List of autoimmune diseases

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

Autoimmune diseases qualifiers

Overview of the qualifiers for the list.

Other Qualifiers
A"Accepted" in prior version of this table.
CA disease, regarded as autoimmune, that is often found in individuals with another autoimmune condition. This designation is given to diseases that are classified by Rose and Bona as having "circumstantial" evidence of autoimmune etiology. Diseases in this list with a "C" are, therefore, actual autoimmune diseases, rather than comorbid symptoms, which appear after this list.
EDisease is an autoimmune response triggered by a specific environmental factor.
FDisease is only caused by autoimmunity in only a fraction of those who have 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

Major organs

Organ/Tissue type

Disease name

Level of acceptance for autoimmunity Hypersensitivity

(I,II,III,IV)

ICD-9

Codes

Notes/Autoantibodies/Synonyms/Rare variants
Heart
Myocarditis[1][2]Moderate, F, R, A, T391.2422429.0Synonyms: Autoimmune myocarditis, Autoimmune cardiomyopathy, Coxsackie myocarditis
Postmyocardial infarction syndrome[2]Limited, R, Y411.0Autoantibodies: Myocardial neo-antigens formed as a result of the MI.
Synonym: Dressler's syndrome
Postpericardiotomy syndromeLimited, N429.4
Subacute bacterial endocarditis (SBE)[3]Limited, YIII421.0Autoantibodies: essential mixed cryoglobulinemia.
Kidney
Anti-Glomerular Basement Membrane nephritis[4]Moderate, R, M, AII446.21Autoantibodies: Anti-Basement Membrane Collagen Type IV Protein.
Synonyms: Goodpastures Syndrome, Glomerulonephritis Type 1
Lupus nephritisComorbidity, N583.81A comorbidity of Systemic Lupus Erythematosus.
Bladder
Interstitial cystitis[5]Limited, S595.1Mast cells.
Liver
Autoimmune hepatitis[6][7][8]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%).
Synonym: Lupoid hepatitis
Primary biliary cholangitis (PBC)[9][10][11][12]Moderate, A571.6Autoantibodies: Anti-p62, Anti-sp100, Anti-Mitochondrial(M2), Anti-Ro aka SSA.
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.
Lung
Antisynthetase syndromeLimited, Y279.49Autoantibodies: Anti-Jo1, Anti-PL7, Anti-PL12.
Skin
Alopecia Areata[13][14][15]Moderate, A704.01Autoantibodies: T-cells.
Synonyms: Alopecia areata - Patchy, Totalis, Universalis
Autoimmune Angioedema[16]Limited, F, N277.6 995.1
Autoimmune progesterone dermatitisLimited, X, A279.49
Autoimmune urticaria[17][18]Comorbidity, A708
Bullous pemphigoid[19]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[20]Moderate, C, Y694.0Autoantibodies: IgA Eosinophilia; anti-epidermal transglutaminase antibodies.
Discoid lupus erythematosus[21]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[22]Limited, C, S705.83
Lichen planusLimited, Y697.0
Lichen sclerosusLimited, C, Y701.0
Linear IgA disease (LAD)[23]Moderate, Y646.8
Morphea[24]Limited, C, S701.0
Pemphigus vulgaris[4][20]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[25]Moderate, AIV?696CD-8 T-cells, HLA-Cw6, IL-12b, IL-23b, TNFalpha, NF-κB.
Systemic scleroderma[24][26]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[27][28]Limited, C, S709.01NALP-1 RERE, PTPN22, LPP, IL2RA, GZMB, UBASH3A and C1QTNF6.

Glands

Organ/Tissue type

Disease name

Level of acceptance for autoimmunity Hypersensitivity

(I,II,III,IV)

ICD-9

Codes

Notes/Autoantibodies/Synonyms/Rare variants
Endocrine
Adrenal gland
Addison's disease[4]Moderate, F, Y255 Autoantibodies: 21 hydroxylase.
Multi-glandular
Autoimmune polyendocrine syndrome (APS) type 1Moderate, AUnknown or multiple258.1Synonyms: Whitaker's Syndrome, Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), Addisons Disease, Polyglandular Autoimmune Syndrome 1 (PGAS-1).
Autoimmune polyendocrine syndrome (APS) type 2[29]Moderate, A258.1DQ2, DQ8 and DRB1*0404.
Autoantibodies: anti-21 hydroxylase, anti-17 hydroxylase.
Synonyms: Schmidt syndrome, Polyglandular Autoimmune Syndrome 2 (PGAS-2).
Autoimmune polyendocrine syndrome (APS) type 3Moderate, A258.1Synonym: Polyglandular Autoimmune Syndrome 3 (PGAS-3).
Pancreas
Autoimmune pancreatitis (AIP)Moderate, A577.1Autoantibodies: ANA, anti-lactoferrin antibodies, anti-carbonic anhydrase antibodies, rheumatoid factor.
Diabetes mellitus type 1[4]Moderate, AIV250.01HLA-DR3, HLA-DR4.
Autoantibodies: Glutamic acid decarboxylase antibodies (GADA), islet cell antibodies (ICA), insulinoma-associated autoantibodies (IA-2), anti-insulin antibodies.
Thyroid gland
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[4]Moderate, M, AII242.0Autoantibodies: Thyroid autoantibodies (TSHR-Ab) that activate the TSH-receptor (TSHR).
Exocrine
Reproductive organs
Autoimmune oophoritisModerate, N614.2
Endometriosis[30]Limited, S617.0
Autoimmune orchitisLimited, N604.0
Salivary glands
Sjögren syndrome[4][10][11][12]Moderate, A710.2Autoantibodies: Anti-Ro (often present also in Systemic Lupus Erythematosus).

Digestive system

Organ/Tissue Type

Disease Name

Level of Acceptance for Autoimmunity Hypersensitivity

(I,II,III,IV)

ICD-9

Codes

Notes/Autoantibodies/Synonyms/Rare Variants
Digestive System
Autoimmune enteropathyModerate, X, Y
Coeliac disease[31][32][33]Moderate, A,EIV??579.0HLA-DQ8 and DQ2.5.
Autoantibodies: Anti-tissue transglutaminase antibodies, anti-endomysial IgA, anti-gliadin IgA.
Crohn's disease[34]Moderate, YIV555Innate immunity; Th17; Th1; ATG16L1; CARD15; XBP1.
Esophageal achalasia-Limited, X[35]
Microscopic colitisLimited, S558.9
Ulcerative colitis[4]Limited, AIV556

Tissue

Organ/Tissue type

Disease name

Level of acceptance for autoimmunity Hypersensitivity

(I,II,III,IV)

ICD-9

Codes

Notes/Autoantibodies/Synonyms/Rare variants
Blood
Antiphospholipid syndrome (APS, APLS)[4]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.
Synonym: Hughes syndrome.
Aplastic anemiaLimited, F, Y284
Autoimmune hemolytic anemiaModerate, M, AII283.0Complement activation.
Autoimmune lymphoproliferative syndromeModerate, A279.41TNFRSF6; defective Fas-CD95 apoptosis.
Synonym: Canale-Smith syndrome.
Autoimmune neutropeniaModerate, F, N288.09
Autoimmune thrombocytopenic purpura[4]Moderate, M, R, A287.31Autoantibodies: Anti gpIIb-IIIa or 1b-IX.
Synonym: Idiopathic Thrombocytopenic Purpura (ITP)
Cold agglutinin diseaseModerate, M, AII283.0Idiopathic or secondary to leukemia or infection.
Autoantibodies: IgM.
Synonym: Autoimmune hemolytic anemia.
Essential mixed cryoglobulinemiaLimited, C, Y273.2
Evans syndromeModerate, Y287.32Syndrome with a combination of hemolytic anemia and thrombocytopenic purpura
Pernicious anemia[36]Moderate, AII281.0Autoantibodies: Anti-parietal cell antibody.
Pure red cell aplasiaLimited, Y284.81
Thrombocytopenia[37][38]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
Adiposis dolorosa[39]Limited, L, S272.8Lipoid tissue.
Synonym: Dercum's disease
Adult-onset Still's disease[40]Moderate, Y714.2Macrophage migration inhibitory factor.
Autoantibodies: ANA.
Ankylosing spondylitis[16]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[41][42][43][44]Limited, C, YMMP3, TRLR2, TLR4, ERAP1.
A subtype of Juvenile Rheumatoid Arthritis.
Eosinophilic fasciitisLimited, F, A728.89Synonym: Shulman's syndrome
Felty syndrome[45]Strong, M, Y714.1
IgG4-related diseaseLimited, C, NCharacteristic histological features (storiform fibrosis, lymphoplasmacytic infiltrate, obliterative phlebitis) are required for definitive diagnosis.
Synonyms: IgG4-related systemic disease, IgG4-related sclerosing disease, IgG4-related systemic sclerosing disease, IgG4-related autoimmune disease, IgG4-associated multifocal systemic fibrosis, IgG4-associated disease, IgG4 syndrome, Hyper-IgG4 disease, Systemic IgG4-related plasmacytic syndrome.
Juvenile arthritis[40]Strong, M, R, Y714.30Autoantibodies: inconsistent ANA, Rheumatoid factor.
Synonyms: Juvenile rheumatoid arthritis, Juvenile idiopathic arthritis
Lyme disease (chronic)[46]Limited, L, T, N088.81
Mixed connective tissue disease (MCTD)[4]Moderate, M, A710.8HLA-DR4.
Autoantibodies: Anti-nuclear antibody, anti-U1-RNP.
Palindromic rheumatism[47]Limited, Y719.3Autoantibodies: Anti-cyclic citrullinated peptide antibodies (anti-CCP) and antikeratin antibodies (AKA).
Synonym: Hench-Rosenberg syndrome.
Parry–Romberg syndromeLimited, Y349.89351.8Autoantibodies: ANA.
Parsonage–Turner syndromeLimited, Y353.5
Psoriatic arthritis[48]Moderate, C, AIV?696.0HLA-B27.
Reactive arthritisLimited, C, F, Y099.3
Relapsing polychondritis[49]Strong, A733.99Synonyms: Atrophic polychondritis, systemic chondromalacia, chronic atrophic polychondritis, Meyenburg-Altherr-Uehlinger syndrome, generalized chondromalacia, systemic chondromalacia
Retroperitoneal fibrosisLimited, Y593.4
Rheumatic fever[50][51]Moderate, T, AII390Autoantibodies: Streptococcal M protein cross reacts with human myosin.
Rheumatoid arthritis[4]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[52][53][54]Limited, SIV135BTNL2; HLA-B7-DR15; HLA DR3-DQ2.
Schnitzler syndromeLimited, L, X, Y273.1IgM.
Systemic lupus erythematosus (SLE)[4][10][11][12][20][55]Strong, M, AIII695.4Autoantibodies: Anti-nuclear antibodies, anti-Ro (often present also in Sjögren syndrome). Eosinophilia.
Synonym: Lupus
Undifferentiated connective tissue disease (UCTD)Moderate, C, A710.9HLA-DR4.
Autoantibodies: anti-nuclear antibody.
Synonyms: Latent lupus, incomplete lupus
Muscle
Dermatomyositis[56][57]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.
Synonym: 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[4]Strong, M, AII358HA-B8 HLA-DR3 HLA-DR1.
Autoantibodies: Nicotinic acetylcholine receptor MuSK protein.
Neuromyotonia[58]Limited, F, SII?333.90Autoantibodies: Voltage-gated potassium channels.
Synonym: Isaacs' syndrome
Paraneoplastic cerebellar degeneration[59][60][61]Limited, YIV? II?334.9Autoantibodies: anti-Yo (anti-cdr-2 in purkinje fibers) anti-Hu, anti-Tr, antiglutamate receptor.
Polymyositis[56]Limited, F, A710.4Autoantibodies: IFN-gamma, IL-1, TNF-alpha, Anti-Jo1, Anti-SRP.
Nervous system
Acute disseminated encephalomyelitis (ADEM)Strong, M, T, A323.61323.81Synonyms: 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[62]Limited, N356.8
Anti-N-Methyl-D-Aspartate (Anti-NMDA) Receptor Encephalitis[63]Moderate, N
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[64]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[4]Strong, M, AIV357.0Autoantibodies: Anti-ganglioside, anti-GQ1b.
Synonyms: Miller–Fisher syndrome, Landry's paralysis.
Hashimoto's encephalopathy[4][65]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 set of different variants 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, pattern II[66][67]Strong, M, AIV340Autoantibody against potassium channel has been reported to present demyelination pattern II. Other cases present autoimmunity against MOG[68] and Anoctamin-2.[69] The three reported autoimmune variants belong to MS pattern II. Also involved HLA-DR2, PECAM-1, Anti-myelin basic protein. Autoantibodies: Anti-Kir4.1, Anti-MOG, Anti-ANO2 (heterogeneous).
Synonyms: Primary progressive multiple sclerosis, Relapsing-remitting multiple sclerosis, disseminated sclerosis, encephalomyelitis disseminata. Also interesting is Multiple sclerosis with HLA-DRB3 histocompatibility. In this case the auto-antigen has been found and is GDP-L-fucose synthase[70]
Progressive inflammatory neuropathyLimited, X, S356.4Similar to Guillain–Barré syndrome.
Autoantibodies: Anti-ganglioside antibodies:anti-GM1, anti-GD1a, anti-GQ1b.
Restless legs syndromeLimited, C, S333.94May occur in Sjögren syndrome, coeliac disease and rheumatoid arthritis, or in derangements of iron metabolism.
Stiff-person syndrome[71]Limited, S333.91GLRA1 (glycine receptor).
Autoantibodies: Glutamic acid decarboxylase (GAD).
Sydenham's choreaLimited, T, Y392
Transverse myelitisLimited, M, A323.82341.2
Eyes
Autoimmune retinopathy[72]Limited, X, N
Autoimmune uveitisModerate, F, A364Autoantibodies: HLAB-27.
Cogan syndromeLimited, F, Y370.52
Graves' ophthalmopathyModerate, M, N242.9
Intermediate uveitisLimited, L, Y364.3 Synonyms: Pars planitis, peripheral uveitis.
Ligneous conjunctivitisLimited, L, N372.39
Mooren's ulcerLimited, L, N370.07
Neuromyelitis optica[73][74]Limited, M, YII?341.0Autoantibodies: NMO-IgG aquaporin 4.
Synonym: Devic's disease.
Opsoclonus myoclonus syndrome[75]Limited, X, SIV?379.59Lymphocyte recruitment to CSF.
Optic neuritisLimited, C, Y377.30
ScleritisLimited, C, Y379.0
Susac's syndromeLimited, C, Y348.39Synonym: Retinocochleocerebral vasculopathy.
Sympathetic ophthalmiaLimited, I, Y360.11Autoantibodies: ocular antigens following trauma.
Tolosa–Hunt syndromeLimited, I, X, Y378.55
Ears
Autoimmune inner ear disease (AIED)[76]Limited, A388.8
Ménière's disease[77]Limited, YIII?386.00Autoantibodies: Major peripheral myelin protein P0.
Vascular system
Behçet's diseaseLimited, I, X, A136.1An immune-mediated systemic vasculitis; linkage to HLA-B51 (HLA-B27); very variable manifestations, with ulcers as common symptom.
Synonyms: Morbus Adamandiades-Behçet.
Rare variant: Hughes–Stovin syndrome.
Eosinophilic granulomatosis with polyangiitis (EGPA)[20][78]Limited, I, X, Y446.4Autoantibodies: p-ANCA Eosinophilia.
Giant cell arteritis[4]Limited, I, R, AIV446.5Synonyms: Cranial arteritis, temporal arteritis.
Granulomatosis with polyangiitis (GPA)[78]Strong, M, A446.4Autoantibodies: Anti-neutrophil cytoplasmic (cANCA).
IgA vasculitis (IgAV)[78]Limited, L, Y287.0Autoantibodies: IgA and complement component 3 (C3).
Synonyms: Anaphylactoid purpura, Henoch-Schonlein purpura, purpura rheumatica, Schönlein–Henoch purpura.
Kawasaki diseaseModerate, S,E[79]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 erythematosus.
Rheumatoid vasculitisModerate, C, N447.6A symptom of lupus.
Microscopic polyangiitis (MPA)Limited, Y446.0Binds to neutrophils causing them to degranulate and damages endothelium.
Autoantibodies: p-ANCA myeloperoxidase.
Synonyms: Microscopic polyarteritis, microscopic polyarteritis nodosa.
Polyarteritis nodosa (PAN)Limited, L, Y446.0Synonyms: Panarteritis nodosa, periarteritis nodosa, Kussmaul disease, Kussmaul–Maier disease.
Polymyalgia rheumaticaLimited, L, Y725
Urticarial vasculitis[80]Limited, X, YII?708.9 Clinically may resemble type I hypersensitivity.
Autoantibodies: anti C1q antibodies.
Vasculitis[19]Strong, I, M, F, AIII447.6Autoantibodies: ANCA (sometimes).
Systemic
Primary immunodeficiencyLimited, N

Autoimmune comorbidities

This list includes conditions that are not diseases but signs common to autoimmune disease. Some, such as chronic fatigue syndrome, are controversial.[2] 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 Name

Level of Acceptance for Autoimmunity Hypersensitivity

(I,II,III,IV)

ICD-9

Codes

Notes/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.
Interstitial lung diseaseComorbidity, NAssociated with several autoimmune connective tissue diseases.
POEMS syndrome[81]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).
Raynaud's phenomenonComorbidity, SSymptomatic of autoimmune diseases or autoimmune activity, but not a disease or a cause of disease.
Primary immunodeficiency[82]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.

Non-autoimmune

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 and is shown 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, direct or indirect, that it is an autoimmune disease.
Organ/Tissue Type

Disease Name

Level of Acceptance for Autoimmunity Hypersensitivity

(I,II,III,IV)

ICD-9

Codes

Notes/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.
AutismNot Autoimmune, NNo consistent evidence of association with maternal autoimmunity.
Autoimmune peripheral neuropathyNot Autoimmune, F, AA symptom of many diseases, including some that may be autoimmune. Not an autoimmune disease.
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[83]Not Autoimmune, SNo consistent evidence of association with autoimmunity.
Chronic obstructive pulmonary disease[84][85]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[86][87][88]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[89]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[90]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[91]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[92][93][94][95] Not Autoimmune, Y II? 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[96][97][98]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.

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