Lupus is such a complex and poorly understood disease that it can be difficult for patients and healthcare providers to even talk about, much less treat.  So, researchers at the Duke Lupus Clinic have proposed a new approach that may hold the key to better conversations – and even better science – when it comes to diagnosing and treating lupus.

• Introduction
• What are type 1 and type 2 lupus?
• Why is this new, redefinition of lupus important?
• In Conclusion: What could this mean for you?

Introduction:

The symptoms of systemic lupus erythematosus (SLE) are so complex and so variable for each individual, that making a definitive diagnosis and treatment plan can be challenging.  Even after a diagnosis, it can be problematic for clinicians – or even the patients themselves – to evaluate the level or state of the disease at any given time.

“If the pain and swelling go down, but I still feel fatigued, am I out of my flare … should I reduce my prednisone?”

“If my blood tests continue to look good, but I still have brain fog and difficulty sleeping, am I in remission?”

It is almost as if lupus were two kinds of disease – one that hits hard with acute symptoms that may flare and fade, and another that is constantly in the background doing its damage more slowly.

Some chronic symptoms like fatigue and depression can be overlooked compared to the more obvious ones like visible rashes, joint pain and other examples of acute inflammation.  Many chronic symptoms that are difficult to describe, much less measure, may be under-appreciated or even ignored.  This can lead to miscommunication and possibly distrust between patients and healthcare providers … and this in turn, can have serious consequences for the patient.

Researchers at the Duke Lupus Clinic recognized this and designed an innovative method for categorizing each symptom of lupus in a better way.  They divided lupus into two subtypes:  Type 1 and Type 2.  Type 1 lupus refers to the classic symptoms that most people associate with lupus like pain and rashes.  These are often most noticeable during flares, and actively attack internal organs.  Type 2 refers to lupus symptoms that are harder to define, and occur even with lower autoimmune activity, like general pain, fatigue, and emotional issues.

By referring to both Type 1 SLE and Type 2 SLE, researchers hope to create a more unified and holistic view of lupus that appropriately values all the different manifestations of lupus.  Ultimately, this could lead to better treatments that respect the true complexity of the disease.

What are type 1 and type 2 lupus?

Both types of lupus involve the immune system attacking its own body, however each does so in a different way and there appears to be biological differences in the kinds of autoantibodies that lead to each type.  More needs to be studied, and there is some overlap in designating symptoms as either Type 1 or Type 2 SLE. However, here are the basics.

Type 1 Lupus:   This refers to the classic symptoms that most people associate with lupus and are the ones often most noticeable during flares.

These include – cutaneous rashes, inflammatory arthritis (joint pain and swelling), alopecia, mouth ulcers, myositis and vasculitis, serositis (inflammation around the heart and/or lungs), lupus nephritis, and other acute symptoms.

• These symptoms are most directly tied to the inflammation of specific tissues and organs damage.
• Because they are acute, they can take up most of the attention of healthcare providers, and understandably, tend to be the target of most therapies.
• Because these symptoms are often relatively easy to measure, they are the focus of most lab tests, biomarkers, and diagnostic tools, such as the SLE Disease Activity Index. They become the basis for most lupus diagnoses and can overshadow other, less measurable symptoms.
• They tend to respond to anti-inflammatory therapies and are the focus of most immunosuppressant drugs.

Type 2 Lupus:  This type of lupus includes symptoms that can be just as common (often more common) and every bit as debilitating as those found in Type 1.  However, they are much more difficult to measure or even define.  Additionally,

They include – fatigue, depression, anxiety, diffuse body pain, difficulty sleeping, brain fog, mood swings and other unspecific cognitive dysfunctions.

• These symptoms are often associated with conditions other than lupus, and so they may not be considered as seriously when it comes to an SLE diagnosis.
• They are often not associated to specific inflammatory reactions, and so they may not respond to standard anti-inflammatory medications or immunosuppressants.
• These symptoms are sometimes considered either nuisances or thought to be caused by lifestyle or environmental issues, not lupus itself. Unfortunately, as the researchers found, these symptoms can be undervalued, or even ignored, by some healthcare providers and patients alike.
• Research has also begun to show that there may some specific autoantibodies, a particular kind of ANA called, anti-RNP antibodies, that may be associated with Type 2 lupus.

Of course, as most people living with lupus know, it is common to experience both types of lupus at different times in life or even at the same time – especially during lupus flares.  For more on the differences between these two faces of lupus, check out our blog-article, Acute vs. Chronic: Living with Lupus.

Why is this new, redefinition of lupus important?

The idea of simply dividing up symptoms this way, may not seem very revolutionary, however there are several advantages to this kind of rethinking about lupus.

Better Communication:  Using the two sub-types helps both patients and healthcare providers organize their thoughts about lupus, so that both can accurately describe all of the symptoms that the person living with lupus faces.  It makes a difference when lupus fog has a place in the conversation as much as ANA levels.

Increased Trust:  Some lupus patients might trust their healthcare providers more when their concerns regarding type 2 symptoms are given value.  For example, a patient may feel that their type 2 lupus is flaring even as their lab tests suggest that their type 1 lupus disease state is low or even in remission.  By valuing both types of lupus, the patient may feel more confident in the assessments of their healthcare providers.

 Improved Treatment Plans:  With increased trust, patients are more likely to adhere to treatment plans.  With a more accurate and holistic view of symptoms, it may be possible to target medications more appropriately and decrease the use of some, like corticosteroids, that may not help their type 2 symptoms.  There may be important changes to treatment plans whether patients are reporting either Persistent or Intermittent Type 2 symptoms.

Improved Research:  The increased acknowledgement of type 2 lupus may encourage more research into finding biomarkers or measurable tests for type 2 symptoms than is taking place now.

The point is to give value and importance to all manifestations of lupus in a way that best defines and describes the disease for patients, healthcare providers and researchers alike!

In Conclusion:  What could this mean for you?

This approach to redefining and describing lupus and all its symptoms is still very new and will take time if it is to be adopted by the lupus community.  However, it has the potential to help patients and medical teams to discuss the many forms of lupus and address its confusing and difficult to measure symptoms.  How?

If you find that it is challenging to discuss, or even think about, your symptoms, it may help to simply ask yourself, “How is my Type 1 lupus and my Type 2 lupus today?”

This may help you identify symptoms that you may have overlooked in the past.

This method may also clarify things enough to give you more confidence in describing your symptoms to your healthcare provider.

It may help those with lupus better approach their chronic longer-term Type 2 SLE symptoms and give healthcare providers a greater appreciation for those as well.  This can be especially, if you can describe whether you are experiencing Persistent or Intermittent Type 2 symptoms to your healthcare provider.

Ultimately, that may lead to better treatments and health outcomes for you!

 References

Colonko, C. (2020). Duke researchers create a type 1, type 2 lupus disease model.  Retrieved from https://www.the-rheumatologist.org/article/duke-researchers-create-a-type-1-type-2-lupus-disease-model/

Eudy, A.M., Rogers, J.L., Corneli, A., McKenna, K., Maheswaranathan, M., Pisetsky, D.S., Criscione-Schreiber, L.G., Doss, J., Sadun, R.E., Sun, K., & Clowse, M.E.B., (2022) Intermittent and Persistent Type 2 lupus: Patient perspectives on two distinct patterns of Type 2 SLE symptoms. Lupus Science and Medicine, 9. Ie000705. DOI: 10.1136/lupus-2022-000705

Pisetsky, D.S., Clowse, M.E.B., Criscione‐Schreiber, L.G., & Roger, J.L. (2018) A novel system to categorize the symptoms of systemic lupus erythematosus. Arthritis Care & Research, 71(6). 735–741. DOI: 10.1002/acr.23794

Pittman, T. (2019) Novel evaluation system helps score and define lupus symptom subtypes. Retrieved from https://physicians.dukehealth.org/articles/novel-evaluation-system-helps-score-and-define-lupus-symptom-sub-types

Author: Greg Dardis, MS

Professor Dardis was formerly the Chair of the Science Department at Marylhurst University and is currently an Assistant Professor at Portland State University.  His focus has been human biology and physiology with an interest in autoimmunity.

All images unless otherwise noted are property of and were created by Kaleidoscope Fighting Lupus. To use one of these images, please contact us at [email protected] for written permission; image credit and link-back must be given to Kaleidoscope Fighting Lupus.

All resources provided by us are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views expressed here do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your healthcare practitioner with any specific questions or concerns.