Friday, 19 February 2016

Autoimmune disease - Lupus Lyme disease and Hughes syndrome

Autoimmune disease

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Autoimmune diseases
Classification and external resources
SpecialtyRheumatology, gastroenterology
ICD-10D84.9, M35.9
Autoimmune diseases arise from an abnormal immune response of the body against substances and tissues normally present in the body (autoimmunity). This may be restricted to certain organs (e.g. in autoimmune 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 immunosuppression—medication that decreases the immune response.
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.[1]


For a disease to be regarded as an autoimmune disease it needs to answer to Witebsky's postulates (first formulated by Ernst Witebsky and colleagues in 1957 and modified in 1994):[2][3]
  • 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 have a wide variety of different effects. They do tend to have one of three characteristic pathological effects which characterize them as autoimmune diseases:[4]
  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.[5]
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.[6]


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.[7]

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.[8] 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.[9]

List by category[edit]

This list of autoimmune diseases is categorized by organ and tissue type to help locate diseases that may be similar.
Other Qualifiers
A"Accepted" in prior version of this table
CA comorbidity common among people with autoimmune disease, but with no evidence of being itself caused by autoimmunity
EDisease is an autoimmune response triggered by a specific environmental factor
FDisease is only caused by autoimmunity in only a fraction of those who suffer from it
IDescribed as an autoinflammatory disease
LEvidence to indicate autoimmunity is extremely limited or circumstantial
MDisease appears under Autoimmune Diseases in MeSH
NNot listed in prior version of this table
RDisease appeared in prior version but has been renamed. In renaming, precedence has been given to scientific names over those based on discoverers.
S"Suspected" in the prior version of this table
TDisease has a known trigger, such as viral infection, vaccination, or injury
XAn extremely rare disease, which would suggest limited opportunity to study it and conclusively determine whether it is caused by autoimmunity
YListed in the prior version of this table with "Accepted/Suspected" left blank

Autoimmune Diseases[edit]

Organ/Tissue Type Disease NameLevel of Acceptance for AutoimmunityHypersensitivity (I,II,III,IV)ICD-9 CodesNotes/Autoantibodies/Synonyms/Rare Variants
Major Organs<Top>
Myocarditis[10][11]Moderate, F, R, A391.2422429.0Synonyms: Autoimmune myocarditis, Autoimmune cardiomyopathy, Coxsackie myocarditis
Postmyocardial infarction syndrome[11]Limited, R, Y411.0Autoantibodies: myocardial neo-antigens formed as a result of the MI. Synonyms: Dressler's syndrome
Postpericardiotomy syndromeLimited, N429.4
Subacute bacterial endocarditis[12]Limited, YIII421.0Autoantibodies: essential mixed cryoglobulinemia. Synonyms: SBE
Anti-Glomerular Basement Membrane nephritis[13]Moderate, R, M, AII446.21Autoantibodies: Anti-Basement Membrane Collagen Type IV Protein. Synonyms: Goodpastures Syndrome, Glomerulonephritis Type 1
Interstitial cystitis[14]Limited, S595.1Mast cells.
Lupus nephritisComorbidity, N583.81A comorbidity of Systemic Lupus Erythematosis..
Autoimmune hepatitis[15][16][17]Moderate, Acell-mediated571.42Autoantibodies: ANA and SMA, LKM-1, LKM-2 or LKM-3; antibodies against soluble liver antigen (anti-SLA, anti-LP) no autoantibodies detected (~20%)[citation needed]. Synonyms: Lupoid hepatitis
Primary biliary cirrhosis[18][19][20][21]Moderate, A571.6Autoantibodies: Anti-p62, Anti-sp100, Anti-Mitochondrial(M2)Anti-Ro aka SSA. Note that Sjogren's is classified in some places (e.g., MeSH) as rheumatoid disease, but there is no published evidence to support that classification.
Primary sclerosing cholangitisLimited, Y576.1Possible overlap with primary biliary cirrhosis. Autoantibodies: HLA-DR52a.
Antisynthetase syndromeLimited, Y279.49
Alopecia Areata[22][23]Moderate, A704.01Autoantibodies: T-cells. Synonyms: Alopecia areata - Patchy, Totalis, Universalis
Autoimmune Angioedema[24][25]Limited, F, N277.6 995.1
Autoimmune progesterone dermatitisLimited, X, A279.49
Autoimmune urticaria[26][27]Comorbidity, A708
Bullous pemphigoid[28]Moderate, Y694.5Autoantibodies: IgG autoantibodies targeting the type XVII collagen component of hemidesmosomes.
Cicatricial pemphigoidLimited, R, X, Y694.61precipitates C3. Autoantibodies: anti-BP-1, anti BP-2. Synonyms: Benign Mucosal Pemphigoid, Ocular cicatricial pemphigoid
Dermatitis herpetiformis[29]Moderate, C, Y694.0Autoantibodies: IgA Eosinophilia; anti-epidermal transglutaminase antibodies.
Discoid lupus erythematosus[30]Limited, YIII695.4IL-2 and IFN-gamma.
Epidermolysis bullosa acquisitaModerate, Y694.8COL7A1.
Erythema nodosumLimited, F, Y695.2
Gestational pemphigoidLimited, R, Y646.8Autoantibodies: IgG and C3 misdirected antibodies intended to protect the placenta.
Hidradenitis suppurativa[31]Limited, C, S705.83
Lichen planusLimited, Y697.0
Lichen sclerosusLimited, C, Y701.0
Linear IgA disease[32]Moderate, Y646.8Synonyms: LAD
Morphea[33]Limited, C, S701.0
Pemphigus vulgaris[13][29]Moderate, M, AII694.4Autoantibodies: Anti-Desmoglein 3 eosinophilia.
Pityriasis lichenoides et varioliformis acutaLimited, C696.2
Mucha-Habermann diseaseLimited, C, Y696.2T-cells. Synonyms: Pityriasis lichenoides, varioliformis acuta
Psoriasis[34]Moderate, AIV?696CD-8 T-cells, HLA-Cw6, IL-12b, IL-23b, TNFalpha, NF-κB.
Systemic scleroderma[33][35]Limited, R, S710.1COL1A2 and TGF-β1. Autoantibodies: anti-nuclear antibodies, anti-centromere and anti-scl70/anti-topoisomerase antibodies. Synonyms: Diffuse cutaneous systemic sclerosis, Systemic sclerosis, Scleroderma
Vitiligo[36][37]Limited, C, S709.01NALP-1 RERE, PTPN22, LPP, IL2RA, GZMB, UBASH3A and C1QTNF6.
Adrenal Gland<Top>
Addison's disease[13]Moderate, F, Y255Autoantibodies: 21 hydroxylase.
Autoimmune polyendocrine syndromeModerate, AUnknown or multiple258.1Synonyms: Whitaker's Syndrome, APECED, Addisons Disease, Polyglandular Autoimmune Syndrome 1, PGAS-1, APS Type 1
Autoimmune polyendocrine syndrome type 2[38]Moderate, A258.1DQ2, DQ8 and DRB1*0404. Autoantibodies: anti-21 hydroxylase, anti-17 hydroxylase. Synonyms: Schmidt syndrome, Polyglandular Autoimmune Syndrome 2, PGAS-2, APS Type 2
Autoimmune polyendocrine syndrome type 3Moderate, A258.1Synonyms: Polyglandular Autoimmune Syndrome 3, PGAS-3, APS Type 3
Autoimmune pancreatitisModerate, A577.1Autoantibodies: ANA; anti-lactoferrin antibodiesanti-carbonic anhydrase antibodies; rheumatoid factor.
Diabetes mellitus type 1[13]Moderate, AIV250.01HLA-DR3, HLA-DR4. Autoantibodies: Glutamic acid decarboxylase antibodies (GADA), islet cell antibodies (ICA), and insulinoma-associated autoantibodies (IA-2), anti-insulin antibodies.
Thyroid Gland<Top>
Autoimmune thyroiditisStrong, AIV245.8HLADR5, CTLA-4. Autoantibodies: antibodies against thyroid peroxidase and/or thyroglobulin. Synonyms: chronic lymphocytic thyroiditis, Hashimoto's thyroiditis
Ord's thyroiditisModerate, Y245.8
Graves' disease[13]Moderate, M, AII242.0Autoantibodies: thyroid autoantibodies (TSHR-Ab) that activate the TSH-receptor (TSHR).
Reproductive Organs<Top>
Autoimmune OophoritisModerate, N614.2
Endometriosis[39]Limited, S617.0
Autoimmune orchitisLimited, N604.0
Salivary Glands<Top>
Sjogren's syndrome[13][19][20][21]Moderate, A710.2Autoantibodies: anti-Ro. Also, they are often present in Sjogren's syndrome..
Digestive System<Top>
Autoimmune enteropathyModerate, X, Y
Celiac disease[40][41][42]Moderate, A,EIV??579.0HLA-DQ8 and DQ2.5. Autoantibodies: Anti-tissue transglutaminase antibodies anti-endomysial IgA, anti-gliadin IgA.
Crohn's disease[43]Moderate, YIV555Innate immunity; Th17; Th1; ATG16L1; CARD15;XBP1;.
Microscopic colitisLimited, S558.9
Ulcerative colitis[13]Limited, AIV556
Antiphospholipid syndrome[13]Moderate, M, A289.81HLA-DR7, HLA-B8, HLA-DR2, HLA-DR3. Autoantibodies: anti-cardiolipin;anti pyruvate dehydrogenase; β2 glycoprotein I; phosphatidylserine; anti apoH; Annexin A5.
Aplastic anemiaLimited, F, Y284
Autoimmune hemolytic anemiaModerate, M, AII283.0complement activation.
Autoimmune lymphoproliferative syndromeModerate, A279.41TNFRSF6; defective Fas-CD95 apoptosis. Synonyms: Canale-Smith Syndrome
Autoimmune neutropeniaModerate, F, N288.09
Autoimmune thrombocytopenic purpura[13]Moderate, M, R, A287.31Autoantibodies: anti gpIIb-IIIa or 1b-IX. Synonyms: Idiopathic Thrombocytopenic Purpura
Cold agglutinin diseaseModerate, M, AII283.0idiopathic or secondary to leukemia or infection. Autoantibodies: IgM. Synonyms: Autoimmune hemolytic anemia?
Essential mixed cryoglobulinemiaLimited, C, Y273.2
Evans syndromeModerate, Y287.32Synonyms: A synonym for a combination of hemolytic anemia and thrombocytopenic purpura
IgG4-related systemic diseaseLimited, C, N
Paroxysmal nocturnal hemoglobinuriaLimited, F, S283.2
Pernicious anemia[44]Moderate, AII281.0Autoantibodies: anti-parietal cell antibody.
Pure red cell aplasiaLimited, Y284.81
Thrombocytopenia[45][46]Limited, F, YII287.5Multiple mechanisms. Autoantibodies: glycoproteins IIb-IIIa or Ib-IX in ITP anti-ADAMTS13 in TTP. and HUS anti-cardiolipin (anti-cardiolipin antibodies) and β2 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[47]Limited, L, S272.8Lipoid tissue. Synonyms: Dercum's disease
Adult-onset Still's disease[48]Moderate, Y714.2macrophage migration inhibitory factor. Autoantibodies: ANA.
Ankylosing Spondylitis[24][25]Limited, S720.0CD8; HLA-B27.
CREST syndromeLimited, Y710.1Autoantibodies: Anti-centromere antibodies Anti-nuclear antibodies.
Drug-induced lupusModerate, Y710.0Autoantibodies: Anti-histone antibodies.
Enthesitis-related arthritis[49][50][51][52]Limited, C, YMMP3, TRLR2, TLR4, ERAP1. Autoantibodies: .. Synonyms: A subtype of Juvenile Rheumatoid Arthritis
Eosinophilic fasciitisLimited, F, A728.89Synonyms: Shulman's syndrome
Felty syndrome[53]Strong, M, Y714.1
Juvenile Arthritis[48]Strong, M, R, Y714.30Autoantibodies: inconsistent ANA Rheumatoid factor. Synonyms: Juvenile rheumatoid arthritis, Juvenile idiopathic arthritis
Lyme disease (Chronic)[54]Limited, L, T, N088.81
Mixed connective tissue disease[13]Moderate, M, A710.8HLA-DR4. Autoantibodies: anti-nuclear antibody anti-U1-RNP.
Palindromic rheumatism[55]Limited, Y719.3Autoantibodies: anti-cyclic citrullinated peptide antibodies (anti-CCP) and antikeratin antibodies (AKA). Synonyms: Hench-Rosenberg syndrome
Parry Romberg syndromeLimited, Y349.89351.8Autoantibodies: ANA.
Parsonage-Turner syndromeLimited, Y353.5
Psoriatic arthritis[56]Moderate, C, AIV?696.0HLA-B27.
Reactive arthritisLimited, C, F, Y099.3Synonyms: Reiter's syndrome
Relapsing polychondritis[57]Strong, A733.99Synonyms: atrophic polychondritis, systemic chondromalacia, chronic atrophic polychondritis, Meyenburg-Altherr-Uehlinger syndrome, generalized chondromalacia, systemic chondromalacia
Retroperitoneal fibrosisLimited, Y593.4
Rheumatic fever[58][59]Moderate, T, AII390Autoantibodies: streptococcal M protein cross reacts with human myosin.
Rheumatoid arthritis[13]Strong, M, AIII714HLA-DR4, PTPN22, depleted B cells, TNF alpha, IL-17, (also maybe IL-1, 6, and 15). Autoantibodies: Rheumatoid factor (anti-IgGFc), Anti-MCV, ACPAs(Vimentin).
Sarcoidosis[60][61][62]Limited, SIV135BTNL2; HLA-B7-DR15; HLA DR3-DQ2.
Schnitzler syndromeLimited, L, X, Y273.1IgM?.
Systemic Lupus Erythematosus[13][19][20][21][29][63]Strong, M, AIII695.4Autoantibodies: Anti-nuclear antibodies anti-Ro. Also, they are often present in Sjogren's syndrome. Eosinophilia. Synonyms: Lupus
Undifferentiated connective tissue diseaseModerate, C, A710.9HLA-DR4. Autoantibodies: anti-nuclear antibody. Synonyms: Latent lupus, incomplete lupus
Dermatomyositis[64][65]Moderate, F, X, A710.3B- and T-cell perivascular inflammatory infiltrate on muscle biopsy. Autoantibodies: histidine-tRNA anti-signal recognition peptide Anti-Mi-2 Anti-Jo1.. Synonyms: Juvenile dermatomyositis
FibromyalgiaLimited, C, F, N729.1
Inclusion body myositisLimited, F, Y359.71Similar to polymyositis but does not respond to steroid therapy-activated T8 cells.
MyositisLimited, F, Y729.1
Myasthenia gravis[13]Strong, M, AII358HA-B8 HLA-DR3 HLA-DR1. Autoantibodies: nicotinic acetylcholine receptor MuSK protein.
Neuromyotonia[66]Limited, F, SII?333.90Autoantibodies: Voltage-gated potassium channels. Synonyms: Isaacs' Syndrome
Paraneoplastic cerebellar degeneration[67][68][69]Limited, YIV? II?334.9Autoantibodies: anti-Yo (anti-cdr-2 in purkinje fibers) anti-Hu, anti-Tr, antiglutamate receptor.
Polymyositis[64]Limited, F, A710.4Autoantibodies: IFN-gamma, IL-1, TNF-alpha.
Nervous System<Top>
Acute disseminated encephalomyelitisStrong, M, T, A323.61323.81Synonyms: ADEM, 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[70]Limited, N356.8
Anti-N-Methyl-D-Aspartate Receptor Encephalitis[71]Moderate, NSynonyms: Anti-NMDA Encephalitis
Balo concentric sclerosisModerate, Y341.1Synonyms: Balo disease, Schilders disease
Bickerstaff's encephalitisLimited, Y323.62similar to Guillain-Barré syndrome. Autoantibodies: Anti-GQ1b 2/3 patients.
Chronic inflammatory demyelinating polyneuropathy[72]Moderate, C, Y357.81similar to Guillain–Barré syndrome. Autoantibodies: anti-ganglioside antibodies. Synonyms: Relapsing polyneuropathy (CRP), chronic inflammatory demyelinating polyradiculoneuropathy, Chronic inflammatory demyelinating polyneuritis
Guillain–Barré syndrome[13]Strong, M, AIV357.0Autoantibodies: Anti-ganglioside, anti-GQ1b. Synonyms: Miller-Fisher syndrome, Landry's paralysis
Hashimoto's encephalopathy[13][73]Moderate, C, X, AIVAutoantibodies: alpha-enolase. Synonyms: Steroid-responsive encephalopathy associated with autoimmune thyroiditis, SREAT, Nonvasculitic autoimmune meningoencephalitis, NAIM, Encephalopathy Associated with Autoimmune Thyroid Disease, EAATD
Idiopathic inflammatory demyelinating diseasesLimited, F, Y356.8a variant of multiple sclerosis.
Lambert-Eaton myasthenic syndromeStrong, M, Y358.1HLA-DR3-B8. Autoantibodies: voltage-gated calcium channels; Q-type calcium channel, synaptogagmin, muscarinic acetylcholine receptor M1.
Multiple sclerosis[74][74][75]Strong, M, AIV340Autoantibody against potassium channel. Also invoved HLA-DR2, PECAM-1, Anti-myelin basic protein. Autoantibodies: Anti-Kir4.1 (heterogeneous). Synonyms: Primary progressive multiple sclerosis, Relapsing-remitting multiple sclerosis, disseminated sclerosis, encephalomyelitis disseminata
Pediatric Autoimmune Neuropsychiatric Disorder Associated with StreptococcusLimited, F, SII?279.49antibodies against streptococcal infection serve as auto-antibodies. Synonyms: PANDAS
Progressive inflammatory neuropathyLimited, X, S356.4similar to Guillain-Barré syndrome. Autoantibodies: Anti-ganglioside antibodies:anti-GM1, anti-GD1a, anti-GQ1b.
Restless leg syndromeLimited, C, S333.94May occur in Sjogren's syndrome, celiac disease, and rheumatoid arthritis or in derangements of iron metabolism.
Stiff person syndrome[76]Limited, S333.91GLRA1 (glycine receptor). Autoantibodies: glutamic acid decarboxylase (GAD).
Sydenham choreaLimited, T, Y392
Transverse myelitisLimited, M, A323.82341.2
Autoimmune retinopathy[77]Limited, X, N
Autoimmune uveitisModerate, F, A364Autoantibodies: HLAB-27?.
Cogan syndromeLimited, F, Y370.52
Graves ophthalmopathyModerate, M, N242.9
Intermediate uveitisLimited, L, Y364.3Synonyms: Pars planitis, Peripheral Uveitis
Ligneous conjunctivitisLimited, L, N372.39
Mooren's ulcerLimited, L, N370.07
Neuromyelitis optica[78][79]Limited, M, YII?341.0Autoantibodies: NMO-IgG aquaporin 4. Synonyms: Devic's disease
Opsoclonus myoclonus syndrome[80]Limited, X, SIV?379.59Lymphocyte recruitment to CSF.
Optic neuritisLimited, C, Y377.30
ScleritisLimited, C, Y379.0
Susac's syndromeLimited, C, Y348.39Synonyms: Retinocochleocerebral Vasculopathy
Sympathetic ophthalmiaLimited, I, Y360.11Autoantibodies: ocular antigens following trauma.
Tolosa-Hunt syndromeLimited, I, X, Y378.55
Autoimmune inner ear disease[81]Limited, A388.8Synonyms: AIED
Ménière's disease[82]Limited, YIII?386.00Autoantibodies: major peripheral myelin protein P0.
Vascular system<Top>
Anti-neutrophil cytoplasmic antibody-associated vasculitis[83]Strong, M, A447.6Autoantibodies: Anti-neutrophil cytoplasmic(cANCA). Synonyms: Wegener Granulomatosis, Granulomatosis with Polyangiitis
Behçet's diseaseLimited, I, X, A136.1immune-mediated systemic vasculitis; linkage to HLA-B51 (HLA-B27); very different manifestations with ulcers as common symptom. Synonyms: Morbus Adamandiades-Behçet. Rare Variant: Hughes-Stovin syndrome
Churg-Strauss syndrome[29]Limited, I, X, Y446.4Autoantibodies: p-ANCA Eosinophilia.
Giant cell arteritis [13]Limited, I, R, AIV446.5Synonyms: Cranial arteritis, Temporal Arteritis
Henoch-Schonlein purpuraLimited, L, Y287.0Autoantibodies: immunoglobulin A (IgA) and complement component 3 (C3). Synonyms: anaphylactoid purpura, purpura rheumatica, Schönlein–Henoch purpura
Kawasaki's diseaseModerate, S,E[84]446.1ITPKC HLA-B51. Synonyms: Kawasaki syndrome, lymph node syndrome, mucocutaneous lymph node syndrome
Leukocytoclastic vasculitisLimited, L, Y447.6
Lupus vasculitisModerate, C, N583.81A comorbidity of Systemic Lupus Erythematosis.
Rheumatoid vasculitisModerate, C, N447.6A symptom of Lupus.
Microscopic polyangiitisLimited, Y446.0Binds to neutrophils causing them to degranulate and damages endothelium. Autoantibodies: p-ANCA myeloperoxidase. Synonyms: microscopic polyarteritis,microscopic polyarteritis nodosa, MPA
Polyarteritis nodosaLimited, L, Y446.0Synonyms: panarteritis nodosa, periarteritis nodosa, Kussmaul disease, Kussmaul-Maier disease
Polymyalgia rheumaticaLimited, L, Y725
Urticarial vasculitis[85]Limited, X, YII?708.9Clinically may resemble type I hypersensitivity. Autoantibodies: anti C1q antibodies.
Vasculitis[28]Strong, I, M, F, AIII447.6Autoantibodies: 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.[11] 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.
Organ/Tissue Type Disease NameLevel of Acceptance for AutoimmunityHypersensitivity (I,II,III,IV)ICD-9 CodesNotes/Autoantibodies/Synonyms
Chronic fatigue syndromeComorbidity, NSymptomatic of autoimmune diseases or autoimmune activity, but not a disease or a cause of disease.
Complex regional pain syndromeComorbidity, NSymptomatic of autoimmune diseases or autoimmune activity, but not a disease or a cause of disease. Synonyms: Amplified Musculoskeletal Pain Syndrome, Reflex Neurovascular Dystrophy, Reflex sympathetic dystrophy
Eosinophilic esophagitisComorbidity, N530.13
GastritisComorbidity, YPossibly symptomatic of autoimmune diseases, but not a disease or a cause of disease. Autoantibodies: serum antiparietal and anti-IF antibodies.
POEMS syndrome[86]Comorbidity, YPossibly symptomatic of autoimmune diseases, but not a disease or a cause of disease. Autoantibodies: interleukin 1β, interleukin 6 and TNFα. vascular endothelial growth factor (VEGF), given the ..
Raynaud phenomenonComorbidity, SSymptomatic of autoimmune diseases or autoimmune activity, but not a disease or a cause of disease.
Primary immunodeficiency[87]Comorbidity, N279.8The condition is inherited, but it is associated with several autoimmune diseases.
Pyoderma gangrenosumComorbidity, YPossibly 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.
Organ/Tissue Type Disease NameLevel of Acceptance for AutoimmunityHypersensitivity (I,II,III,IV)ICD-9 CodesNotes/Autoantibodies/Synonyms
AgammaglobulinemiaNot Autoimmune, Y279.00An immune system disorder but not an autoimmune disease.. Autoantibodies: IGHM; IGLL1: CD79A; CD79B; BLNK; LRRC8A.
AmyloidosisNot Autoimmune, N277.30No consistent evidence of association with autoimmunity.
Amyotrophic lateral sclerosisNot Autoimmune, Y335.20No consistent evidence of association with autoimmunity. Autoantibodies: Amyotrophic lateral sclerosis (Also Lou Gehrig's disease; Motor Neuron Disease).
Anti-tubular basement membrane nephritisNot Autoimmune, NNo consistent evidence of association with autoimmunity.
Atopic allergyNot Autoimmune, YI691.8A hypersensitivity.
Atopic dermatitisNot Autoimmune, YI691.8A hypersensitivity.
Autoimmune peripheral neuropathyNot Autoimmune, F, AA class of diseases, some of which may be autoimmune. See specific diseases that are listed as autoimmune..
Blau syndromeNot Autoimmune, YOverlaps both sarcoidosis and granuloma annulare. No evidence of association with autoimmunity.
CancerNot Autoimmune, YNo consistent evidence of association with autoimmunity.
Castleman's diseaseNot Autoimmune, YAn immune system disorder but not an autoimmune disease.. Autoantibodies: Over expression of IL-6.
Chagas disease[88]Not Autoimmune, SNo consistent evidence of association with autoimmunity.
Chronic obstructive pulmonary disease[89][90]Not Autoimmune, SNo consistent evidence of association with autoimmunity.
Chronic recurrent multifocal osteomyelitisNot Autoimmune, YLPIN2, D18S60. Synonyms: Majeed syndrome
Complement component 2 deficiencyNot Autoimmune, YPossibly symptomatic of autoimmune diseases, but not a disease.
Congenital heart blockNot Autoimmune, NMay be related to autoimmune activity in the mother.
Contact dermatitisNot Autoimmune, YIVA hypersensitivity.
Cushing's syndromeNot Autoimmune, YNo consistent evidence of association with autoimmunity.
Cutaneous leukocytoclastic angiitisNot Autoimmune, YNo consistent evidence of association with autoimmunity. Autoantibodies: neutrophils.
Dego's diseaseNot Autoimmune, YNo consistent evidence of association with autoimmunity.
Eczema[91][92][93]Not Autoimmune, YNo consistent evidence of association with autoimmunity. Autoantibodies: LEKTI, SPINK5, filaggrin., Brain-derived neurotrophic factor (BDNF) and Substance P..
Eosinophilic gastroenteritisNot Autoimmune, YPossibly a hypersensitivity. Autoantibodies: IgE, IL-3, IL-5, GM-CSF, eotaxin.
Eosinophilic pneumoniaNot Autoimmune, F, YA class of diseases, some of which may be autoimmune. Specifically, Churg-Strauss syndrome, a subtype of Eosinophilic pneumonia, is autoimmune.
Erythroblastosis fetalisNot Autoimmune, YIIMother's immune system attacks fetus. An immune system disorder but not autoimmune. Autoantibodies: ABO, Rh, Kell antibodies.
Fibrodysplasia ossificans progressivaNot Autoimmune, YPossibly an immune system disorder but not autoimmune. Autoantibodies: ACVR1 Lymphocytes express increased BMP4.
Gastrointestinal pemphigoidNot Autoimmune, ANo consistent evidence of association with autoimmunity.
HypogammaglobulinemiaNot Autoimmune, YAn immune system disorder but not autoimmune. Autoantibodies: IGHM, IGLL1, CD79A, BLNK, LRRC8A, CD79B.
Idiopathic giant-cell myocarditis[94]Not Autoimmune, NNo consistent evidence of autoimmune cause though the disease has been found comorbid with other autoimmune diseases. Synonyms: Giant cell myocarditis
Idiopathic pulmonary fibrosis[95][95]Not Autoimmune, YAutoantibodies: SFTPA1, SFTPA2, TERT, and TERC.. Synonyms: Fibrosing alveolitis
IgA nephropathyNot Autoimmune, YIII?Autoantibodies: IgA produced from marrow rather than MALT. Synonyms: IgA nephrits, Berger's disease, Synpharyngitic Glomerulonephritis. An immune system disorder but not an autoimmune disease.
Immunoregulatory lipoproteins[96]Not Autoimmune, NNot a disease.
IPEX syndromeNot Autoimmune, NA genetic mutation in FOXP3 that leads to autoimmune diseases, but no consistent evidence that it is an autoimmune disorder itself.. Synonyms: X-linked polyendocrinopathy, immunodeficiency and diarrhea-syndrome (XLAAD)
Ligneous conjunctivitisNot Autoimmune, NNo consistent evidence of association with autoimmunity.
Majeed syndromeNot Autoimmune, YNo consistent evidence of association with autoimmunity. Autoantibodies: LPIN2.
Narcolepsy[97][98][99][100]Not Autoimmune, YII?No evidence of association with autoimmunity. Research not reproducible. Autoantibodies: hypocretin or orexin, HLA-DQB1*0602.
Rasmussen's encephalitisNot Autoimmune, YNo consistent evidence of association with autoimmunity. Autoantibodies: anti-NR2A antibodies.
Schizophrenia[101][102][103]Not Autoimmune, SNo consistent evidence of association with autoimmunity.
Serum sicknessNot Autoimmune, YIIIA hypersensitivity.
SpondyloarthropathyNot Autoimmune, YNo consistent evidence of association with autoimmunity. Autoantibodies: HLA-B27.
Sweet's syndromeNot Autoimmune, YNo consistent evidence of association with autoimmunity. Autoantibodies: GCSF.
Takayasu's arteritisNot Autoimmune, YNo consistent evidence of association with autoimmunity.
Undifferentiated spondyloarthropathyNot Autoimmune, YSee 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.[104][105][106]

See also[edit]


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