Immunity is the ability to resist infection by an invading pathogen.

The body quickly

launches an immune response and prevents the symptoms of disease occurring.
This can happen in two ways naturally or artificially.

Types of immunity

Types of immunity
Immunity is the ability to resist infection by an invading pathogen. The body quickly launches
an immune response and prevents the symptoms of disease occurring. This can happen in
two ways naturally or artificially. Natural immunity

occurs without human intervention

and artificial immunity

occurs when antigens or antibodies are given to a person by
artificial means, eg by injection.

Both natural and artificial immunity can be either

passive or active.
Passive immunity is when a person is given antibodies produced by someone else. This could
happen naturally when a mother passes her own antibodies to her baby either through her
placenta or her breast milk. Another method of gaining passive immunity is artificially, for
example when a person is given an injection of antibodies if they suspect that they have been
exposed to a disease such as tetanus or diphtheria. In this situation, immunity is established
immediately. This form of immunity is short lived as no memory cells are produced.
Longer-term immunity is gained via active immunity. Examples of this involve the body being
stimulated to produce antibodies via a specific immune response. This occurs either by a
person contracting a disease which is referred to as natural active immunity or via an
injection of weakened (attenuated) or dead antigens. In this case an immune response is
activated resulting in the production of antibodies and memory cells. This latter form of
immunity is called artificial active immunity. The principle of immunisation is based on this.
Below is a graph showing the results of an initial immunisation and a booster injection. The
first injection produces an initial supply of antibodies but the booster injection produces a
larger and faster response.

Search Results

1117 378 - courses.lumenlearning.net

urmounting Tumor-induced Immune Suppression by Frequent

Vaccination or Immunization in the Absence of B Cells
Oizumi, Satoshi* ; Deyev, Vadim* ; Yamazaki, Koichi* ; Schreiber, Taylor* ; Strbo, Natasa* ; Rosenblatt,
Joseph* ; Podack, Eckhard R.*

Abstract
Tumor-induced immune suppression is one of the most difficult obstacles to the success of tumor
immunotherapy. Here, we show that established tumors suppress CD8 T cell clonal expansion in vivo,
which is normally observed in tumor-free mice upon antigen-specific glycoprotein (gp) 96-chaperone
vaccination. Suppression of CD8 T-cell expansion by established tumors is independent of tumorassociated expression of the antigen that is recognized by the CD8T-cell receptor. Vaccination of
tumor-bearing mice is associated with increased cellular recruitment to the vaccine site compared with
tumor-free mice. However, rejection of established, suppressive tumors required frequent (daily) gp96
vaccination. B cells are known to attenuate T helper cell-1 responses. We found that in B-cell deficient
mice, tumor rejection of established tumors can be achieved by a single vaccination. Accordingly, in
tumor-free B-cell deficient mice, cognate CD8 cytotoxic T lymphocyte clonal expansion is enhanced in
response to gp96-chaperone vaccination. The data have implications for the study of tumor-induced
immune suppression and for translation of tumor immunotherapy into the clinical setting. Frequent
vaccination with cellular vaccines and concurrent B-cell depletion may greatly enhance the activity of
anticancer vaccine therapy in patients.

urmounting Tumor-induced Immune Suppression by Frequent

Vaccination or Immunization in the Absence of B Cells
Oizumi, Satoshi* ; Deyev, Vadim* ; Yamazaki, Koichi* ; Schreiber, Taylor* ; Strbo, Natasa* ; Rosenblatt,
Joseph* ; Podack, Eckhard R.*

Abstract
Tumor-induced immune suppression is one of the most difficult obstacles to the success of tumor
immunotherapy. Here, we show that established tumors suppress CD8 T cell clonal expansion in vivo,
which is normally observed in tumor-free mice upon antigen-specific glycoprotein (gp) 96-chaperone
vaccination. Suppression of CD8 T-cell expansion by established tumors is independent of tumorassociated expression of the antigen that is recognized by the CD8T-cell receptor. Vaccination of
tumor-bearing mice is associated with increased cellular recruitment to the vaccine site compared with
tumor-free mice. However, rejection of established, suppressive tumors required frequent (daily) gp96
vaccination. B cells are known to attenuate T helper cell-1 responses. We found that in B-cell deficient
mice, tumor rejection of established tumors can be achieved by a single vaccination. Accordingly, in
tumor-free B-cell deficient mice, cognate CD8 cytotoxic T lymphocyte clonal expansion is enhanced in
response to gp96-chaperone vaccination. The data have implications for the study of tumor-induced
immune suppression and for translation of tumor immunotherapy into the clinical setting. Frequent
vaccination with cellular vaccines and concurrent B-cell depletion may greatly enhance the activity of
anticancer vaccine therapy in patients.

Allergy (Allergies)

1.
2.
3.

Common Allergy Triggers Slideshow Pictures

Take the Quiz on Allergies
Allergy Proof Your Home Slideshow

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Table of Contents

Allergy facts

Allergy involves an exaggerated response of the immune

system, often to common substances such as foods
or pollen.

The immune system is a complex system that normally

defends the body against foreign invaders, such as
bacteria and viruses, while also surveying for abnormal
tissue changes, such as cancer.

Allergens are substances that are foreign to the body and

that cause an allergic reaction.

IgE is the allergic antibody.

Although many individuals outgrow allergies over

time, allergies can also develop at any age, including
during adulthood.

While the environment plays a role in the development

of allergy, there is a greater risk of developing allergic
conditions if a person has a family history of allergy,
especially in parents or siblings.

Allergy overview
This is a review regarding how the allergic response of the immune
system occurs and why certain people become allergic. The most
common allergic diseases are described, including allergic
rhinitis (nasal allergies), allergic conjunctivitis (eye allergies),
allergic asthma, urticaria (hives), and food allergies. Continue Reading
1/18
Reviewed on 4/18/2016

NEXT:What is an allergy?

Medical Author:

Allison Ramsey, MD
Coauthor:

Syed Shahzad Mustafa, MD

Medical Editor:

William C. Shiel Jr., MD, FACP, FACR

References

TOP 10 Allergy Related Articles

1.

2.

Asthma

Atopic Dermatitis

3.

Bugs & Bites Slideshow

4.

Breastfeeding

5.

Canker Sores

6.

7.

8.

Colic

Human Body Quiz

Pneumococcal Vaccination

9.

Poison Ivy Picture

10.

Sinus Surgery
11.

Complete List

Patient Comments
Viewers share their comments

Allergies - Describe Your ExperiencePlease describe your experience with

allergies.

View 55 Comments

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allergies?

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allergic to?

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An autoimmune disease is a pathological state arising from an abnormal immune response of

the body to substances and tissues that are normally present in the body.
Autoimmunity, on the other hand, is the presence of self-reactive immune response (e.g., autoantibodies, self-reactive T-cells), with or without damage or pathology resulting from it. [1] This may
be restricted to certain organs (e.g. inautoimmune thyroiditis) or involve a particular tissue in
different places (e.g. Goodpasture's disease which may affect the basement membrane in both
the lung and the kidney).
The treatment of autoimmune diseases is typically with immunosuppressionmedication that
decreases the immune response. New novel treatments include Cytokine blockade (or the
blockade of cytokine signaling pathways), removal of effector T-cells and B-cells (e.g. anti-CD20
therapy can be effective at removing instigating B-cells).[1] Intravenous Immunoglobulin has been
helpful in treating some antibody mediated autoimmune diseases as well, possibly through
negative feedback mechanisms.[2]
A large number of autoimmune diseases are recognized. A major understanding of the
underlying pathophysiology of autoimmune diseases has been the application of genome wide
association scans that have identified a striking degree of genetic sharing among the
autoimmune diseases.[3]
Contents
[hide]

1Definition

2Effects

3Pathophysiology
o

3.1Cryptic determinants/molecular sequestration

3.2Molecular mimicry

3.3Altered glycan theory

4List by category
o

4.1Autoimmune Diseases

4.2Autoimmune Comorbidities

4.3Not Autoimmune

5Development of therapies

6See also

7References

8Further reading

9External links

Definition[edit]
For a disease to be regarded as an autoimmune disease it needs to answer to Witebsky's
postulates (first formulated by Ernest Perez and colleagues in 1957 and modified in 1994):[4][5]

Direct evidence from transfer of disease-causing antibody or disease-causing T

lymphocyte white blood cells

Indirect evidence based on reproduction of the autoimmune disease in experimental

animals

Circumstantial evidence from clinical clues

Genetic evidence suggesting "clustering" with other autoimmune diseases

Autoimmune diseases are incurable

Effects[edit]
Autoimmune diseases have a wide variety of different effects. They do tend to have one of three
characteristic pathological effects which characterize them as autoimmune diseases: [6]
1. Damage to or destruction of tissues
2. Altered organ growth
3. Altered organ function
It has been estimated that autoimmune diseases are among the leading causes of death among
women in the United States in all age groups up to 65 years. [7]

A substantial minority of the population suffers from these diseases, which are often chronic,
debilitating, and life-threatening.[citation needed]
There are more than 80 illnesses caused by autoimmunity.[8] Autoimmune diseases affect
approximately 2-5% of the western world's population. Women are found to be more commonly
affected than men. Environmental events can trigger some cases of autoimmune diseases such
as exposure to radiation or certain drugs which can damage tissues of the body. Infection can
also be a trigger of some autoimmune diseases for example Lupus which is thought to be a
milder version of an idiopathic disorder where there is an increased production of antihistone
antibodies.[7]

Pathophysiology[edit]
The human immune system typically produces both T-cells and B-cells that are capable of being
reactive with self-antigens, but these self-reactive cells are usually either killed prior to becoming
active within the immune system, placed into a state of energy (silently removed from their role
within the immune system due to over-activation), or removed from their role within the immune
system by regulatory cells. When any one of these mechanisms fail, it is possible to have a
reservoir of self-reactive cells that become functional within the immune system. The
mechanisms of preventing self-reactive T-cells from being created takes place through Negative
selection process within the thymus as the T-cell is developing into a mature immune cell.
Some infections, such as Campylobacter jejuni, have antigens that are similar (but not identical)
to our own self-molecules. In this case, a normal immune response to C. jejuni can result in the
production of antibodies that also react to a lesser degree with receptors on skeletal muscle
(i.e., Myasthenia gravis).
There are many theories as to how an autoimmune disease state arises. Some common ones
are listed below.

Cryptic determinants/molecular sequestration[edit]

Although it is possible for a potential auto antigen to be geographically sequestered in an
immune privileged site within the body (e.g. the eye), mechanisms exist to express even these
antigens in a tolerogenic fashion to the immune system. However, it is impossible to induce
tolerance (immune unresponsiveness) to all aspects of an autoantigen. This is because under
normal physiologic conditions some regions of a self-antigen are not expressed at a sufficient
level to induce tolerance. These poorly displayed areas of an antigen are called "cryptic
determinants." The immune system maintains a high-affinity repertoire to the cryptic self because
the presentation of these determinants was insufficient to induce strong tolerance. [9]

Molecular mimicry[edit]

The concept of molecular mimicry describes a situation in which a foreign antigen can initiate an
immune response in which a T or B cell component cross-recognizes self. The cross reactive
immune response is responsible for the autoimmune disease state.[10] Cross-reactive immune
responses to self were first described for antibodies.

Altered glycan theory[edit]

According to this theory the effector function of the immune response is mediated by
the glycans (polysaccharides) displayed by the cells and humoral components of the immune
system. Individuals with autoimmunity have alterations in their glycosylation profile such that a
proinflammatory immune response is favored. It is further hypothesized that individual
autoimmune diseases will have unique glycan signatures.[11]

List by category[edit]
It has been suggested that this list be split into a new article titled List of
autoimmune diseases. (Discuss.) (April 2016)

This list of autoimmune diseases is categorized by organ and tissue type to help locate diseases
that may be similar.

Major Organs
Heart
Kidney
Liver
Lung
Skin

Glands
Endocrine
Adrenal Gland
Multi-glandular
Pancreas
Thyroid Gland
Exocrine
Reproductive Organs
Salivary Glands

Digestive System

Tissue
Blood
Connective Tissue, Systemic, and multi-organ
Muscle
Nervous System
Eyes
Ears
Vascular system

Autoimmune Comorbidities

Not Autoimmune
Other Qualifiers

"Accepted" in prior version of this table

A comorbidity common among people with autoimmune disease, but with no evidence of
being itself caused by autoimmunity

Disease is an autoimmune response triggered by a specific environmental factor

Disease is only caused by autoimmunity in only a fraction of those who suffer from it

Described as an autoinflammatory disease

Evidence to indicate autoimmunity is extremely limited or circumstantial

Disease appears under Autoimmune Diseases in MeSH

Not listed in prior version of this table

Disease appeared in prior version but has been renamed. In renaming, precedence has

been given to scientific names over those based on discoverers.

"Suspected" in the prior version of this table

Disease has a known trigger, such as viral infection, vaccination, or injury

An extremely rare disease, which would suggest limited opportunity to study it and
conclusively determine whether it is caused by autoimmunity

Listed in the prior version of this table with "Accepted/Suspected" left blank

Autoimmune Diseases[edit]
This is a dynamic list and may never be able to satisfy particular standards for
completeness. You can help by expanding it with reliably sourced entries.
Level of
Organ/Tissue

Acceptanc

Type

e for

Disease Name

Autoimmu

Hypersensit
ivity
(I,II,III,IV)

ICD-9
Codes

Notes/Autoantibodies/Syno
nyms/Rare Variants

nity

Major Organs<Top>
Heart<Top>
Synonyms: Autoimmune
Myocarditis[12][13]

Moderate,

391.24224

myocarditis, Autoimmune

F, R, A

29.0

cardiomyopathy, Coxsackie
myocarditis

Postmyocardial
infarction
syndrome[13]

Postpericardiotomy

Autoantibodies: myocardial
Limited, R,
Y

411.0

neo-antigens formed as a
result of the MI. Synonyms:
Dressler's syndrome

Limited, N

429.4

syndrome

Subacute bacterial
endocarditis[14]

Autoantibodies: essential
Limited, Y

III

421.0

mixed cryoglobulinemia.
Synonyms: SBE

Kidney<Top>
Autoantibodies: AntiAnti-Glomerular

Basement Membrane

Basement

Moderate,

Membrane

R, M, A

II

446.21

nephritis[15]

Collagen Type IV Protein.

Synonyms: Goodpastures
Syndrome, Glomerulonephritis
Type 1

Interstitial cystitis[16]

Lupus nephritis

Limited, S

595.1

Comorbidit

583.81

y, N

Mast cells.
A comorbidity of Systemic
Lupus Erythematosis..

Liver<Top>
Autoantibodies: ANA and
SMA, LKM-1, LKM-2 or LKM3; antibodies against soluble
Autoimmune

Moderate,

cell-

hepatitis

mediated

[17][18][19]

571.42

liver antigen (anti-SLA, antiLP) no autoantibodies

detected (~20%)[citation
needed]. Synonyms: Lupoid
hepatitis
Autoantibodies: Anti-p62, Antisp100, AntiMitochondrial(M2)Anti-Ro aka

Primary biliary

Moderate,

cirrhosis[20][21][22][23]

SSA. Note that Sjogren's is

571.6

classified in some places

(e.g., MeSH) as rheumatoid
disease, but there is no
published evidence to support
that classification.

Primary sclerosing
cholangitis

Possible overlap with primary

Limited, Y

576.1

biliary cirrhosis.
Autoantibodies: HLA-DR52a.

Lung<Top>
Antisynthetase

Limited, Y

syndrome

279.49

Skin<Top>

Alopecia Areata

[24][25]

Autoantibodies: T-cells.

Moderate,

704.01

Patchy, Totalis, Universalis

Autoimmune

Limited, F,

277.6 995.

Angioedema[26][27]

Autoimmune
progesterone
dermatitis

Limited, X,

279.49

Autoimmune

Comorbidit

urticaria[28][29]

y, A

Synonyms: Alopecia areata -

708

Autoantibodies: IgG
Bullous
pemphigoid

Moderate,
[30]

694.5

autoantibodies targeting the

type XVII collagen component
of hemidesmosomes.
precipitates C3.

Cicatricial

Limited, R,

pemphigoid

X, Y

Autoantibodies: anti-BP-1, anti

694.61

BP-2. Synonyms: Benign

Mucosal Pemphigoid, Ocular
cicatricial pemphigoid

Dermatitis

Moderate,

herpetiformis[31]

C, Y

Discoid lupus

Limited, Y

Autoantibodies: IgA
694.0

Eosinophilia; anti-epidermal
transglutaminase antibodies.

III

695.4

IL-2 and IFN-gamma.

erythematosus[32]
Epidermolysis

Moderate,

bullosa acquisita

Erythema nodosum

694.8

Limited, F,

COL7A1.

695.2

Autoantibodies: IgG and C3

Gestational

Limited, R,

pemphigoid

646.8

misdirected antibodies
intended to protect the
placenta.

Hidradenitis

Limited, C,

suppurativa[33]

Lichen planus

Limited, Y

Lichen sclerosus

disease

646.8

Limited, C,

Moderate,

vulgaris

M, A

Synonyms: LAD

701.0

Pemphigus
[15][31]

701.0

Y
Moderate,

Morphea[35]

697.0

Limited, C,

Linear IgA
[34]

705.83

II

694.4

Autoantibodies: AntiDesmoglein 3 eosinophilia.

Pityriasis
lichenoides et

Limited, C

696.2

varioliformis acuta

Mucha-Habermann

Limited, C,

disease

Psoriasis

[36]

Moderate,
A

T-cells. Synonyms: Pityriasis

696.2

lichenoides, varioliformis
acuta
CD-8 T-cells, HLA-Cw6, IL-

IV?

696

12b, IL-23b, TNFalpha, NFB.

COL1A2 and TGF-1.

Autoantibodies: anti-nuclear
antibodies, anti-centromere
Systemic
scleroderma

Limited, R,
[35][37]

710.1

and anti-scl70/antitopoisomerase antibodies.

Synonyms: Diffuse cutaneous
systemic sclerosis, Systemic
sclerosis, Scleroderma

Vitiligo[38][39]

NALP-1 RERE, PTPN22, LPP,

Limited, C,

709.01

IL2RA, GZMB, UBASH3A and

C1QTNF6.

Glands<Top>
Endocrine<Top>
Adrenal Gland<Top>

Addison's disease[15]

Moderate,

255

F, Y

Autoantibodies: 21
hydroxylase.

Multi-glandular<Top>
Synonyms: Whitaker's
Autoimmune
polyendocrine
syndrome

Syndrome, APECED,
Moderate,

Unknown or

multiple

258.1

Addisons Disease,
Polyglandular Autoimmune
Syndrome 1, PGAS-1, APS
Type 1
DQ2, DQ8 and DRB1*0404.
Autoantibodies: anti-21

Autoimmune
polyendocrine
syndrome type 2[40]

hydroxylase, anti-17
Moderate,
A

258.1

hydroxylase. Synonyms:
Schmidt syndrome,
Polyglandular Autoimmune
Syndrome 2, PGAS-2, APS
Type 2

Autoimmune

Moderate,

258.1

Synonyms: Polyglandular

polyendocrine
syndrome type 3

Autoimmune Syndrome 3,

PGAS-3, APS Type 3

Pancreas<Top>
Autoantibodies: ANA; antiAutoimmune

Moderate,

pancreatitis

577.1

lactoferrin antibodiesanticarbonic anhydrase

antibodies; rheumatoid factor.
HLA-DR3, HLA-DR4.
Autoantibodies: Glutamic acid

Diabetes mellitus

Moderate,

type 1[15]

decarboxylase antibodies
IV

250.01

(GADA), islet cell antibodies

(ICA), and insulinomaassociated autoantibodies (IA2), anti-insulin antibodies.

Thyroid Gland<Top>
HLADR5, CTLA-4.
Autoantibodies: antibodies
Autoimmune
thyroiditis

against thyroid peroxidase

Strong, A

IV

245.8

and/or thyroglobulin.
Synonyms: chronic
lymphocytic thyroiditis,
Hashimoto's thyroiditis

Ord's thyroiditis

Moderate,

245.8

Autoantibodies: thyroid
Graves' disease[15]

Moderate,
M, A

II

242.0

autoantibodies (TSHR-Ab)
that activate the TSH-receptor
(TSHR).

Exocrine<Top>
Reproductive Organs<Top>
Autoimmune

Moderate,

614.2

Oophoritis

Endometriosis[41]

Limited, S

617.0

Limited, N

604.0

Autoimmune
orchitis
Salivary Glands<Top>

Sjogren's

Moderate,

syndrome[15][21][22][23]

Autoantibodies: anti-Ro. Also,

710.2

they are often present in

Sjogren's syndrome..

Digestive System<Top>
Autoimmune

Moderate,

enteropathy

X, Y
HLA-DQ8 and DQ2.5.

Celiac disease

[42][43][44]

Moderate,
A,E

Autoantibodies: Anti-tissue
IV??

579.0

transglutaminase antibodies
anti-endomysial IgA, antigliadin IgA.

Crohn's disease[45]

Moderate,
Y

Microscopic colitis

Limited, S

Ulcerative colitis[15]

Limited, A

IV

555

Innate immunity; Th17; Th1;

ATG16L1; CARD15;XBP1;.

558.9
IV

556

Tissue<Top>
Blood<Top>
Antiphospholipid

Moderate,

syndrome

M, A

[15]

289.81

HLA-DR7, HLA-B8, HLA-DR2,

HLA-DR3. Autoantibodies:
anti-cardiolipin;anti pyruvate
dehydrogenase; 2
glycoprotein I;
phosphatidylserine; anti apoH;

Annexin A5.

Aplastic anemia

Limited, F,

Autoimmune

Moderate,

hemolytic anemia

M, A

Autoimmune
lymphoproliferative
syndrome

neutropenia

F, N

purpura[15]

283.0

complement activation.

TNFRSF6; defective Fas279.41

Moderate,

thrombocytopenic

II

Moderate,

Autoimmune

Autoimmune

284

CD95 apoptosis. Synonyms:

Canale-Smith Syndrome

288.09

Autoantibodies: anti gpIIb-IIIa

Moderate,

287.31

M, R, A

or 1b-IX. Synonyms:
Idiopathic Thrombocytopenic
Purpura
idiopathic or secondary to

Cold agglutinin

Moderate,

disease

M, A

leukemia or infection.
II

283.0

Autoantibodies: IgM.
Synonyms: Autoimmune
hemolytic anemia?

Essential mixed

Limited, C,

cryoglobulinemia

273.2

Synonyms: A synonym for a

Evans syndrome

Moderate,
Y

287.32

combination of hemolytic
anemia and thrombocytopenic
purpura

IgG4-related

Limited, C,

systemic disease

Paroxysmal
nocturnal
hemoglobinuria

Limited, F,
S

283.2

Pernicious

Moderate,

anemia

[46]

Pure red cell

aplasia

II

Limited, Y

281.0

Autoantibodies: anti-parietal
cell antibody.

284.81

Multiple mechanisms.
Autoantibodies: glycoproteins
IIb-IIIa or Ib-IX in ITP antiADAMTS13 in TTP. and HUS
Thrombocytopenia[4

Limited, F,

7][48]

anti-cardiolipin (anti-cardiolipin
II

287.5

antibodies) and 2
glycoprotein I in
Antiphospholipid syndrome
anti-HPA-1a, anti-HPA-5b, and
others in NAIT. Synonyms:
Neonatal thrombocytopenia

Connective Tissue, Systemic, and multi-organ<Top>

Adiposis dolorosa[49]

Limited, L,
S

Adult-onset Still's

Moderate,

disease[50]

Ankylosing
Spondylitis[26][27]

Limited, S

272.8

Lipoid tissue. Synonyms:

Dercum's disease
macrophage migration

714.2

inhibitory factor.
Autoantibodies: ANA.

720.0

CD8; HLA-B27.

Autoantibodies: AntiCREST syndrome

Limited, Y

710.1

centromere antibodies Antinuclear antibodies.

Drug-induced lupus

Moderate,
Y

710.0

Autoantibodies: Anti-histone
antibodies.
MMP3, TRLR2, TLR4,

Enthesitis-related

Limited, C,

ERAP1. Autoantibodies: ..

arthritis

Synonyms: A subtype of

[51][52][53][54]

Juvenile Rheumatoid Arthritis

Eosinophilic

Limited, F,

fasciitis

Felty syndrome[55]

728.89

Strong, M,

Synonyms: Shulman's
syndrome

714.1

Autoantibodies: inconsistent
Juvenile Arthritis

[50]

ANA Rheumatoid factor.

Strong, M,

714.30

R, Y

Synonyms: Juvenile
rheumatoid arthritis, Juvenile
idiopathic arthritis

Lyme disease

Limited, L,

(Chronic)[56]

T, N

Mixed connective

Moderate,

tissue disease[15]

M, A

088.81

HLA-DR4. Autoantibodies:
710.8

anti-nuclear antibody anti-U1RNP.

Autoantibodies: anti-cyclic

Palindromic
rheumatism[57]

citrullinated peptide antibodies

Limited, Y

719.3

(anti-CCP) and antikeratin

antibodies (AKA). Synonyms:
Hench-Rosenberg syndrome

Parry Romberg
syndrome
Parsonage-Turner
syndrome

Psoriatic arthritis[58]

Reactive arthritis

Relapsing
polychondritis[59]

349.89351.

Limited, Y

Limited, Y

Moderate,
C, A
Limited, C,
F, Y
Strong, A

Autoantibodies: ANA.

353.5

IV?

696.0

HLA-B27.

099.3

Synonyms: Reiter's syndrome

733.99

Synonyms: atrophic
polychondritis, systemic
chondromalacia, chronic
atrophic polychondritis,

Meyenburg-Altherr-Uehlinger
syndrome, generalized
chondromalacia, systemic
chondromalacia
Retroperitoneal

Limited, Y

fibrosis

Rheumatic fever

[60][61]

Moderate,
T, A

593.4

Autoantibodies: streptococcal
II

390

M protein cross reacts with

human myosin.
HLA-DR4, PTPN22, depleted
B cells, TNF alpha, IL-17,

Rheumatoid

Strong, M,

arthritis

[15]

III

714

(also maybe IL-1, 6, and 15).

Autoantibodies: Rheumatoid
factor (anti-IgGFc), Anti-MCV,
ACPAs(Vimentin).

Sarcoidosis[62][63][64]

Limited, S

Schnitzler

Limited, L,

syndrome

X, Y

IV

135

273.1

BTNL2; HLA-B7-DR15; HLA

DR3-DQ2.

IgM?.

Autoantibodies: Anti-nuclear
Systemic Lupus
Erythematosus[15][21]
[22][23][31][65]

Strong, M,
A

antibodies anti-Ro. Also, they

III

695.4

are often present in Sjogren's

syndrome. Eosinophilia.
Synonyms: Lupus

Undifferentiated
connective tissue
disease

HLA-DR4. Autoantibodies:
Moderate,
C, A

710.9

anti-nuclear antibody.
Synonyms: Latent lupus,
incomplete lupus

Muscle<Top>
Dermatomyositis[66]

Moderate,

[67]

F, X, A

710.3

B- and T-cell perivascular

inflammatory infiltrate on
muscle biopsy.

Autoantibodies: histidine-tRNA
anti-signal recognition peptide
Anti-Mi-2 Anti-Jo1..
Synonyms: Juvenile
dermatomyositis
Limited, C,

Fibromyalgia

729.1

F, N

Inclusion body

Limited, F,

myositis

Similar to polymyositis but

359.71

therapy-activated T8 cells.

Limited, F,

Myositis

does not respond to steroid

729.1

HA-B8 HLA-DR3 HLA-DR1.

Myasthenia gravis[15]

Strong, M,
A

II

358

Autoantibodies: nicotinic
acetylcholine receptor MuSK
protein.

Neuromyotonia

[68]

Limited, F,
S

Autoantibodies: Voltage-gated
II?

333.90

Synonyms: Isaacs' Syndrome

Autoantibodies: anti-Yo (anti-

Paraneoplastic
cerebellar

Limited, Y

IV? II?

334.9

degeneration[69][70][71]

Polymyositis[66]

potassium channels.

cdr-2 in purkinje fibers) antiHu, anti-Tr, antiglutamate

receptor.

Limited, F,
A

710.4

Autoantibodies: IFN-gamma,
IL-1, TNF-alpha.

Nervous System<Top>
Acute disseminated

Strong, M,

323.61323. Synonyms: ADEM,

encephalomyelitis

T, A

81

Perivenous encephalomyelitis,
Acute hemorrhagic
leukoencephalitis, AHL, AHLE,
acute necrotizing
encephalopathy (ANE), acute

hemorrhagic
encephalomyelitis (AHEM),
acute necrotizing hemorrhagic
leukoencephalitis (ANHLE),
Weston-Hurst syndrome,
Hurst's disease
Acute motor axonal
neuropathy[72]
Anti-N-Methyl-DAspartate Receptor
Encephalitis[73]

Limited, N

356.8

Moderate,

Synonyms: Anti-NMDA

Encephalitis

Balo concentric

Moderate,

sclerosis

341.1

Synonyms: Balo disease,

Schilders disease
similar to Guillain-Barr

Bickerstaff's

Limited, Y

encephalitis

323.62

syndrome. Autoantibodies:
Anti-GQ1b 2/3 patients.
similar to GuillainBarr
syndrome. Autoantibodies:
anti-ganglioside antibodies.

Chronic

Synonyms: Relapsing

inflammatory

Moderate,

demyelinating
polyneuropathy

357.81

C, Y

polyneuropathy (CRP),
chronic inflammatory
demyelinating

[74]

polyradiculoneuropathy,
Chronic inflammatory
demyelinating polyneuritis
Autoantibodies: AntiGuillainBarr

Strong, M,

syndrome

[15]

IV

357.0

ganglioside, anti-GQ1b.
Synonyms: Miller-Fisher
syndrome, Landry's paralysis

Hashimoto's

Moderate,

encephalopathy[15][75]

C, X, A

IV

Autoantibodies: alphaenolase. Synonyms: Steroidresponsive encephalopathy

associated with autoimmune

thyroiditis, SREAT,
Nonvasculitic autoimmune
meningoencephalitis, NAIM,
Encephalopathy Associated
with Autoimmune Thyroid
Disease, EAATD
Idiopathic
inflammatory

Limited, F,

demyelinating

356.8

A set of different variants of

multiple sclerosis.

diseases
HLA-DR3-B8. Autoantibodies:
Lambert-Eaton
myasthenic
syndrome

voltage-gated calcium
Strong, M,

358.1

channels; Q-type calcium

channel, synaptogagmin,
muscarinic acetylcholine
receptor M1.
Autoantibody against
potassium channel has been
reported to present
demyelination pattern II. Other
cases present autoimmunity
against MOG[78] and Anoctami
n-2.[79] The three reported
autoimmune variants belong

Multiple
sclerosis, pattern
II

[76][76][77]

Strong, M,
A

to MS pattern II. Also involved

IV

340

HLA-DR2, PECAM-1, Antimyelin basic protein.

Autoantibodies: Anti-Kir4.1,
Anti-MOG, Anti-ANO2
(heterogeneous). Synonyms:
Primary progressive multiple
sclerosis, Relapsing-remitting
multiple sclerosis,
disseminated sclerosis,
encephalomyelitis disseminata

Oshtoran
Syndrome[80]

F06.9

Heritable, abnormalities in
the kynurenine and glutamate

metabolism
Pediatric
Autoimmune

antibodies against

Neuropsychiatric

Limited, F,

Disorder

II?

279.49

Associated with

streptococcal infection serve

as auto-antibodies.
Synonyms: PANDAS

Streptococcus
similar to Guillain-Barr
Progressive
inflammatory
neuropathy

Limited, X,
S

syndrome. Autoantibodies:
356.4

Anti-ganglioside
antibodies:anti-GM1, antiGD1a, anti-GQ1b.
May occur in Sjogren's

Restless leg

Limited, C,

syndrome

syndrome, celiac disease, and

333.94

rheumatoid arthritis or in
derangements of iron
metabolism.

Stiff person
syndrome[81]

Sydenham chorea

Transverse myelitis

GLRA1 (glycine receptor).

Limited, S

333.91

Autoantibodies: glutamic acid

decarboxylase (GAD).

Limited, T,
Y

392

Limited, M,

323.82341.

Eyes<Top>
Autoimmune

Limited, X,

retinopathy

[82]

Autoimmune uveitis

Cogan syndrome

Moderate,
F, A
Limited, F,
Y

364

370.52

Autoantibodies: HLAB-27?.

Graves

Moderate,

ophthalmopathy

M, N

Intermediate uveitis

Limited, L,

Limited, L,

conjunctivitis

optica[83][84]

myoclonus
syndrome[85]

Optic neuritis

Scleritis

Susac's syndrome

Limited, X,
S

Autoantibodies: NMO-IgG
II?

IV?

Limited, C,

348.39

Tolosa-Hunt

Limited, I,

syndrome

X, Y

Lymphocyte recruitment to
CSF.

Synonyms:

Limited, C,

379.59

379.0

ophthalmia

aquaporin 4. Synonyms:

377.30

Limited, I,

341.0

Devic's disease

Limited, C,

Sympathetic

Peripheral Uveitis

370.07

Limited, M,

Synonyms: Pars planitis,

372.39

Limited, L,

Neuromyelitis

Opsoclonus

364.3

Ligneous

Mooren's ulcer

242.9

Retinocochleocerebral
Vasculopathy

360.11

Autoantibodies: ocular
antigens following trauma.

378.55

Ears<Top>
Autoimmune inner
ear disease[86]

Limited, A

Mnire's disease[87] Limited, Y

III?

388.8

Synonyms: AIED

386.00

Autoantibodies: major

peripheral myelin protein P0.

Vascular system<Top>
Autoantibodies: Anti-neutrophil

Anti-neutrophil
cytoplasmic
antibodyassociated

cytoplasmic(cANCA).
Strong, M,

447.6

Synonyms: Wegener
Granulomatosis,
Granulomatosis with

vasculitis[88]

Polyangiitis
immune-mediated systemic
vasculitis; linkage to HLA-B51
(HLA-B27); very different

Behet's disease

manifestations with ulcers as

Limited, I,

136.1

X, A

common symptom.
Synonyms: Morbus
Adamandiades-Behet. Rare
Variant: Hughes-Stovin
syndrome

Churg-Strauss

Limited, I,

syndrome

X, Y

[31]

Giant cell arteritis [15]

Limited, I,
R, A

446.4

IV

446.5

Autoantibodies: p-ANCA
Eosinophilia.
Synonyms: Cranial arteritis,
Temporal Arteritis
Autoantibodies:
immunoglobulin A (IgA) and

Henoch-Schonlein

Limited, L,

purpura

complement component 3
287.0

(C3). Synonyms:
anaphylactoid purpura,
purpura rheumatica,
SchnleinHenoch purpura
ITPKC HLA-B51. Synonyms:

Kawasaki's disease

Moderate,
S,E[89]

Kawasaki syndrome, lymph

446.1

node syndrome,
mucocutaneous lymph node
syndrome

Leukocytoclastic

Limited, L,

vasculitis

Lupus vasculitis

447.6

Moderate,

583.81

C, N

Rheumatoid

Moderate,

vasculitis

C, N

447.6

A comorbidity of Systemic
Lupus Erythematosis.

A symptom of Lupus.

Binds to neutrophils causing

them to degranulate and
damages endothelium.
Microscopic
polyangiitis

Limited, Y

446.0

Autoantibodies: p-ANCA
myeloperoxidase. Synonyms:
microscopic
polyarteritis,microscopic
polyarteritis nodosa, MPA
Synonyms: panarteritis

Polyarteritis nodosa

Limited, L,

446.0

nodosa, periarteritis nodosa,

Kussmaul disease, KussmaulMaier disease

Polymyalgia

Limited, L,

rheumatica

725

Clinically may resemble type I

Urticarial

Limited, X,

vasculitis[90]

II?

708.9

hypersensitivity.
Autoantibodies: anti C1q
antibodies.

Vasculitis[30]

Strong, I,
M, F, A

III

447.6

Autoantibodies: sometimes
ANCA.

Autoimmune Comorbidities[edit]
This list includes conditions that are not diseases but signs common to
autoimmune disease. Some, such as Chronic Fatigue Syndrome, are
controversial.[13] These conditions are included here because they are frequently
listed as autoimmune diseases but should not be included in the list above until
there is more consistent evidence.

Level of
Organ/Tissue

Acceptance

Type

for

Disease Name

Autoimmunit

Hypersensitivi

ICD-9

ty

Notes/Autoantibodies/Synon
Code

(I,II,III,IV)

yms

y
Symptomatic of autoimmune

Chronic fatigue

Comorbidity,

diseases or autoimmune

syndrome

activity, but not a disease or a

cause of disease.
Symptomatic of autoimmune
diseases or autoimmune
activity, but not a disease or a

Complex regional

Comorbidity,

cause of disease. Synonyms:

pain syndrome

Amplified Musculoskeletal Pain

Syndrome, Reflex
Neurovascular Dystrophy,
Reflex sympathetic dystrophy

Eosinophilic

Comorbidity,

530.1

esophagitis

3
Possibly symptomatic of
autoimmune diseases, but not a

Gastritis

Comorbidity,

disease or a cause of disease.

Autoantibodies: serum
antiparietal and anti-IF
antibodies.
Possibly symptomatic of
autoimmune diseases, but not a

POEMS

Comorbidity,

syndrome[91]

disease or a cause of disease.

Autoantibodies: interleukin 1,
interleukin 6 and TNF.
vascular endothelial growth
factor (VEGF), given the ..

Raynaud's

Comorbidity,

Symptomatic of autoimmune

phenomenon

diseases or autoimmune
activity, but not a disease or a

cause of disease.
Primary
immunodeficiency
[92]

The condition is inherited, but it

Comorbidity,

279.8

is associated with several

autoimmune diseases.

Pyoderma

Comorbidity,

gangrenosum

Possibly symptomatic of
autoimmune diseases, but not a
disease or a cause of disease.

Not Autoimmune[edit]
At this time, there is not sufficient evidence - direct, indirect, or circumstantial - to
indicate that these diseases are caused by autoimmunity. These conditions are
included here because:
1. The disease was listed in the prior version of this table
2. The disease is included in several widely used lists of autoimmune
disease. It is included here to ensure that a person visiting this page
does not conclude that the disease was not considered. Before moving
a condition from here to the list of autoimmune diseases, references
should be provided in the Wikipedia page for the condition that point to
evidence of autoimmunity.
Level of
Organ/Tissue Type
Disease Name

Acceptance
for
Autoimmuni

Hypersensitiv

ICD-9

ity

Notes/Autoantibodies/Syno
Code

(I,II,III,IV)

nyms

ty
An immune system disorder
Not
Agammaglobulinemia

Autoimmune
,Y

279.0
0

but not an autoimmune

disease.. Autoantibodies:
IGHM; IGLL1: CD79A;
CD79B; BLNK; LRRC8A.

Not
Amyloidosis

Autoimmune
,N

277.3
0

No consistent evidence of
association with
autoimmunity.

No consistent evidence of
Amyotrophic lateral
sclerosis

association with

Not
Autoimmune
,Y

335.2

autoimmunity. Autoantibodies:

Amyotrophic lateral sclerosis

(Also Lou Gehrig's disease;
Motor Neuron Disease).

Anti-tubular

Not

No consistent evidence of

basement membrane

Autoimmune

association with

nephritis

,N

autoimmunity.

Not
Atopic allergy

Autoimmune

691.8

A hypersensitivity.

691.8

A hypersensitivity.

,Y
Not
Atopic dermatitis

Autoimmune
,Y

Autoimmune

Not

peripheral

Autoimmune

neuropathy

, F, A

Not
Blau syndrome

Autoimmune
,Y

Cancer

Chagas disease

See specific diseases that are

listed as autoimmune..
Overlaps both sarcoidosis
and granuloma annulare. No
evidence of association with
autoimmunity.
No consistent evidence of

Autoimmune

association with

,Y

autoimmunity.

Autoimmune
,Y

[93]

which may be autoimmune.

Not

Not
Castleman's disease

A class of diseases, some of

An immune system disorder

but not an autoimmune
disease.. Autoantibodies:
Over expression of IL-6.

Not

No consistent evidence of

Autoimmune

association with

,S

autoimmunity.

Chronic obstructive

Not

No consistent evidence of

pulmonary disease

Autoimmune

association with

[95]

,S

autoimmunity.

Chronic recurrent

Not

multifocal

Autoimmune

osteomyelitis

,Y

Complement

Not

Possibly symptomatic of

component 2

Autoimmune

autoimmune diseases, but not

deficiency

,Y

a disease.

Not

May be related to

Autoimmune

autoimmune activity in the

,N

mother.

[94]

Congenital heart
block

LPIN2, D18S60. Synonyms:

Majeed syndrome

Not
Contact dermatitis

Autoimmune

IV

A hypersensitivity.

,Y

Cushing's syndrome

Not

No consistent evidence of

Autoimmune

association with

,Y

autoimmunity.

Cutaneous

Not

leukocytoclastic

Autoimmune

angiitis

,Y

Dego's disease

No consistent evidence of
association with
autoimmunity. Autoantibodies:
neutrophils.

Not

No consistent evidence of

Autoimmune

association with

,Y

autoimmunity.
No consistent evidence of
association with

Eczema[96][97][98]

Not

autoimmunity. Autoantibodies:

Autoimmune

LEKTI, SPINK5, filaggrin.,

,Y

Brain-derived neurotrophic
factor (BDNF) and Substance
P..

Eosinophilic
gastroenteritis

Not

Possibly a hypersensitivity.

Autoimmune

Autoantibodies: IgE, IL-3, IL-

,Y

5, GM-CSF, eotaxin.
A class of diseases, some of

Eosinophilic
pneumonia

which may be autoimmune.

Not

Specifically, Churg-Strauss

Autoimmune

syndrome, a subtype of

, F, Y

Eosinophilic pneumonia, is
autoimmune.
Mother's immune system

Erythroblastosis
fetalis

Not
Autoimmune
,Y

attacks fetus. An immune

II

system disorder but not

autoimmune. Autoantibodies:
ABO, Rh, Kell antibodies.
Possibly an immune system

Fibrodysplasia

Not

disorder but not autoimmune.

ossificans

Autoimmune

Autoantibodies: ACVR1

progressiva

,Y

Lymphocytes express
increased BMP4.

Gastrointestinal
pemphigoid

Not

No consistent evidence of

Autoimmune

association with

,A

autoimmunity.
An immune system disorder

Hypogammaglobuline
mia

Not

but not autoimmune.

Autoimmune

Autoantibodies: IGHM, IGLL1,

,Y

CD79A, BLNK, LRRC8A,

CD79B.
No consistent evidence of
autoimmune cause though

Idiopathic giant-cell
myocarditis[99]

Not

the disease has been found

Autoimmune

comorbid with other

,N

autoimmune diseases.
Synonyms: Giant cell
myocarditis

Idiopathic pulmonary
fibrosis[100][100]

Autoantibodies: SFTPA1,

Not

SFTPA2, TERT, and TERC..

Autoimmune

Synonyms: Fibrosing

,Y

alveolitis
Autoantibodies: IgA produced
from marrow rather than
MALT. Synonyms: IgA

Not
IgA nephropathy

Autoimmune

III?

,Y

nephrits, Berger's disease,

Synpharyngitic
Glomerulonephritis. An
immune system disorder but
not an autoimmune disease.

Immunoregulatory
lipoproteins[101]

Not
Autoimmune

Not a disease.

,N
A genetic mutation in FOXP3
that leads to autoimmune
diseases, but no consistent

IPEX syndrome

Not

evidence that it is an

Autoimmune

autoimmune disorder itself..

,N

Synonyms: X-linked
polyendocrinopathy,
immunodeficiency and
diarrhea-syndrome (XLAAD)

Ligneous
conjunctivitis

Not

No consistent evidence of

Autoimmune

association with

,N

autoimmunity.
No consistent evidence of

Not
Majeed syndrome

association with

Autoimmune

autoimmunity. Autoantibodies:

,Y

Narcolepsy[102][103][104][105]

Not

LPIN2.
II?

No evidence of association

Autoimmune

with autoimmunity. Research

,Y

not reproducible.
Autoantibodies: hypocretin or

orexin, HLA-DQB1*0602.

Rasmussen's

association with

Autoimmune

encephalitis

Schizophrenia

No consistent evidence of

Not

autoimmunity. Autoantibodies:

,Y

[106][107][108]

anti-NR2A antibodies.

Not

No consistent evidence of

Autoimmune

association with

,S

autoimmunity.

Not
Serum sickness

Autoimmune

III

A hypersensitivity.

,Y

Not
Spondyloarthropathy

Autoimmune
,Y

Not
Sweet's syndrome

Autoimmune
,Y

Takayasu's arteritis

Undifferentiated
spondyloarthropathy

No consistent evidence of
association with
autoimmunity. Autoantibodies:
HLA-B27.
No consistent evidence of
association with
autoimmunity. Autoantibodies:
GCSF.

Not

No consistent evidence of

Autoimmune

association with

,Y

autoimmunity.

Not
Autoimmune
,Y

See Enthesitis-related
arthritis.

Development of therapies[edit]
In both autoimmune and inflammatory diseases, the condition arises through
aberrant reactions of the human adaptive or innate immune systems. In
autoimmunity, the patient's immune system is activated against the body's own
proteins. In chronic inflammatory diseases, neutrophils and other leukocytes are
constitutively recruited by cytokines and chemokines, leading to tissue damage.

Mitigation of inflammation by activation of anti-inflammatory genes and the

suppression of inflammatory genes in immune cells is a promising therapeutic
approach.[109][110][111]