoimmune diseases

Do you need to get the pneumonia vaccine? Patients that have concurrent autoimmune diseases are at a higher risk of infection than others. In fact, infection is one of the most common causes of hospitalization in patients suffering from rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE); therefore, it is important to do everything possible to protect yourself from getting sick. The “pneumonia vaccine” or pneumococcal vaccine protects patients from pneumococcal diseases caused by the bacterium, Streptococcus pneumonia. Pneumococcal diseases can be serious and even fatal. Each year, in the United States alone, approximately 18,000 older adults die from pneumococcal diseases.

What is Streptococcus pneumoniae?

Pneumoniae is a gram-positive, spherical bacterium that has more than 90 known variations of the species, also known as serotypes. These bacteria typically group together in pairs or chains. They are the bacteria responsible for pneumococcal diseases including pneumonia, meningitis, bacteremia (infection of the blood), and many other severe illnesses. Pneumococcal pneumonia is the most common clinical presentation of a pneumococcal disease. It is a major cause for community-acquired pneumonia and results in about 400,000 hospitalizations every year. Pneumococcal meningitis can result in deafness and brain damage. It kills about 1 child in 10 who get it.

What are some signs and symptoms of pneumonia?

Pneumonia can look a lot like the common cold or the flu. Common symptoms include:

  • Abrupt onset of fever
  • Chills or rigors (uncontrollable shaking)
  • Chest pain
  • Productive cough
  • Trouble breathing
  • Malaise
  • Feeling of weakness

Which vaccine should I get?

Currently, there are two forms of pneumonia vaccine that is available in the US. Both forms of the vaccine are inactivated or “killed” vaccines.

Vaccine Brand Name Abbreviation
Pneumococcal conjugate vaccine Prevnar 13 PCV13
Pneumococcal polysaccharide vaccine Pneumovax PPSV23

The pneumococcal conjugate vaccine (PCV) 13, also known as Prevnar 13, covers 13 serotypes. The pneumococcal polysaccharide vaccine (PPSV) 23, also known as Pneumovax, covers 23 serotypes. Typically, both PCV13 are PPSV23 are required with PCV13 being given prior to PPSV23. The minimum interval between PCV13 and PPSV23 is 8 weeks. The table below shows the recommended vaccine schedule for immunocompromised people. Talk to your healthcare provider or pharmacist for more information about which vaccine would be most appropriate for you.

Pneumococcal vaccine status: Age FIRST give: THEN give: THEN give:
None/Unknown 19-64 years PCV13 PPSV23

(at least 8 weeks later)

PPSV23

(at least 5 years after first PPSV23 dose)

None/Unknown 65+ years PCV13 PPSV23

(12 months after PCV 13)

PPSV23 65+ years PCV13

(at least 1 year after PPSV23)

PPSV23

(6-12 months after PCV 13 AND 5 years after PPSV23)

PPSV23 Under 65 years PCV13

(at least 1 year after PPSV23)

Second dose of PPSV23

(at least 8 weeks after PCV13 AND at least 5 years after first dose of PPSV23)

Third dose of PPSV23

at age 65 (if at least 5 years have passed since last dose of PPSV23)

PCV13 No additional PCV13 doses are needed. At least 8 weeks must elapse before getting a dose of PPSV23.

What are some potential side effects?

These vaccines are normally well tolerated; however, side effects may still occur. Possible side effects may include

  • Injection site reactions including redness, pain, and swelling where the shot was given
  • Flu-like symptoms (mild fever, fatigue, headache, chills, or muscle pain)
  • Loss of appetite
  • Irritability
  • Life-threatening allergic reactions from this vaccine may also occur but are very, very rare

Where can I get the pneumonia vaccine?

Most doctor’s offices carry the pneumococcal vaccination. Call your primary care provider or specialist to see whether they can give you your pneumococcal vaccination. If not, most pharmacies also give this service as well.

Who should NOT get the vaccine?

Patients with a known hypersensitivity or allergy to any part of the vaccine should not receive the vaccine. Patients who have had allergic reactions to vaccines containing diphtheria (ex: Tdap, DTaP, tetanus vaccine) should tell their healthcare provider or pharmacist before receiving PCV13.

If you are feeling sick, wait until you feel better before getting the pneumonia vaccine.

Patients who are pregnant should not get the vaccine. Although there is no evidence of the vaccine being dangerous to the mother or the baby, as a precaution, it is recommended to receive the vaccine prior to conception.

Key points

  • The pneumonia vaccine can help protect you against serious or even fatal diseases.
  • Medications used in autoimmune conditions including RA, psoriatic arthritis, lupus, etc. can further weaken your immune system and predispose you to getting infections. Staying vaccinated can help keep you healthy and lower your risk of getting sick.
  • Pneumococcal diseases can spread from person to person through close contact.
  • Patients should receive up to 1 dose of PCV13 and up to 3 doses of PPSV23 in their lifetime. PCV13 and PPSV23 should not be administered on the same day.
  • The pneumonia vaccine can be given year-round. You can even get the flu shot on the same day. Just try to get one in each arm to reduce any pain associated with getting the vaccines.
  • When thinking about starting chemotherapy or other immunosuppressive therapy (ex. steroids, biologics, etc.), the interval between vaccination and initiation of immunosuppressive therapy should be at least 2 weeks.
  • You will not get sick after getting the vaccine; however, it is not uncommon to have flu-like symptoms that are caused by your body’s response to the vaccine.
  • It will take a couple weeks before the vaccine will take its full effect.

Final thoughts

For immunocompromised people, it is recommended to receive vaccination against the bacterium S. pneumoniae as outlined by the Center for Disease Control (CDC) vaccine schedule. Many studies and government organizations also support the use of the vaccination as the benefits outweigh the risks of this preventable infection. Other people who are also immunocompromised or over the age of 65 should also receive the vaccine. These patients may include those with congenital or acquired immunodeficiency, HIV infection, chronic renal failure, nephrotic syndrome, leukemia, lymphoma, Hodgkin disease, generalized malignancy, multiple myeloma, solid organ transplant, or iatrogenic immunosuppression. Iatrogenic immunosuppression is based on the use of immunosuppressive drugs, including long-term systemic corticosteroids (e.g., prednisone) and radiation therapy. Talk to your healthcare provider or pharmacist if you have any questions or concerns on the pneumonia vaccine.

 

Guest Authors: Yahya Rasoully, PharmD Candidate 2018; Stephanie Tchen, PharmD, PGY-1 Pharmacy Resident; and Jessica Farrell, PharmD

References

  • Pneumoccoal Disease. Centers for Disease Control and Prevention. Epidemiology and Prevention of Vaccine-Preventable Diseases. Hamborsky J, Kroger A, Wolfe S, eds. 13th ed. Washington D.C. Public Health Foundation, 2015.
  • Rákóczi É, Szekanecz Z. Pneumococcal vaccination in autoimmune rheumatic diseases. RMD Open. 2017;3:e000484.
  • Nagel J, Saxne T, Geborek P, et al. Treatment with belimumab in systemic lupus erythematosus does not impair antibody response to 13-valent pneumococcal conjugate vaccine. Lupus 2017;26:1072–81.
  • Alten R, Bingham CO, Cohen SB, et al. Antibody response to pneumococcal and influenza vaccination in patients with rheumatoid arthritis receiving abatacept. BMC Musculoskelet Disord 2016;17:231.
  • Centers for Disease Control and Prevention. Pneumococcal Vaccines (PCV13 and PPSV23): Addressing Common Questions about Pneumococcal Vaccination for Adults. Available from https://www.cdc.gov/vaccines/hcp/adults/downloads/fs-pneumo-hcp.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.