10/05/2024, 16:48

Autoinflammatory vs.
Autoimmune:
Dysfunction in
Different Immune
Systems
It’s all about innate and adaptive immunity
By Adrienne Dellwo Updated on January 07, 2024

Medically reviewed by David Ozeri, MD

Autoimmune and autoinflammatory diseases all stem from problems in the

immune system, but they’re differentiated by which part of the immune system is
malfunctioning. In autoinflammation, it’s the innate immune system, while it’s the
adaptive immune system that’s involved in autoimmunity. However, some
diseases have aspects of both autoimmunity and autoinflammation.

In order to understand all of this, it helps to understand the innate and adaptive
immune systems and how they act in these diseases. The immune system has
two main functions: keep dangerous things out of your body, and attack and kill
dangerous things that get in.

The Innate Immune System

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Think of your body as a secure facility, and infectious agents as thieves trying to
break in. Your innate immune system is the security that responds to an alarm
and either keeps out or captures the intruders.

As the name suggests, the innate immune system is the one you’re born with.
It’s your first line of defense, designed to recognize and defend you against
broad categories of dangerous things: viruses, bacteria, fungi, parasites, and
other potentially harmful particles. The innate immune system is part “keep it
out” and part “attack and kill.”

To protect you from harm, the innate immune system uses:

Physical barriers: Skin and skin oils, body hair (such as eyelashes and
nose hair), mucous membranes, and the respiratory and digestive tracts all
present challenges to particles trying to make their way into your body.
Defense mechanisms: Some barriers are passive (like hair and skin), while
others have active defense mechanisms, including mucus and tears that
flush things out, sneezing and coughing that forcibly expel harmful
substances, stomach acids that destroy them, and fevers that kill them off
with heat.
General immune response: The body recognizes a foreign invader, tags
invading cells for destruction, and begins destroying them.

Going deeper into the general immune response, once the body detects
something that’s not part of you, it launches a response. A cascade of chemical
signals goes out, telling the immune system that something got in and it needs
to send help and mark invading cells as dangerous.

That help comes in the form of inflammation, which gets extra blood to carry a
host of immune cells to the site. Your capillaries expand, causing the area to
swell, and white blood cells called leukocytes rush in. These leukocytes
immediately set out to consume and kill the invading cells.

You do have several types of leukocytes, including some that are specialized for
bacteria, fungi, parasites, and allergens, and some that kill your cells that have
become infected. The innate immune system’s response is immediate and works
for, on average, about 96 hours (four days) before the adaptive immune system is

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ready to take over.

The Adaptive Immune System

When you hear about vaccines and how they teach your body to fight a
particular pathogen, it’s the adaptive immune system that’s being discussed.
This system learns and adapts as it encounters new intruders, devising
specialized attacks for each specific pathogen it encounters.

So rather than attacking viruses in general, cells of the adaptive immune system
—called antibodies—are highly specialized. An antibody created to attack the
common cold can’t protect you from the flu or COVID-19. You need special
antibodies for that. These are no mere security guards; these are snipers.

The cells involved in adaptive immunity are B-cells and T-cells. These cells don’t
just hunt down and destroy specific invaders, they also remember them so
they’re prepared for the next encounter. Vaccines introduce pathogens or parts
of pathogens into your immune system to create this memory so your body
knows what to do next time that pathogen invades your body.

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Autoinflammatory Autoimmune
Symptoms Symptoms
Inflammation Inflammation

Swollen lymph nodes Swollen lymph nodes

Rash Rash

Recurrent fever Recurrent low-grade

fever
Chills
Pain
Body-wide inflammation
that may cause Fatigue
symptoms based on
Difficulty concentrating
affected organs and
systems Symptoms specific to
what’s being targeted

Autoinflammatory vs. Autoimmunity

Medical science recognized autoimmune diseases well before they did
autoinflammatory diseases. In fact, autoinflammation is still much less
recognized and understood than autoimmunity.

In autoimmune diseases, the adaptive immune system makes a mistake and

determines that a cell type that’s actually “self” is “other.” It then forms
autoantibodies to attack and destroy that type of cell. It may be a liver cell, a
type of brain cell, a blood cell, or just about any type of cell in your body.

More than 100 different autoimmune diseases have been identified, each with its
own unique antibodies. The antibodies’ attack creates inflammation, damage,
and pain. Beyond that, symptoms vary greatly depending on what type of tissue
is under attack.

But some diseases with these symptoms, which were initially assumed to be

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autoimmune, don’t involve autoantibodies. The inflammation is there, but the

adaptive immune system isn’t attacking. Instead, it appears that the innate
immune system triggers the alarm, calling for the cascade of chemicals that lead
to inflammation, and the alarm gets stuck.

Research suggests that much of the time, this is due to genetics—the genes
you’re born with—so these diseases run in families. However, some
autoinflammatory diseases have been discovered that don’t appear to be directly
inherited and instead may stem from somatic mutations—which take place
during your lifetime—that affect innate immune cells.

The primary symptoms of autoinflammatory diseases are inflammation and fever,

as those are part of the innate immune response. Systemic inflammation can
cause numerous other symptoms, depending on where the inflammation is and
which organs or systems it affects.

Disease Spectrum
While the mechanisms of autoinflammation and autoimmunity are different, they
have a lot of overlapping symptoms, genetics, and physiological features. Some
researchers have suggested that these diseases aren’t two separate things but
rather opposite ends of a spectrum, with many diseases featuring a mix of innate
and adaptive dysregulation.

Autoinflammatory-Autoimmune Disease Spectrum

Autoinflammatory Autoimmune

Autoimmune lymphoproliferative
TRAPS*
syndrome

Crohn’s disease IPEX**

Gout Rheumatoid arthritis

Cryopyrin-associated periodic
Type 1 diabetes
syndromes

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Deficiency of IL-1-receptor antagonist Sjogren’s syndrome

Hyper IgD syndrome Lupus

*TNF receptor-associated periodic syndrome **Immunodysregulation

polyendocrinopathy enteropathy X-linked syndrome

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

A Word From Verywell

Autoinflammatory disease is still considered a new category, and the related
illnesses aren’t well understood. You may even find healthcare workers who
aren’t aware of this classification. While autoimmunity has been recognized for
longer and researched considerably more, the medical community still has much
to learn about it.

Ongoing research into both types of diseases and the immune system itself is
likely to bring about a better understanding, increased awareness, better
treatments, and possibly even preventive measures for these potentially
debilitating conditions.

5 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the
facts within our articles. Read our editorial process to learn more about how we fact-check and
keep our content accurate, reliable, and trustworthy.

1. National Institutes of Health, U.S. National Library of Medicine: MedlinePlus. Immune

response.

2. Kaiser G. The Innate Immune System: An Overview. Cantonsville, Community College of

Baltimore Country.

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3. American Autoimmune Related Diseases Association, Inc. There are more than 100
autoimmune diseases.

4. Rheumatology Advisor. Autoinflammatory disorder.

5. Arakelyan A, Nersisyan L, Poghosyan D, et al. Autoimmunity and autoinflammation: a

systems view on signaling pathway dysregulation profiles. PLOS ONE.
2017;12(11):e0187572. doi:10.1371/journal.pone.0187572

Additional Reading

British Society for Immunology. Autoinflammation.

Ciccarelli F, De Martinis M, Ginaldi L. An update on autoinflammatory diseases. Curr Med

Chem. 2014;21(3):261-269. doi:10.2174/09298673113206660303

National Institutes of Health, U.S. National Library of Medicine: MedlinePlus. Autoimmune

diseases.

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