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Diseases and Conditions

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.

Diseases and Conditions Self-Injection Videos

How to inject Actemra and Kevzara

October 24, 2017
How to inject Actemra and Kevzara

In this week’s edition of RheumDoctor, Dr. Farrell will teach how to inject Actemra and Kevzara in the comfort of your home.  Both of these medications work by blocking interleukin-6, a cytokine involved in inflammation.  Kevzara, also known as sarilumab, recently obtained FDA approval for the treatment of rheumatoid arthritis. Actemra, also known as tocilizumab, is also prescribed for rheumatoid arthritis but also recently obtained FDA approval for the treatment of giant cell arteritis.  Without further adieu…

Preparing for your injection

  • Keep your medication stored in the refrigerator until use
    • Before injecting medication, take the prefilled syringe out of the refrigerator.
    • Allow it to warm up to room temperature.
  • Pick a place in your house that is clean and has room for your materials (such as the kitchen table).
  • Wash your hands thoroughly with either:
    • Soap & water
    • Hand sanitizer
  • Chose an area to inject – Thigh or Stomach.
    • Chose an area that is intact and clear.
    • It should not have any of the following:
      • Cuts
      • Scrapes
      • Bruises
      • Psoriasis patches
      • If you have extensive psoriasis, inject between patches
      • Moles
      • Scars
    • Please rotate area each time you inject (shown in picture below).

Areas to inject subcutaneous medication

  • Cleanse chosen area
    • Cleanse chosen area with either of the following:
      • Alcohol swab
      • Alcohol and a cotton ball
    • Use the chosen alcohol material to “swipe” area
      • Can either use a circular motion or wipe in “strips”
      • Allow the area to dry

Injecting Actemra or Kevzara with a prefilled syringe

  • Pull off the cap and observe the syringe.
  • Pinch the skin around the injection site and enter at a 45-degree angle
  • Press the plunger (slowly) to administer the medication
  • Once the medication is fully administered, the plunger will reach the bottom and a spring will place a cover over the needle

After the injection

  • Properly dispose of the entire prefilled syringe
    • Sharps Container
      • Can be purchased at your local pharmacy
      • Disposal
        • Hospitals may take full sharps containers, ask first.
        • Pharmacies and Doctors’ offices are not allowed to take used syringes or needles
  • Discard remaining materials in the trash (cap, alcohol swabs, etc.)

For more information about Actemra.

For more information about Kevzara.

Credits

Jessica Farrell, PharmD.  Clinical Pharmacist, The Center for Rheumatology/Associate Professor, Albany College of Pharmacy and Health Sciences

With the help of Autumn Koniowka. Doctor of Pharmacy Candidate Class of 2018, Albany College of Pharmacy and Health Sciences, and Megan Phillips. Doctor of Pharmacy Candidate Class of 2018, Albany College of Pharmacy and Health Sciences.

A special thanks to Tammy Garren, PhD. Instructional Designer, Center for Innovative Learning, Albany College of Pharmacy and Health Sciences.

Injection site image: By British Columbia Institute of Technology (BCIT). Download this book for free at http://open.bccampus.ca [CC BY 4.0 (http://creativecommons.org/licenses/by/4.0)], via Wikimedia Commons

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 When to see a rheumatologist

What is a positive rheumatoid factor?

October 17, 2017
What is a positive rheumatoid factor?

Introduction

What does it mean to have a positive rheumatoid factor (RF)?  Does this mean that you have rheumatoid arthritis, are you at risk of developing rheumatoid arthritis, is your prognosis worse, or is it completely unrelated to arthritis?

Before answering, what is a positive rheumatoid factor, we need to understand antibodies and how they are made.  We can then better understand autoantibodies, and more specifically rheumatoid factor.

What is an antibody?

The goal of the immune system is to protect yourself from bacteria, viruses, as well as cancers.  The immune system is made up of many different components, however, in broad terms it can be divided into three categories: physical barriers, the innate immune system, as well as the adaptive immune system.  Physical barriers include skin as well as mucous membrane barriers that line your digestive, respiratory, and your reproductive tracts.  The innate immune system is the second line of defense.  This is the part of the immune system that deals with immediate dangers.  For example, the skin reaction you get when you get bitten by a dog or you when you accidently cut your hand with a knife.  The third level of defense is called the adaptive immune system.  This is the part of the immune system responsible for making antibodies.

Antibodies, also called immunoglobulin (Ig), are specialized proteins that defend against specific invaders.  An antigen stimulates your body to make an antibody.  For example, if someone gets the chickenpox (antigen) the body will make antibodies directed against the chickenpox virus.  So the next time that person is exposed to the chickenpox virus, the body will have chickenpox antibodies to prevent the infection.

Antibody structure

Immunoglobulin are made up of light and heavy chains.  They also have two regions, Fab regions and the Fc region.  The Fab region is also called the variable region.  This is the place where specific antigens attach to the antibody.  It is variable, because each antigen has its own antibody.  The Fc region helps the antibody communicate with other parts of the immune system.  The Fc region is also called the constant region.  It’s constant because it doesn’t really vary all that much.[1]

Immunoglobulin structure

 

Are there different types of antibodies?

Yes.  There are 5 different classes of antibodies: IgG (75%), IgA, IgD, IgE, and IgM. Remember how I said the Fc region “doesn’t vary all that much”? The Fc region determines the class of the antibody and in turn, which immune system cells the antibody will interact with.  For example, dust mite particles cause the immune system to make IgE antibodies.  When the body is exposed to dust mites for a second time, IgE antibodies interact with mast cells, which release histamine, which cause an allergic reaction.[2]

Every class of immunoglobulin looks different and as I mentioned interacts with different parts of the immune system. IgM antibodies are made very early on during an infection.  IgG antibodies are made about 2-3 weeks after an infection and you typically find them in blood.  IgA antibodies are made in the gut, nose, lungs, and mammary glands.  IgE antibodies are made to parasites and they trigger allergies.  Currently, we don’t know too much about IgD.

What is a rheumatoid factor?

A rheumatoid factor is created when an IgM antibody binds to the Fc portion of an IgG antibody.  In simple terms, this is a situation where an antibody attacks another antibody.  When you have many antibodies doing this at once, they can form large IgM-IgG antibody complexes, which can stimulate the immune system to make some serious inflammation.

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

The answer to this question is varies depending which lab your doctor sent your blood.  Each lab has their own specific cut off points.  Below you will find the cutoff points for Labcorp and Quest Diagnostics.

Labcorp

Methodology: Latex immunoturbidmetry

Negative: < 14.0 IU/mL

Quest Diagnostics

Methodology: Immunoturbidimetric

Negative: ≤ 14 IU/mL

Note: There are many different ways to measure rheumatoid factor and even specific types of rheumatoid factor: IgM, IgG, IgA.  These will all have their own cutoff points.

Now, what does having a positive rheumatoid factor mean?  The answer to this question depends on your symptoms.

Rheumatoid factor and rheumatoid arthritis

About 70% to 80% of people that have rheumatoid arthritis have a positive rheumatoid factor.  Not having a positive rheumatoid factor doesn’t necessarily rule out the disease, but rather, it makes it less likely.  Having rheumatoid arthritis AND having a positive rheumatoid factor is associated with a more severe form of the disease and having non-joint symptoms like nodules or lung involvement (interstitial lung disease).  Unlike lupus, rheumatoid factor doesn’t really go up or down depending how active rheumatoid arthritis, so we don’t follow it if you have an established diagnosis.[3]

What are some other diseases that cause a positive rheumatoid factor?

Rheumatoid factor has an 86% specificity for rheumatoid arthritis.  That being said, if you test positive for rheumatoid factor, there’s a pretty good chance that you have or will have rheumatoid arthritis in the future, but this is by no means set in stone. It’s very important to realize that someone can test positive for rheumatoid factor, AND NOT have rheumatoid arthritis because many diseases can cause the immune system to form antibodies directed towards the Fc portion of an IgG antibody.  However, in most non-autoimmune conditions the rheumatoid factor tends to be lower than in rheumatoid arthritis.  Moreover, when one finds more than one different type of rheumatoid factor particular in the fluid of a joint (e.g., IgM RF and IgA RF), this indicates that we are dealing with rheumatoid arthritis.

Category Disease
Rheumatic diseases
  • Rheumatoid arthritis
  • Systemic lupus erythematosus (SLE)
  • Scleroderma
  • Mixed connective tissue disease
  • Sjogren’s syndrome
Viral infections
  • HIV/AIDS
  • Mononucleosis (Epstein-Barr virus)
  • Hepatitis
  • Influenza
Parasitic infections
  • Malaria
  • Schistosomiasis
  • Trypanosomiasis
  • Kala-azar
  • Filariasisis
Chronic bacterial infections
  • Subacute bacterial endocarditis
  • Tuberculosis
  • Syphilis
  • Salmonellosis
  • Brucellosis
  • Leprosy
  • Yaws
Cancers
  • Lymphoma
  • Leukemia
  • Multiple myeloma
Hyperglobulinemic diseases
  • Sarcoidosis
  • Chronic liver disease
  • Chronic pulmonary disease
  • Cryoglobulinemia
  • Hypergammaglobulinemic purpura[4]

 

It is also important to mention that approximately 5 – 25% of individuals aged 60 years and older have a positive rheumatoid factor without any underlying causative disease.

I have a positive rheumatoid factor, but I have no symptoms

This is where it gets a little tricky.  Here are the following possible scenarios:

  1. You may develop rheumatoid arthritis in the future.
  2. It may be that you actually have another non-rheumatic disease that is causing you to test positive for rheumatoid factor.
  3. The rheumatoid factor is not clinically significant.

When faced with a positive rheumatoid factor, it’s important to rule out other conditions that cause positivity.  If the workup is negative, care monitoring is recommended.  Knowing the symptoms of autoimmune joint disease is of utmost importance as well.

Can you predict who will develop rheumatoid arthritis?

We cannot predict with 100% certainty, who will and who won’t develop rheumatoid arthritis.  There are a few factors that increase the odds like having both a rheumatoid factor AND anti-citrullinated peptide antibodies (anti-CCP)[5].  This is particularly true for people that have high levels of anti-CCP antibodies.[6]

The time between the formation of autoantibodies and rheumatoid arthritis is called preclinical rheumatoid arthritis.  Other terms include: pre-RA, autoantibody-positive arthralgia, probable RA, very early RA, and early undifferentiated arthritis progressing to RA.

Phases of preclinical rheumatoid arthritis

Preclinical RA goes through many phases.  Not everyone progresses so smoothly and some people can even skip steps.

Phase I

People with certain genetic predispositions are exposed to environmental factors that trigger an immune response.  Smoking is a known trigger.

Phase II

The immune system starts making rheumatoid factor and/or anti-CCP antibodies.

Phase III

People start experiencing some joint pain and stiffness, but no swelling or over autoimmune joint pain.

Phase IV

There is some joint swelling, but it’s limited to 1-2 joints.  We can now call this undifferentiated arthritis but it’s not rheumatoid arthritis yet.  If it happens on and off, we sometimes call it palindromic rheumatism.  We think that 30 – 50% of people with undifferentiated arthritis will go into remission, 30-40% will progress to other diseases, and 30 – 40% will evolve into rheumatoid arthritis.

Phase V

It’s spreading!  Now we can call this rheumatoid arthritis.[7]

Is there anything I can do to prevent rheumatoid arthritis?

The following are some modifiable risk factors that can help prevent rheumatoid arthritis.

  • Avoid/quit smoking
  • Maintain good dental hygiene
  • Eat a balanced diet containing fish oil, antioxidants, and vitamin D
  • Avoid excess coffee and foods with high salt content
  • Keep a healthy body weight
  • Prevent infections[8]

Conclusion

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

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] L Sompayrac. How the Immune System Works 4th edition

[2] L Sompayrac. How the Immune System Works 4th edition

[3] Rheumatology Secrets 3rd edition

[4] Adapted from Kelley’s Textbook of Rheumatology, 8th edition

[5] Sun J, Zhang Y, Liu L, Liu G. Diagnostic accuracy of combined tests of anti cyclic citrullinated peptide antibody and rheumatoid factor for rheumatoid arthritis: a meta-analysis. Clin Exp Rheumatol. 2014 Jan-Feb;32(1):11-21.

[6] Heidari B, Firouzjahi A, Heidari P, Hajian K. The prevalence and diagnostic performance of anti-cyclic citrullinated peptide antibody in rheumatoid arthritis: the predictive and discriminative ability of serum antibody level in recognizing rheumatoid arthritis. Ann Saudi Med. 2009 Nov-Dec;29(6):467-70.

[7] Paul BJ, Kandy HI, Krishnan V. Pre-rheumatoid arthritis and its prevention. Eur J Rheumatol. 2017 Jun; 4(2): 161–165.

[8] Paul BJ, Kandy HI, Krishnan V. Pre-rheumatoid arthritis and its prevention. Eur J Rheumatol. 2017 Jun; 4(2): 161–165.

Immunoglobulin image By Fvasconcellos 19:03, 6 May 2007 (UTC) [Public domain], via Wikimedia Commons

Diseases and Conditions

Guide to living with psoriatic arthritis: Part 1

September 26, 2017
Psoriatic arthritis is an autoimmune disease that not only affects skin, but also joints

In an earlier post I presented a guide to living with rheumatoid arthritis (RA).  That’s all good if you have RA, but what if your rheumatologist diagnosed you with psoriatic arthritis?  What’s psoriatic arthritis and how is it similar or dissimilar to RA?  This week I’ll present to you Part 1 of a Guide to living with psoriatic arthritis.  I’m going to present this as a three-part series.  Part 1 will cover the basics: what is psoriatic arthritis, the cause, risks, symptoms, diagnosis, and treatment.  In Part 2 I’ll cover prognosis, what to expect, diet and exercise.  In Part 3, I’ll be covering the financial side of psoriatic arthritis: How to get access to medications and how to navigate the complicated world of health insurance.

What is psoriatic arthritis?

Psoriatic arthritis (PsA) is a type of autoimmune inflammatory arthritis that afflicts people who suffer from psoriasis (PsO).  It’s estimated that about 26% of people who suffer from psoriasis will get psoriatic arthritis at one point during their lifetime.  Typically, people develop psoriasis first and then get the arthritis.  In some cases, people develop arthritis first and then get psoriasis but this is a lot less common.   Psoriatic arthritis is one of the more common causes of autoimmune arthritis affecting about 2 to 3% of the population.[1]

What is psoriasis and what are the different types of psoriasis?

Psoriasis is an autoimmune disease that affects the skin.  It typically involves the elbows, knees, and scalp, but you can find it in many other areas.  It typically causes itchiness, burning as well as a stinging sensation.  Psoriasis affects about 2% of African-Americans and affects about 3.6% of Caucasians.  Usually people develop it between the ages of 15 and 35, but it can also happen in very young children and older adults as well.

There are many different types of psoriasis and they are all associated with psoriatic arthritis.

  • Plaque psoriasis
  • Guttate psoriasis
  • Inverse psoriasis
  • Pustular psoriasis
  • Erythrodermic psoriasis (life-threatening type of psoriasis)

Please follow this link to learn more about psoriasis.[2]

What causes psoriatic arthritis?

Like most diseases in rheumatology, we’re not sure.  We do know that there’s a strong genetic and environmental part to psoriatic arthritis.  Here are some genetic associations.

  • HLA-Cw6 is associated with severe early onset skin psoriasis
  • HLA-B38 and HLA-B39 are associated with psoriatic arthritis
  • HLA-B27 is associated with psoriatic arthritis that affects the spine.

Although genes do play a part in psoriatic arthritis, most people who have psoriatic arthritis have no genetic risk factors.

The Koebner Phenomenon

Have you ever heard of the Koebner phenomenon?  This phenomenon describes a new skin lesion in an area where healthy skin was injured.  For example, let’s imagine that you have psoriasis.  A mosquito comes along and bites you, it itches, so you scratch.  Then, about 10 days later, you notice that you’ve developed psoriasis in the area you scratched.  That’s the Koebner phenomenon.[3]

Now try to imagine the Koebner phenomenon involving joints.  It’s thought that about 25% of people who get psoriatic arthritis develop the condition after trauma to a joint.  We call this the deep Koebner phenomenon.[4]

Ultimately, we still don’t know exactly why people develop psoriatic arthritis.  Our best guess like most autoimmune diseases, is that certain people are born with a predisposition to develop both psoriasis and psoriatic arthritis.  Then, something in the environment triggers the disease to “come online”.

Does everyone with psoriasis get psoriatic arthritis?

No.  A recent Japanese study tried to find certain risk factors that predispose patients with psoriasis to develop psoriatic arthritis.  First, they found that about 17% of people with psoriasis also had psoriatic arthritis.   Second, they found that people who had psoriasis involving their nails had a higher chance of having psoriatic arthritis: 29% (PsO) versus 62% (PsA).  Interestingly, they also found that people who had high uric acid levels also had a higher risk of having psoriatic arthritis 9% (PsO) versus 22% (PsA).[5]

As a side note, when uric acid levels are high, this increases the risk of gout.

How does psoriasis affect the nails?

Nail psoriasis is very common.  It ranges from about 50% to 87% of people who have psoriasis.  Now, nail psoriasis can present in many ways depending on the anatomic site of the psoriasis inside the nail.  First, a bit of anatomy.

Nail anatomy

 

The nail consists of the nail fold, the nail matrix, and the nail bed.  The nail fold is where the blood vessels supplying the nail come from.  They can be compromised in many diseases such as scleroderma.  The nail matrix is responsible for formation of the nail plate and the nail bed is responsible for attaching the nail plate firmly in place.

Anatomy of the nail

 

When psoriasis affects the nail matrix.  It can cause pitting, crumbling, white spots and red spots in the lunula.  When psoriasis affects the nail bed, it can cause splinter hemorrhages and splitting of the nail from the nail bed (onychyolysis).[6]  Please click the following link to learn more about nail psoriasis + pics.

Please note that none of the features of nail psoriasis are exclusive to psoriasis.  Other diseases can cause these, including:

  • Reactive arthritis
  • Alopecia areata
  • Chemical dermatitis
  • Pemphigus vulgaris.
  • Incontinentia pigmenti

How is psoriatic arthritis different from rheumatoid arthritis?

Although both psoriatic arthritis and rheumatoid arthritis are both autoimmune diseases that affect joints, they are both distinct diseases.  It isn’t simply because you have psoriasis and inflammation in your joints, that you have psoriatic arthritis. Many people with psoriasis have rheumatoid arthritis.  Psoriatic arthritis and rheumatoid arthritis have their own pathophysiology, epidemiology, and symptoms.  Although they do share many treatment options, they also have some medications tailor-made for them.

Here some of the main clinical differences between psoriatic arthritis and rheumatoid arthritis.

  Psoriatic arthritis Rheumatoid arthritis
Joint distribution Asymmetrical Symmetrical
Joint involvement DIP, dactylitis MCP, PIPs, wrists, and MTPs
Involvement of the spine Common Rare, involves the cervical spine
Labs* RF and CCP antibody negative RF and/or CCP antibody positive

* RF = rheumatoid factor, CCP = Cyclic citrullinated peptide antibodies

As you’ll see later on, it’s a lot more complicated that.  Many people presenting with psoriatic arthritis present almost exactly like rheumatoid arthritis.  Here were a few other features that favor a diagnosis of psoriatic arthritis.

  • Presence of nail pits
  • When there is inflammation of the distal interphalangeal joints (Tip of your finger) without any evidence of osteoarthritis
  • “Sausage digits” = dactylitis. This happens when the tendons that supply of the fingers and toes get inflamed.
  • Any inflammation of tendons and ligaments, such as Achilles tendinitis and plantar fasciitis.
  • When there is a family history of psoriasis or psoriatic arthritis, particularly in a first-degree relative. That mom, dad, kids and siblings.
  • The spine is involved.

What are the symptoms of psoriatic arthritis?

If you’re experiencing joint pain and you have a history of psoriasis, particularly psoriasis that involves your nails, you need to think about psoriatic arthritis.  So what do I mean by joint pain?  When it comes to joint pain, what I really mean is, autoimmune or more specifically, inflammatory joint pain.

Psoriatic arthritis can affect almost any joint: knuckles, wrists, toes, knees, shoulders, elbows, hips, and the spine.  Mechanical joint pain is very different from inflammatory joint pain.  Let me explain.

Peripheral inflammatory joint pain

Peripheral joints include all joints except those involving the spine.  When there is inflammation in a peripheral joint, typically people experience pain, swelling, and stiffness, particularly in the morning that lasts at least an hour.  Sometimes they do see some redness and the joints may feel hot at times.  Often times, people also feel a lot more tired than usual, and they can even run low-grade fevers.

Axial inflammatory joint pain

Axial joints are those that involve the spine.  Inflammation involving the back causes symptoms that are very different from your usual mechanical back pain.  Here are some of the following key characteristics:

  • 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.

Enthesitis

Enthesitis means inflammation of connective tissue that attaches to bones.  These include tendons, ligaments, and bursae.  Most cases of enthesitis are caused by 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 with Achilles tendinitis on both feet, runner’s knee, and plantar fasciitis happening all at once, for no good reason.  Not a pleasant experience.

Uveitis

Uveitis is a general term that we use to describe a group of inflammatory diseases that cause inflammation in many parts of 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.

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 know and keep in the back of your mind.[7]

Patterns of disease

Just like rheumatoid arthritis, psoriatic arthritis, can manifest in many ways.  For those of you who want to get really technical, I’ve included a table describing the most common ways psoriatic arthritis presents.

Subtype Percentage Typical joints
Asymmetric oligoarticular* disease 15-20% DIP joints and PIP joints of the hands and feet.  MCP joints, MTP joints, knees, hips, and ankles.#
Predominant DIP involvement 2-5% DIP joints
Arthritis mutilans$ 5% DIP and PIP joints
Polyarthritis! “rheumatoid–like” 50-60% MCP joints, PIP joints, and wrists.
Axial involvement only (spine) 2-5% Sacroiliac joints, vertebral
Enthesitis predominant Tendons and ligaments[8]

* oligoarticular = 2 – 4 joints

# DIP = distal interphalangeal joints, PIP = proximal interphalangeal joints, MCP = metacarpophalangeal joints, MTP = metatarsophalangeal joints

$ Mutilans = severely deformed

! Polyarthritis = 5 or more joints involved

How is psoriatic arthritis diagnosed?

We currently use the CASPAR criteria to make the diagnosis of psoriatic arthritis.  You need three points to get the diagnosis because having 3 or more points has a 99% specificity and 92% sensitivity for the diagnosis of psoriatic arthritis.  Obviously, there are exceptions as the CASPAR criteria are predominantly used for research purposes.

As you can see, you don’t need to have psoriasis to get a diagnosis of psoriatic arthritis.  I know this sounds counterintuitive.

CASPAR classification criteria

  • Evidence of psoriasis (current, past, family)
    • 2 points if current
    • 1 point if history of psoriasis or family history
  • Psoriatic nail dystrophy = 1 point
  • Negative rheumatoid factor = 1 point
  • Dactylitis = 1 point
  • X-ray changes = 1 point

HLA-B*27 antigen

Unlike rheumatoid arthritis, we do not have blood tests to help with the diagnosis of psoriatic arthritis.  At times, your rheumatologist may order something called a HLA-B*27 test.

HLA-B*27 is a genetic test. The majority of people who have a positive HLA-B*27 are perfectly healthy. HOWEVER, having a positive HLA-B*27 can put you at increased risk of developing what we call spondyloarthritis-associated diseases. This is a family of autoimmune diseases. They include:

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

Not every person with psoriatic arthritis will test positive for HLA-B*27, however, those that do, have a higher risk of having axial involvement.[9]  This is important to know, because it may affect the medication your rheumatologist recommends.

Is there a cure for psoriatic arthritis?

The simple answer to this question is no.  Psoriatic arthritis is a chronic, lifelong disease.  Although there is no cure for psoriatic arthritis, there are many medications that can help halt or slow down progression: disease modifying anti-rheumatic drugs (DMARD).

Cardiovascular disease and psoriatic arthritis

In recent years, scientists have found an association between cardiovascular disease and many autoimmune diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis, psoriasis, Crohn’s disease, ulcerative colitis, and psoriatic arthritis.  Basically, people who suffer from psoriatic arthritis have a higher risk of developing cardiovascular disease.[10][11]  [12] Unfortunately, they also tend to have more traditional cardiovascular risk factors like high blood pressure, high cholesterol, and diabetes.  [13]On the upside, effective treatment of psoriatic arthritis can decrease this risk.[14]

How is psoriatic arthritis treated?

Like rheumatoid arthritis, psoriatic arthritis is treated with disease modifying anti-rheumatic drugs (DMARDs). These medications are designed to stop or slow down the progression of psoriatic arthritis by targeting the faulty part of the immune system.  Without treatment, psoriatic arthritis, can cause permanent damage to joints, tendons and ligaments leading to functional impairment and a decrease in quality of life.

Which DMARDs are used to treat psoriatic arthritis?

The following are some of the medications that doctors often use to treat psoriatic arthritis.  Your doctor will recommend certain treatments based on the involved joints and organs, as well as severity, allergies, and other medical conditions you may have.

I’ve broken down the different medications into the following broad categories.

Nonsteroidal anti-inflammatory drugs

  • Ibuprofen
  • Meloxicam
  • Naproxen
  • Sulindac
  • Etodolac
  • Diclofenac
  • Indomethacin
  • Celecoxib

Conventional DMARDs

  • Hydroxychloroquine (Plaquenil) – caution as this medication may make psoriasis flare
  • Methotrexate
  • Leflunomide (Arava)
  • Sulfasalazine
  • Azathioprine – rarely used for psoriatic arthritis

Biologics

Tumor necrosis factor – alpha (TNF-alpha) inhibitors

  • Certolizumab pegol (Cimzia)
  • Etanercept (Enbrel)
  • Adalimumab (Humira)
  • Infliximab (Remicade)
  • Golimumab (Simponi)

Interleukin 12 and 23 inhibitors

  • Ustekinumab (Stelara)

Interleukin 17 inhibitors

  • Secukinumab (Cosentyx
  • Brodalumab (Siliq) – not FDA approved for PsA
  • Ixekizumab (Taltz) – not FDA approved for PsA

T cell inhibitors

  • Abatacept

Interleukin 23 inhibitors

  • Guselkumab

Phosphodiesterase 4 inhibitors

  • Apremilast (Otezla)

To read more about treatment for psoriatic arthritis.  Please follow this link.

Biosimilars

Here in the US, we are starting to see biosimilar medications. These are medications that are sort of copied from existing biologic medications.  They are NOT generic medications. The problem with biosimilars is that because of their complexity, it literally is impossible to exactly copy a biologic medication. If you want to learn more about biosimilar medications, please check this article.

Can I stop my medications if I’m feeling better?

No.  Psoriatic arthritis is a life-long disease.  If you’re feeling better, great!  However, it’s probably your medications that are keeping you that way.  If you stop your medications the psoriatic arthritis will likely come back.  Psoriatic arthritis subsides spontaneously in a VERY small subset of people.

If your medication is making you feel sick, talk to your rheumatologist.  They truly have your best interest at mind and they want to find the best treatment for you.

Do not stop your medications without first consulting your rheumatologist.

Next steps

Let’s recap what we’ve learned today.

  • Psoriatic arthritis is an inflammatory arthritis that affects about 26% of people that suffer from psoriasis and affects about 2 to 3% of the population.
  • We know that there is a strong genetic link and environmental component to psoriatic arthritis, but the majority of cases happen spontaneously.
  • People with nail psoriasis have a higher risk of getting psoriatic arthritis.
  • The Koebner phenomenon describes the appearance of a new skin lesion in an area where healthy skin was injured. The same thing can happen in joints.  This is  the deep Koebner phenomenon.
  • Psoriatic arthritis can present in many ways. It can cause peripheral inflammatory arthritis, axial inflammatory arthritis, enthesitis, and uveitis.
  • Doctors use the CASPAR criteria to help make a diagnosis of psoriatic arthritis. You need three points to get the diagnosis because having 3 or more points has a 99% specificity and 92% sensitivity for the diagnosis of psoriatic arthritis.
  • There are no specific tests help make the diagnosis of psoriatic arthritis, however, people that test positive for HLA-B*27 have a higher chance of having psoriatic arthritis in their spine.
  • People with psoriatic arthritis have a higher risk of having cardiovascular disease but treatment can possibly decrease that risk.
  • Psoriatic arthritis is treated with disease modifying anti-rheumatic drugs.

In part 2 of the Guide to living with psoriatic arthritis, I’ll be covering topics such as natural treatments for nail psoriasis and psoriatic arthritis, the FODMAP diet, how to exercise, and strategies on how to reduce stress.   In part 3 of the Guide to living with psoriatic arthritis, I’ll be covering the financial aspect of psoriatic arthritis most notably, health insurance coverage and the prior authorization process for expensive medications.

Stay tuned and please leave your comments below!

Please follow this link to request a rheumatology consultation.

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

Sick woman main areas of the human body affected by psoriasis: By ann131313 via Shutterstock

Nail anatomy by  Blausen.com staff (2014). “Medical gallery of Blausen Medical 2014”. WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.010. ISSN 2002-4436. (Own work) [CC BY 3.0 (http://creativecommons.org/licenses/by/3.0)], via Wikimedia Commons

[1] Rheumatology secrets, 3rd edition

[2] https://www.psoriasis.org/about-psoriasis

[3] https://www.dermnetnz.org/topics/the-koebner-phenomenon/

[4] Rheumatology secrets, 3rd edition

[5] 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]

[6] Manhart R, Rich P. Nail psoriasis. Clin Exp Rheumatol. 2015 Sep-Oct;33(5 Suppl 93):S7-13.

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

[8] Rheumatology Secrets, third edition

[9] Jadon DR, et al. Axial disease in psoriatic arthritis study: defining the clinical and radiographic phenotype of psoriatic spondyloarthritis. Ann Rheum Dis. 2017 Apr;76(4):701-707. doi: 10.1136/annrheumdis-2016-209853. Epub 2016 Dec 2.

[10] Ozkan SG, Yzisiz H. Gokbelen YA, Borlu F, Yazisiz V.  Prevalence of metabolic syndrome and degree of cardiovascular disease risk in patients with psoriatic arthritis. Eur J Rheumatol. 2017 Mar;4(1):40-45. doi: 10.5152/eurjrheum.2017.16052. Epub 2017 Mar 1.

[11] Fernandez-Gutierrez B, et al. Cardiovascular disease in immune-mediated inflammatory diseases: A cross-sectional analysis of 6 cohorts. Medicine (Baltimore). 2017 Jun;96(26):e7308. doi: 10.1097/MD.0000000000007308.

[12] Castaneda S, et al. Cardiovascular morbidity and associated risk factors in Spanish patients with chronic inflammatory rheumatic diseases attending rheumatology clinics: Baseline data of the CARMA project. Semin Arthritis Rheum. 2015 Jun;44(6):618-26. doi: 10.1016/j.semarthrit.2014.12.002. Epub 2014 Dec 25.

[13] Jafri K, Bartels CM, Shin D, Gelfand JM, Ogdie A.  Incidence and management of cardiovascular risk factors in psoriatic arthritis and rheumatoid arthritis: a population-based study. Arthritis Care Res (Hoboken). 2017 Jan;69(1):51-57. doi: 10.1002/acr.23094. Epub 2016 Nov 28.

[14] Agca R, Heslinga M, Kneepkens EL, van Dongen C, Nurmohamed MT. The effects of five-year etanercept therapy on cardiovascular risk factors in patients with psoriatic arthritis. J Rheumatol. 2017 Jun 1. pii: jrheum.161418. doi: 10.3899/jrheum.161418. [Epub ahead of print]

Diseases and Conditions Patient Advocacy

Guide to living with rheumatoid arthritis: part 3

August 30, 2017
the prior authorization process: how medication get covered by your insurance company

If you’re reading this post, there’s a good chance you’ve just been diagnosed with rheumatoid arthritis (RA).  In Part 1 of the Guide to Living with Rheumatoid Arthritis, we went over the symptoms, diagnosis, and treatment of rheumatoid arthritis.  In Part 2 of the Guide to Living with Rheumatoid Arthritis, we went over expectations, how to break the news to your loved ones and your boss, as well as important topics like food, exercise, and lifestyle.

Now that you’re acquainted with RA, you may have realized that some of these medications are very expensive.  In Part 3 of Guide to Living with Rheumatoid Arthritis I’ll be covering the way doctors prescribe medications, how health insurance pays for the cost of medications, and finally how they end up in your possession.

The Process

As we’ve discussed previously, to treat rheumatoid arthritis you need to fight fire with fire.  In the case of RA, fire = a disease modifying anti-rheumatic drug or DMARD.  This is a medication that puts the immune system back in-check and calms it down.

They’re are two kinds of DMARDs: conventional DMARDs and biologics.  Conventional DMARDs like methotrexate are less complex and do not target a particular cytokine (type of inflammation).  They tend to cost less and typically don’t need pre-approval from your insurance company. Fortunately, methotrexate is the gold standard for the treatment of rheumatoid arthritis.

Biologics are the second type of DMARDs. These medications are a lot more complex and they do target a specific cytokine.  Biologics are typically used if there’s a good reason you can’t take a conventional DMARD or if they haven’t worked in the past.  Sometimes a rheumatologist may use a biologic and a conventional DMARD at the same time because they work better together.  Biologics come as self-injectable pens, prefilled syringes, and infusions (i.e., via an IV going into your veins).

Unlike conventional DMARDs, biologics are VERY expensive and do need pre-approval or prior authorization from your insurance company before starting the medication.  This means your doctor needs to justify this medication to the insurance company.

Prior Authorization

You may have heard your doctor say, “I’m going to need to get a prior authorization”.  A prior authorization is the process by which your doctor and his or her team will justify the use of the medication to your insurance company.  It typically involves A LOT of paperwork and phone calls.  Sometimes the process takes weeks to days, but sometimes it can take months.  If this is your first biologic, it typically takes 2 – 3 weeks from the moment your doctor prescribes the medication to the moment you receive it.  But again, every situation is different.  This is just an average.

First, every single medical office does prior authorizations slightly differently.  Let’s go through an example.

Getting started

  • Your doctor talks to you about the risks and benefits of a certain medication. If you consent to treatment, he or she will ask his assistant to start a prior authorization.
  • The assistant then gathers all your medical insurance information and starts filling out forms. There’s a different form for every medication and for every different insurance company.  The assistant then sends your doctor’s last progress note stating why you should receive this medication.  The package is then sent to your health insurance plan.
  • At this point, your health insurance plan will go over your case and decide whether they will approve the medication. The medical reviewers follow a strict set of guidelines set forth by the insurance plan.
  • Let’s say they don’t approve the medication. In some cases, your doctor could appeal their decision by speaking to a medical director at the insurance company.  At times, they want more information or a written letter with supporting scientific papers.  Sometimes this works and sometimes it doesn’t.  If it does great!  If it doesn’t your doctor may try to look into patient assistance programs if one is available or they may alter your treatment plan.

You’re approved!

Now let’s say the medication gets approved! The insurance company will contact your doctor’s office and let them know.  Because these medications are so expensive, your local pharmacy will not carry them.  They may need to go to a specialty pharmacy who will mail them to your house.

  • The assistant will alert your doctor and he/she will send the prescription to your pharmacy.
  • The specialty pharmacy then prepares the script and sets a delivery date with you.
  • The medication is then mailed to your house.
  • The process is a little different when it comes to infusible biologics. In this case, the medication will NOT be mailed to your house.  Instead, once your doctor’s office obtains the prior authorization, the infusion team at your doctor’s office will call to set up an appointment.  Sometimes, you may need to go to an infusion clinic or a hospital for treatment.

Now you see why prior authorizations take a long time!

Biologic medications

There are many types of biologic medications that work in all sorts of different ways, however, they are given in either of these forms:

  1. Infusions
  2. Self-injectables
  3. Pills

If prescribed an infusion you will get the medicine at your doctor’s office, an infusion center, or a hospital.  Treatment can range between 1-5 hours.  The doctor’s office will give you the medication and bill your insurance.

If you’re prescribed a medication that is self-injected, the medication will be mailed directly to you.  This may seem very daunting, however, many clinics have a team in place to help you through this process.  In my clinic, we have a dedicated team that will help you inject the medication for the first time in a supervised setting.  During that visit you can ask questions that you may have about the medication: how to store it, how to dispose of the syringes and/or pens, what to do when traveling with biologics, side effect, etc.

If you’re prescribed an oral biologic medication, then the medication will be mailed directly to your house and you would take it just like you would take any other pill, i.e., follow your doctor’s written instructions.

What if your insurance company does not want to cover the full cost of your medication or if you have a high deductible plan?

You should rest assured that there are several financial assistance options available if your insurance will not cover the full cost of the medication or if you have a high deductible plan.  Mind you, this does not guarantee that you will qualify but you won’t know unless you try.

For Commercial Insurance Plans (Group, Individual, Exchange) not Medicare

Some commercial insurance companies let their customers use a copay assistance card issued by the pharmaceutical company to help with the cost of the drug.  Call me old-fashioned, but in my experience, talking to an actual human being is much faster and effective than email or using a contact form.  For more information call the appropriate phone number listed below. If your plan allows the use of a copay card, the drug can often be obtained at a much smaller charge.

For Medicare Plans

The same medication options and medication administration options are available if you have Medicare or a Medicare Supplemental Plan, except you CANNOT use a copay assistance card.

Depending on the type of Medicare/supplemental plan that you have, the coverage of in-office infusions or self-administered shots widely vary.  They may cover the entire cost or only a portion of the medication.  For example, if your doctor prescribed you rituximab, your insurance company may cover 80% of the cost of the medication, leaving you with 20% of the total bill.  When a medication costs about $22,000, 20% is a lot!

It’s always advisable to contact the doctor’s office or Medicare to find the out-of-pocket costs before receiving any treatment to fully understand the potential costs.

Regardless if you have a Medicare, commercial health insurance plan or if you do not have any health insurance at all, you may still qualify for financial assistance if you cannot afford treatment.  Pharmaceutical companies and other non-profit organizations have many options that can even cover the full cost of treatment.

If you have concerns or questions about the costs of your treatment, please speak to your doctor or the patient advocate in your doctor’s office.

Conclusion

I hope this guide to living with rheumatoid arthritis has been informative and that you learned some valuable information about your diagnosis.  Here are a few final thoughts:

  • Please follow your rheumatologist’s management plan. If you have any concerns, about your symptoms or your medications, it’s always important to keep an open line of communication.
  • It’s important to tell all your different doctors about your new diagnosis and any new medications that you are taking.
  • Always carry an updated list of you medications in your wallet. You never know when someone may ask for it.  The doctor in the emergency room may not have access to your doctor’s records at 2 AM.
  • Make sure that you regularly follow-up with your rheumatologist in clinic. Your doctor may need to adjust your medications and watch for any side effects or complications.
  • If you cannot make a follow-up appointment, please contact your doctor’s office at least 48 hours in advance and re-schedule.
  • Learn as much as you can about your condition.
  • Talk to your friends and your family about your condition. You’re not alone.
  • Stay positive, keep active, and keep smiling!

Please leave your comments or questions below.

 

Co-written by Jessica Chapman, MD and Ilene Leveston, Patient advocate

Edited by Jessica Farrell, PharmD

Patient assistance programs for non-Medicare patients

Cimzia

1 (866) 952 – 7968

https://cimplicityonline.com/

Enbrel

1 (888) 4ENBREL or 1 (888) 436 – 2735

https://www.enbrel.com/support/financial-assistance/

Humira

1 (800) 4HUMIRA or 1 (800) 448 – 6472

http://www.abbviepaf.org/

https://www.humira.com/humira-complete/cost-and-copay

Simponi

1 (877) MYSIMPONI or 1 (877) 697 – 4676

http://www.janssenprescriptionassistance.com/simponi-cost-assistance

Remicade

http://www.janssenprescriptionassistance.com/remicade-cost-assistance

Xeljanz

1 (855) 493 – 5526

http://ra.xeljanz.com/rheumatoid-arthritis-support-resources/financial-help

Orencia

1 (800) ORENCIA or 1 (800) 673 – 6242

https://www.orencia.bmscustomerconnect.com/orencia-patient-assistance

Actemra

1 (800)-ACTEMRA or 1 (800) 228-3672

https://www.genentech-access.com/hcp/brands/actemra/find-patient-assistance.html

Kevzara

1 (844) 538 – 9272

https://www.kevzarahcp.com/kevzara-connect

https://www.kevzara.com/kevzara-copay-card

Rituxan

https://www.genentech-access.com/hcp/brands/rituxan/rituxan-ra/find-patient-assistance.html

Note: Rules, regulations, and contact information are subject to change.

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.