Autoimmune v. Autoinflammatory: The Verdict

 


In those early days as an advocate for the PRP community, the rank and file (patients and caregivers) couldn’t care less if pityriasis rubra pilaris was an autoimmune or an autoinflammatory disease.

I should have known I was in trouble when I first asked Drs. Google and Yahoo.

“Autoinflammatory disorders are immune-mediated diseases with increased production of inflammatory cytokines and absence of detectable autoantibodies. They course with recurrent episodes of systemic inflammation and fever is the most common symptom. Cutaneous manifestations are prevalent and important to diagnosis and early treatment of the syndromes.”

Beat me over the head with a shovel. Yet. it seemed to me that the PRP Survival Guide needed a cogent answer to the question. 

With that in mind I first approached the American Autoimmune Related Diseases Association (AARDA) in 2015. Should the PRP Alliance become a member organization?

To my disappointment, PRP wasn’t on their list of autoimmune diseases (and still isn’t). We are not between pernicious anemia and POEM syndrome. I say, “Balderdash!”

My next stop was the Autoinflammatory Alliance, a non-profit organization dedicated to promoting awareness, proper diagnosis and treatment, and improved care for people with autoinflammatory diseases. Our kind of people, I thought. Maybe we would be on their list.

Not only was pityriasis rubra pilaris not on their list, but the message “Your search for pityriasis rubra pilaris returned 0 results” was the death knell. I can take a hint.

That’s when I decided to ask my own dermatologist. BINGO. Then other PRP patients and caregivers asked their dermatologists. Again, BINGO! BINGO! 

Call it a consensus or a verdict, but from the perspective of the PRP global community, pityriasis rubra pilaris is an autoinflammatory disease. Take it to the bank?

— Editor


Understanding Autoinflammatory Diseases

The Genetic and Rare Diseases Information Center once again provides patient-friendly information. Their “coverage” of autoinflammatory Diseases meets that standard. The following information is dated January 2017. If you REALLY want to know about autoinflammatory diseases, this is worth a cup of coffee and some “Quiet Time”.  FOLLOW LINK


AARDA: PRP is not an autoimmune disease

“The American Autoimmune Related Diseases Association is dedicated to the eradication of autoimmune diseases and the alleviation of suffering and the socioeconomic impact of autoimmunity through fostering and facilitating collaboration in the areas of education, public awareness, research, and patient services in an effective, ethical, and efficient manner.


Autoinflammatory Alliance

The Autoinflammatory Alliance is a 501 (c)(3) non-profit public charity dedicated to promoting awareness, proper diagnosis and treatment, and improved care for people with autoinflammatory diseases. Read more about this “Alliance”.  FOLLOW LINK


NIAMS

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS).
Understanding autoinflammatory diseases. https://www.niams.nih.gov/Health_Info/Autoinflammatory/default.asp January 2017. Accessed August 7, 2017.


Autoimmune or autoinflammatory?

Russ D, (Luton, Bedfordshire, England) posted the following om April 20, 2020: “…Could anyone confirm is PRP is an autoimmune disease as some sources say it is and others say its not. The reason I ask is with the COVID-19 pandemic on us and as both myself and other half are classified as” key workers”,  I”m a little worried about my other half returning to work in a school. Should I be worried?”

This post generated 69 comments, most addressing the question: Is PRP and autoimmune or autoinflammatory disease. CLICK HERE for the unabridged version. You must be a member to access.


Autoinflammatory vs autoimmune
What is the difference? Why does it Matter?

To the patients who suffer from very similar symptoms, such as inflamed and painful joints, the differences of autoinflammatory vs. autoimmune may not matter to them. They all want and need good treatment. However, the common treatments for autoimmune conditions may not work well for autoinflammatory conditions because the cause is different. To best treat each condition, researchers need to understand the cause and target that specific mechanism of the innate immune system that is overactive to help develop the best treatment. Even amongst the different systemic autoinflammatory diseases, different treatments work best for different fever syndromes because different parts of the innate immune system are overactive or not controlled. While interleukin-1ß inhibitors may work very well in some fever syndromes, TNF inhibitors work better in others; but for other conditions, neither of these types of drugs may be the best choice. Also, understanding the basic mechanisms of autoinflammatory diseases helps researchers and patients understand possible complications of the disease that may differ from autoimmune conditions. LEARN MORE


Classical Autoinflammatory Disorder Definition.

The autoinflammatory disorders are a group of diseases that, in their purest forms, manifest as recurrent fevers, high acute phase responses and a proclivity for inflammation of skin, joints, serosal surfaces and other organ involvement including the nervous system. These disorders lack the classical characteristics of autoimmune diseases such as high-titer autoantibodies. The innate immune system plays the primary pathophysiologic role in autoinflammatory diseases and in fact, these diseases are synonymous with dysregulation of the cells and molecular cascades intrinsic to innate immunity. Had this been appreciated at the outset these diseases could well have been designated as “innate autoimmunity” in contradistinction to “adaptive autoimmunity”. LEARN MORE


Many Autoinflammatory Diseases Have a Range of Severity

Autoinflammatory diseases are caused by genetic mutations in molecules that are involved in regulating the innate immune response-a “hard wired” defense system that evolved to quickly recognize and act against infectious agents and other danger signals produced by our bodies.

It is important not to confuse autoinflammatory syndromes with autoimmune diseases, such as: Lupus, Rheumatoid Arthritis and others that are caused by the body’s adaptive immune system developing antibodies to antigens that then attack healthy body tissues.

Autoinflammatory diseases are not contagious. When patients are having flares of their disease symptoms they cannot infect anyone, even if they are having a fever, rash, non-infectious conjunctivitis, vomiting, diarrhea or other symptoms that can be confused as an illness. These symptoms are triggered by the autoinflammatory disease, which in most cases are caused by genetic mutations. It is important to distinguish disease flares from infectious diseases.