Autoinflammatory vs. Autoimmune: What is the Difference? (2022)

The periodic fever syndromes fall into the autoinflammatory disease category. These conditions include familial Mediterranean fever (FMF), cryopyrin associated periodic syndrome (CAPS), hyperimmunoglobulinemia D syndrome (HIDS), TNF receptor-associated periodic syndrome (TRAPS), and several other chronic diseases that have recurring fevers and inflammation as the main symptoms. As researchers learn more about these conditions, more diseases that were once thought to be autoimmune are now being considered autoinflammatory. Recent research has placed some diseases that were previously known as autoimmune diseases, such as systemic-onset juvenile idiopathic arthritis (SJIA, also known as soJIA), Sweet’s syndrome, and Behçet’s disease, into the autoinflammatory category or as having autoinflammatory components.

But what’s the difference between autoimmune diseases and autoinflammatory diseases? And does it matter?

The Cliff Notes
Autoinflammatory = Malfunction in the Innate Immune System
Autoimmune = Malfunction in the Adaptive Immune System

Both autoinmmune and autoinflammatory diseases do have an immune system malfunction as the underlying cause of the symptoms. And both share some of the same symptoms, such as joint pain and swelling, rash, and fatigue. However, the underlying cause or mechanism of the diseases are different. This difference affects treatment options, long-term health risks, and possible complications from the systemic inflammation.

The Acquired Immune System and the Innate Immune System

There are two main parts to the immune system, the acquired, or adaptive immune system, and the innate immune system. The acquired immune system learns throughout a person’s life what pathogens to attack. Once exposed to a pathogen, such as a flu virus, the acquired immune system learns from it and remembers it. When encountered again, it makes antibodies to attack it. Targets of the adaptive immune system are specific.

The body’s innate immune system is more primitive, according to the National Institutes of Health. The innate immune system uses white blood cells and acute inflammation to attack pathogens. The innate immune system may be activated by triggers, but in some autoinflammatory diseases, the genetic mutation causing the disease makes certain danger sensors (known as NLRs, NOD-like receptors) in cells to become frequently, or even continuously activated. These activated molecules in the cell join with other molecules to form an inflammasome that coordinates a strong innate immune system response. Fever is a primary symptom of an activated innate immune system. This chronic activation of the innate immune system can lead to systemic inflammation that occurs throughout the body.

In autoimmune diseases, the adaptive immune system produces antibodies that target and attack specific parts of the body. pseudolongino/Bigstockphoto.com

Autoimmune Diseases – A Dysfunction in the Adaptive or Acquired Immune System

In short, in autoimmune diseases, the adaptive or acquired part of the immune system has mistakenly identified something specific in the body as harmful and attacks it causing symptoms. The initial inflammation occurs mostly at the location in the body that the autoantibodies are targeting, but it can progress to other parts of the body.

(Video) Autoimmune vs. Autoinflammatory

Normally an immune system’s white blood cells produce antibodies to attack illnesses that would harm the body. The targets of these antibodies include viruses, bacteria, parasites, and even cancer cells. In autoimmune diseases, the body produces antibodies that essentially attack different parts of the body. For example in Grave’s disease, the body produces antibodies that target the thyroid causing the thyroid to produce too much hormone. In rheumatoid arthritis, the antibodies attack the lining surrounding the joints causing inflammation. Allergies are also considered an autoimmune disorder because the body is attacking substances that it should ignore, the proteins of plant pollens or certain foods.

In some cases, once these antibodies become active the innate immune system may also become active, causing inflammation throughout the body in response to the antibodies fighting off a perceived infection. This innate immune system acts as back up to fight off the infection.

Autoimmune diseases overall are rather common, affecting about 23.5 million Americans according to WomensHealth.gov. Some individual autoimmune diseases are quite rare while others are considered common. The causes of autoimmune diseases can vary. Some are hereditary, some are triggered by another illness or environmental factors, and some are caused by a combination of factors. More women than men are affected by autoimmune conditions.

Because autoimmune conditions are common, there is more research, knowledge, and medications available to treat these conditions.

In autoinflammatory diseases, the innate immune systems reacts often without cause. A primary tool of the innate immune system is fever. AlexWhite/Bigstockphoto.com

Autoinflammatory Diseases – A Dysfunction of the Innate Immune System

In short, in systemic autoinflammatory diseases, the innate part of the immune systems reacts often without cause and without control. Sometimes there may be an injury or a real virus or other pathogen that triggers the innate immune system, but the reaction is not well controlled and is over reactive. The inflammation is often general and not targeted to one area of the body, meaning that from the start, the inflammation can occur anywhere in the body and is not limited to a specific region. Fever, being one tool of the innate immune system to fight infection, becomes the most common symptom of many systemic autoinflammatory diseases. Muscles, joints, skin, the gastrointestinal system, and internal organs, can also all be affected by this inflammation simultaneously.

Because it’s the innate immune system that overreacts first, not the acquired or adaptive immune system, in most patients with autoinflammatory conditions, diagnostic autoantibody tests are usually negative. The innate immune system is reacting without antibodies to trigger it. In autoinflammatory conditions, the genetic mutation causing disease leads to activation of the innate immune system, leading to the dysregulation of inflammatory cytokines, such as interleukin-1 and TNF-alpha and others that are over produced, rather than autoantibodies driving the inflammation.

Many autoinflammatory diseases have been identified as being caused by genetic mutations. A mis-spelling is another term for this sort of mutation, since some of the autosomal dominant hereditary autoinflammatory diseases (such as CAPS) are due to one amino acid in the DNA taking the place of the standard amino acid for that area of the genetic code. Genetic tests are now available to help to aid in the diagnosis for a number of autoinflammatory diseases, but there still can be mutation-negative cases with full clinical symptoms of the disease, so genetic testing is just one tool in the diagnostic process. With the autosomal dominance of some of these diseases, the mutation occurred somewhere in the family lineage, so patients may have a parent and grandparents with the same condition.

(Video) Ask Dr Kan Show #171 - Autoimmune vs Autoinflammatory diseases

However, some patients may also have an autosomal dominant mutation that happened spontaneously, at some point in their conception, or in the first cells in a fertilized egg. Some diseases follow an autosomal recessive inheritance, where each parent must pass on the mutation to cause disease. In recessive conditions, rarely does a family history include that disease, except in some diseases like familial Mediterranean fever that the recessive trait is carried in the DNA of 1 out of 5 people from certain ethnicities. In some cases, the genetics are not that simple or completely understood.

Autoinflammatory conditions are very rare diseases. The most common is thought to be periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) but there are more rare ones, such as CANDLE syndrome, are only identified in a handful of patients around the world. Because of the rarity of these conditions, knowledge is usually limited about these diseases, and so are treatment options. They can be more difficult to diagnose because the long list of symptoms may seem at first to be unrelated. For example, in Muckle-Wells syndrome (MWS)–a form of CAPS, doctors and patients may not recognize that hearing problems, recurring fever, and skin rashes are part of the same syndrome.

Why Does the Difference 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.

The Autoinflammatory Alliance is a 501(c)(3) non-profit organization dedicated to helping those with autoinflammatory diseases.

Donate now to help with awareness, education, and research for these rare diseases.

*Healthy T-cell photo by NIAID, Flickr

References

  1. National Institutes of Health: Understanding Autoinflammatory Diseases
  2. Dermnet NZ: Autoinflammatory syndromes
  3. MedlinePlus: Autoimmune disorders
  4. WomensHealth.gov: Autoimmune diseases fact sheet
  5. University of South Carolina School of Medicine: Innate (non-Specific) Immunity
  6. U.S. National Library of Medicine National Institutes of Health: Autoinflammatory syndromes: diagnosis and management
  7. PubMed.gov: Autoinflammatory syndromes
  8. Science Direct: Autoinflammation and autoimmunity: Bridging the divide
  9. Immune Regulation Research Group, and Immunology Research Centre, School of Biochemistry and Immunology, Trinity College Dublin 2, Ireland: IL-1 – Master mediator or initiator of inflammation
  10. European PubMed Central: Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease
  11. National Institutes of Health: Periodic fever syndrome and autoinflammatory diseases
  12. National Institutes of Health: Immunology in clinic review series; focus on autoinflammatory diseases: update on monogenic autoinflammatory diseases: the role of interleukin (IL)-1 and an emerging role for cytokines beyond IL-1
  13. The Autoinflammatory Alliance: CAPS: Cryopyrin-Associated Periodic Syndromes
  14. Learning About Familial Mediterranean Fever
  15. The inflammasome-A Linebacker of innate defense
  16. Inflammasome and innate immune system discussion from the Autoinflammatory Alliance CAPS guidebook, written by leading experts.
  17. NOD-like Receptors Review
  18. Periodic Fever: A Review on Clinical, Management and Guideline for Iranian Patients – Part II
  19. Expression of cytokines, chemokines and other effector molecules in two prototypic autoinflammatory skin diseases, pyoderma gangrenosum and Sweet’s syndrome.
  20. Amyloidosis Patient Information Site: The inherited periodic fever syndromes – general information
  21. Hindawi: Systemic Arthritis in Children: A Review of Clinical Presentation and Treatment

More for You

Normal Body Temperature and the Periodic Fever Syndromes

PFAPA vs. HIDS

Get Your "Rare Wonder" Limited Edition Custom Photo Ornament-Donate Today!

(Video) The Diagnosis and Management of Autoinflammatory and Hyperinflammatory Conditions | UPMC Children's
(Video) Autoimmune vs. Autoinflammatory Diseases

What are Undifferentiated Autoinflammatory Diseases?

What is the Most Accurate Way to Take a Temperature?

FAQs

Is autoinflammatory and autoimmune the same thing? ›

Autoimmune diseases involve the adaptive immune system, while autoinflammatory diseases involve the innate immune system.

Is inflammatory disease the same as autoimmune disease? ›

Autoinflammatory and autoimmune diseases both cause inflammation directed against your own body. Disorders of the innate immune system cause autoinflammatory disease. By contrast, disorders of the adaptive immune system cause autoimmune disease.

What are the 4 types of autoimmune diseases? ›

In an autoimmune disease, the immune system mistakes part of your body, like your joints or skin, as foreign.
...
Here are 14 of the most common ones.
  • Type 1 diabetes. ...
  • Rheumatoid arthritis (RA) ...
  • Psoriasis/psoriatic arthritis. ...
  • Multiple sclerosis. ...
  • Systemic lupus erythematosus (SLE)

What does autoinflammatory mean? ›

Autoinflammatory diseases refer to problems with the innate immune system's reactions. Immune cells target the body's own healthy tissues by mistake, signaling the body to attack them. This can cause intense episodes of inflammation that result in such symptoms as fever, rash, or joint swelling.

Are all autoimmune diseases inflammatory? ›

Inflammatory disease is a general term that applies to autoimmune diseases and chronic inflammation conditions in which a person's immune system attacks the body's own tissues. Familiar inflammatory diseases include rheumatoid arthritis, systemic lupus erythematosus, inflammatory bowel disease, and multiple sclerosis.

Is rheumatoid arthritis autoimmune or autoinflammatory? ›

At the population level, rheumatoid arthritis (RA) is generally viewed as autoimmune in nature with a small subgroup of cases having a palindromic form or systemic autoinflammatory disorder (SAID) phenotype.

What are the 7 autoimmune diseases? ›

Common autoimmune disorders include:
  • Addison disease.
  • Celiac disease - sprue (gluten-sensitive enteropathy)
  • Dermatomyositis.
  • Graves disease.
  • Hashimoto thyroiditis.
  • Multiple sclerosis.
  • Myasthenia gravis.
  • Pernicious anemia.
24 Apr 2021

What are some examples of inflammatory diseases? ›

Some common inflammatory diseases
  • Fatty liver disease. Fatty liver disease can be caused by poor diet, which can set off an inflammatory response. ...
  • Endometriosis. ...
  • Type 2 diabetes mellitus. ...
  • Type 1 diabetes mellitus. ...
  • Inflammatory bowel disease (IBD) ...
  • Asthma. ...
  • Rheumatoid arthritis. ...
  • Obesity.

What is the most common autoimmune disease? ›

Nearly 4% of the world's population is affected by one of more than 80 different autoimmune diseases, the most common of which include type 1 diabetes, multiple sclerosis, rheumatoid arthritis, lupus, Crohn's disease, psoriasis and scleroderma.

What are the most serious autoimmune diseases? ›

Four of the most frequently fatal ones include:
  • Giant cell myocarditis.
  • Anti-NMDA receptor encephalitis.
  • Mixed connective tissue disease.
  • Autoimmune vasculitis.

What are 5 common symptoms of an autoimmune disorder? ›

Common symptoms of autoimmune disease include:
  • Fatigue.
  • Joint pain and swelling.
  • Skin problems.
  • Abdominal pain or digestive issues.
  • Recurring fever.
  • Swollen glands.

What can trigger autoimmune disease? ›

What causes autoimmune diseases?
  • Some medications. ...
  • Having relatives with autoimmune diseases. ...
  • Smoking.
  • Already having one autoimmune disease. ...
  • Exposure to toxins.
  • Being female — 78% of people who have an autoimmune disease are women.
  • Obesity.
  • Infections.
21 Jul 2021

How do you test for autoinflammatory disease? ›

Genetic testing methods for autoinflammatory diseases
  1. Sanger sequencing. Sanger sequencing still constitutes the mainstay of molecular testing for most SAIDs. ...
  2. NGS approaches. ...
  3. NGS gene panels. ...
  4. Whole exome sequencing. ...
  5. Whole genome sequencing.
26 Nov 2019

What are the 5 classic signs of inflammation? ›

Based on visual observation, the ancients characterised inflammation by five cardinal signs, namely redness (rubor), swelling (tumour), heat (calor; only applicable to the body' extremities), pain (dolor) and loss of function (functio laesa).

Who treats autoinflammatory disease? ›

Rheumatologists evaluate and treat autoimmune, inflammatory or other musculoskeletal conditions like: Rheumatoid arthritis. Systemic lupus erythematosus. Systemic sclerosis (scleroderma)

How do you stop autoimmune inflammation? ›

Use nutrients such as fish oil, vitamin C, vitamin D, and probiotics to help calm your immune response naturally. Exercise regularly — it's a natural anti-inflammatory. Practice deep relaxation like yoga, deep breathing, biofeedback, or massage, because stress worsens the immune response.

How do I know if I have an inflammatory disease? ›

Classic signs of inflammation include fatigue, fever, and joint and muscle pain. Inflammation is also known for causing symptoms that are considered atypical. This can include things like balance issues, insulin resistance, muscle weakness, eye problems, skin issues, and more.

Is Crohn's autoimmune or autoinflammatory? ›

Categorizing Crohn's Disease

In the past, it has been referred to as an autoimmune condition. But it is now understood that it is not autoimmune because it lacks the conditions that need to be met for that classification. Crohn's disease is more accurately described as autoinflammatory.

Which arthritis is an autoimmune inflammatory disease? ›

Rheumatoid arthritis, or RA, is an autoimmune and inflammatory disease, which means that your immune system attacks healthy cells in your body by mistake, causing inflammation (painful swelling) in the affected parts of the body. RA mainly attacks the joints, usually many joints at once.

Are autoinflammatory diseases rare? ›

Autoinflammatory diseases are a group of rare diseases characterised by seemingly unprovoked episodes of fever and inflammation. Because the inflammatory episodes occur regularly, the diseases are also known as 'periodic fever syndromes.

Is osteoarthritis an autoinflammatory disease? ›

Osteoarthritis is not an autoimmune disease, and although the exact causes are not known, multiple risk factors have been identified. In a healthy joint, cartilage provides cushioning and a smooth joint surface for motion.

What is the test for autoimmune disease? ›

The antinuclear antibody (ANA) immunofluorescence assay (IFA) is a first-line screening test for patients with a suspected autoimmune disease. This test is the gold standard because of its high sensitivity compared to other assays.

Can you reset your immune system? ›

Six years ago, a study showed that a 3-day fast can essentially reset the immune system, providing many potential benefits. These benefits include better cardiovascular health, better endurance, lower blood pressure, and reduced inflammation.

Can autoimmune disease go away? ›

Autoimmune disorders in general cannot be cured, but the condition can be controlled in many cases. Historically, treatments include: anti-inflammatory drugs – to reduce inflammation and pain. corticosteroids – to reduce inflammation.

What are 3 diseases linked with chronic inflammation? ›

Research has shown that chronic inflammation is associated with heart disease, diabetes, cancer, arthritis, and bowel diseases like Crohn's disease and ulcerative colitis.

What are the 3 main causes of inflammation? ›

These are the most common: Pathogens (germs) like bacteria, viruses or fungi. External injuries like scrapes or damage through foreign objects (for example a thorn in your finger) Effects of chemicals or radiation.

What are 5 health issues linked to inflammation? ›

But inflammation has also emerged as a key factor in serious diseases, such as cardiovascular disease, cancer, Alzheimer's disease, obesity, diabetes, and a variety of infectious diseases including HIV/AIDS.

Can vitamin D reverse autoimmune disease? ›

In a new study, investigators from Brigham and Women's Hospital found the people who took vitamin D, or vitamin D and omega-3 fatty acids, had a significantly lower rate of autoimmune diseases — such as rheumatoid arthritis, polymyalgia rheumatica, autoimmune thyroid disease, and psoriasis — than people who took a ...

Which blood type has more autoimmune diseases? ›

The ratio of people with blood type O was found to be higher among those with Hashimoto's than in people with other thyroid diseases. They also found that autoimmune diseases were reported significantly less often in people with blood type AB.

Who suffers more from autoimmune disease? ›

Did you know that women are much more likely to be afflicted with an autoimmune disease than men? An estimated 80% of those with autoimmune diseases are women. Sadly, autoimmune diseases are one of the leading causes of death and disability in girls and women ages 65 and younger.

Can you have 2 autoimmune diseases? ›

Disorders of an autoimmune nature are known to occur with increased frequency in patients with another autoimmune disease. About 25 percent of patients with autoimmune diseases have a tendency to develop additional autoimmune disorders (3).

Why is autoimmune so common now? ›

Internationally, it is now estimated that cases of autoimmune diseases are rising by between 3% and 9% a year. Most scientists believe environmental factors play a key role in this rise. “Human genetics hasn't altered over the past few decades,” said Lee, who was previously based at Cambridge University.

Do autoimmune diseases get worse with age? ›

Older persons have higher autoimmunity but a lower prevalence of autoimmune diseases. A possible explanation for this is the expansion of many protective regulatory mechanisms highly characteristic in the elderly. Of note is the higher production of peripheral T-regulatory cells.

What kind of doctor treats autoimmune diseases? ›

Rheumatologist. A rheumatologist treats arthritis and other rheumatic diseases, autoimmune and inflammatory diseases that can cause your immune system to attack its joints, muscles, bones, or organs.

What organs are affected by autoimmune disorder? ›

Areas often affected by autoimmune disorders include:
  • Blood vessels.
  • Connective tissues.
  • Endocrine glands such as the thyroid or pancreas.
  • Joints.
  • Muscles.
  • Red blood cells.
  • Skin.

What does an autoimmune flare up feel like? ›

Many types of autoimmune diseases cause redness, swelling, heat, and pain, which are the signs and symptoms of inflammation. But other illnesses can cause the same symptoms. The symptoms of autoimmune diseases can come and go. During a flare-up, your symptoms may get severe for a while.

What kind of stress causes autoimmune disease? ›

Psychological stress is reported to be a risk factor for autoimmune diseases, including Graves' disease and rheumatoid arthritis.

What food calms immune system? ›

Choose Calming Foods

Fruits and vegetables (aim for a broad rainbow of colors to get the most antioxidant variety), fish and fish oil, olive oil, ground flaxseeds, and spices like ginger, rosemary, basil and turmeric can all have a quieting effect on an overactive immune system.

Can you suddenly get an autoimmune disease? ›

For many autoimmune diseases, symptoms come and go, or can be mild sometimes and severe at others. When symptoms go away for a while, it's called remission. Flares are the sudden and severe onset of symptoms.

Is rheumatoid arthritis an autoinflammatory disease? ›

What is rheumatoid arthritis (RA)? Rheumatoid arthritis, or RA, is an autoimmune and inflammatory disease, which means that your immune system attacks healthy cells in your body by mistake, causing inflammation (painful swelling) in the affected parts of the body.

What is considered an inflammatory disease? ›

Inflammation disease is when the immune system attacks the body's own tissues, resulting in inflammation. Rheumatoid arthritis is an example of an inflammatory disease.

Is Lupus an autoinflammatory disease? ›

Systemic lupus erythematosus (SLE), is the most common type of lupus. SLE is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels.

Is Crohns autoimmune or autoinflammatory? ›

Categorizing Crohn's Disease

In the past, it has been referred to as an autoimmune condition. But it is now understood that it is not autoimmune because it lacks the conditions that need to be met for that classification. Crohn's disease is more accurately described as autoinflammatory.

Is autoinflammatory curable? ›

Autoinflammatory diseases cannot be cured, and treatment is usually to relieve symptoms during an attack. Patients are often treated with high doses of corticosteroids, a broad-brush approach to suppress the immune system.

Are autoinflammatory diseases curable? ›

Autoimmune disorders in general cannot be cured, but the condition can be controlled in many cases. Historically, treatments include: anti-inflammatory drugs – to reduce inflammation and pain. corticosteroids – to reduce inflammation.

What is lupus called now? ›

Systemic lupus erythematosus (SLE), the most common form of lupus.

Videos

1. Autoimmune & Autoinflammatory Disease and Immune Mediated Hearing Loss
(House Institute Professional Education)
2. Autoinflammatory Syndromes
(Learning in 10)
3. The Autoinflammatory Syndromes: All Who Fever Are Not Infected
(ClevelandClinicCME)
4. Is Hidradenitis Suppurativa (HS) Autoimmune or Autoinflammatory with Dr. Kirby
(HS Connect)
5. What causes Inflammation and Autoimmune disease?
(Cook 2 Nourish)
6. Human Kinome as Target for Autoimmune and Autoinflammatory Disease
(ClevelandClinicCME)

Top Articles

Latest Posts

Article information

Author: Annamae Dooley

Last Updated: 10/15/2022

Views: 6406

Rating: 4.4 / 5 (45 voted)

Reviews: 84% of readers found this page helpful

Author information

Name: Annamae Dooley

Birthday: 2001-07-26

Address: 9687 Tambra Meadow, Bradleyhaven, TN 53219

Phone: +9316045904039

Job: Future Coordinator

Hobby: Archery, Couponing, Poi, Kite flying, Knitting, Rappelling, Baseball

Introduction: My name is Annamae Dooley, I am a witty, quaint, lovely, clever, rich, sparkling, powerful person who loves writing and wants to share my knowledge and understanding with you.