List of autoimmune diseases explained

This article provides a list of autoimmune diseases. These conditions, where the body's immune system mistakenly attacks its own cells, affect a range of organs and systems within the body. Each disorder is listed with the primary organ or body part that it affects and the associated autoantibodies that are typically found in people diagnosed with the condition. Each disorder is also categorized by its acceptance as an autoimmune condition into four levels: confirmed, probable, possible, and uncertain. This classification is based on the current scientific consensus and reflects the level of evidence supporting the autoimmune nature of the disorder. Lastly, the prevalence rate, specifically in the United States, is included to give a sense of how common each disorder is within the population.

Integumentary system

The integumentary system, composed of the skin, hair, nails, and associated glands, serves as a protective barrier between the body and the environment. It also plays a critical role in regulating body temperature and maintaining fluid balance.

DiseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Alopecia areataHair folliclesNone specificConfirmed2.1%[1] [2]
Autoimmune angioedemaSkinC1 inhibitorProbableLess than 5,000[3]
Autoimmune progesterone dermatitisSkinProgesteroneProbableExtremely rare[4]
Autoimmune urticariaSkinIgG against IgE or IgE receptorProbableNot well established[5] [6]
Bullous pemphigoidSkinAnti-BP180, Anti-BP230Confirmed6-30 per 100,000 (mostly older adults)[7]
Cicatricial pemphigoidMucous membranes, sometimes skinAnti-BP180, Anti-BP230ConfirmedRare[8]
Dermatitis herpetiformisSkinAnti-tissue transglutaminaseConfirmed10 per 100,000[9]
DermatomyositisSkin and musclesAnti-Jo1, Anti-Mi2, Anti-SRP, Anti-TIF1Confirmed9 in 1,000,000[10]
Discoid lupus erythematosusSkinANA, Anti-dsDNA, Anti-SmConfirmedPart of SLE prevalence (20-150 per 100,000)[11]
Epidermolysis bullosa acquisitaSkinAnti-type VII collagenConfirmedExtremely rare[12]
Erythema nodosumSkinNone specificPossibleNot well established[13]
Gestational pemphigoidSkinAnti-BP180, Anti-BP230ConfirmedRare[14]
Hidradenitis suppurativaSkinNone specificUncertain1-4%[15]
Lichen planusSkin, mucous membranesNone specificProbable1%[16]
Lichen sclerosusSkinNone specificProbableRare[17]
Linear IgA diseaseSkinAnti-epidermal basement membrane IgAConfirmedExtremely rare[18]
MorpheaSkinNone specificProbableNot well established[19]
PsoriasisSkinVarious, not specificConfirmed2-3%[20]
Pemphigus vulgarisSkin and mucous membranesAnti-desmoglein 3, Anti-desmoglein 1Confirmed1-5 per 100,000[21]
Scleroderma (systemic sclerosis)Skin, organsANA, anti-Scl-70, anti-centromereConfirmed240 per 1,000,000[22]
VitiligoSkinVarious, not specificConfirmed1%[23]

Digestive system

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Autoimmune enteropathySmall intestineAnti-enterocyte antibodiesProbableRare[24]
Autoimmune hepatitisLiverANA, ASMA, anti-LKM1Confirmed1 in 10,000 to 1 in 50,000[25]
Celiac diseaseSmall intestineAnti-tissue transglutaminase antibodies (tTG), Endomysial antibody (EMA), Deamidated gliadin peptide (DGP)Confirmed1 in 100[26]
Crohn's diseaseDigestive tractASCA, Anti-OmpC, Anti-CBir1, ANCAProbable201 per 100,000 adults[27]
Pernicious anemiaStomachAnti-IF, Anti-parietal cellConfirmed0.1%[28]
Ulcerative colitisColon and rectumpANCA, ASCAProbable249 per 100,000 adults[29]

Heart and vascular system

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Rheumatic heart diseaseHeart valvesAnti-streptolysin O (ASO), anti-DNase BConfirmedDeclining due to improved treatment of strep throat[30]
Kawasaki diseaseCoronary arteriesUnknownProbable20 per 100,000 children under age 5[31]
Giant cell arteritisLarge and medium arteries, can affect coronary arteriesNone specificConfirmed200 per 100,000 (over age 50)[32] [33]
Takayasu's arteritisLarge arteries, including the aortaNone specificConfirmedRare, more common in East Asia[34]
Behçet's diseaseSmall to large vessels in mouth, genitals, eyes, skinNone specificProbable[35]
Eosinophilic granulomatosis with polyangiitis (EGPA)Small to medium vessels in respiratory tract, skin, heart, kidneys, nervesANCAConfirmed[36]
Granulomatosis with polyangiitis (GPA)Small to medium vessels in respiratory tract, kidneysc-ANCA/PR3-ANCAConfirmed
IgA vasculitis (IgAV)Small vessels in skin, joints, kidneys, gastrointestinal tractIgA immune complexesProbable[37]
Leukocytoclastic vasculitisSmall vessels in skinVarious immune complexesProbable[38]
Lupus vasculitisSmall to medium vessels in multiple organsANA, anti-dsDNA, anti-Smith, othersConfirmed[39]
Rheumatoid vasculitisSmall to medium vessels in skin, nerves, eyes, heartRheumatoid factor, ACPAProbable[40]
Microscopic polyangiitis (MPA)Small vessels in kidneys, lungs, nerves, skinp-ANCA/MPO-ANCAConfirmed[41]
Polyarteritis nodosa (PAN)Medium to small vessels in kidneys, skin, muscles, joints, nervesNone specificProbable[42]
Polymyalgia rheumaticaLarge to medium vessels in shoulders, hipsNone specificPossible[43]
Urticarial vasculitisSmall vessels in skinC1q antibodiesProbable[44]
VasculitisAll vessel sizes in multiple organsDepends on specific typeUncertain[45]

Urinary system

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Goodpasture syndromeKidneys, lungsAnti-GBM antibodiesConfirmed1 per million people[46]
IgA nephropathyKidneysIgA autoantibodiesConfirmed3.5 per 100,000[47]
Membranous nephropathyKidneysAnti-PLA2R antibodiesConfirmed10 per 100,000[48]
Lupus nephritisKidneysAnti-dsDNA, Anti-Sm, Anti-nuclear antibodiesConfirmedUp to 60% of those with Lupus[49]
Interstitial nephritisKidneysVarious autoantibodiesProbableVaries widely, often drug-induced[50]
Interstitial cystitisBladderAnti-urothelial and anti-nuclear antibodiesProbable100-450 per 100,000 women, less common in men[51]
Primary sclerosing cholangitisBile ducts, can affect gallbladderP-ANCA, Anti Smooth Muscle Antibodies (ASMA)Confirmed1 per 100,000[52]

Nervous system

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Acute disseminated encephalomyelitisCentral nervous systemUnknownConfirmed0.8 per 100,000[53]
Acute motor axonal neuropathyPeripheral nervesAnti-GM1ConfirmedPart of Guillain-Barré syndrome prevalence
Anti-NMDA receptor encephalitisBrainAnti-NMDA receptorConfirmed1.5 per million[54]
Autoimmune encephalitisBrainVarious, depending on subtype (e.g., NMDA receptor antibodies, LGI1 antibodies)ConfirmedRare[55]
Balo concentric sclerosisCentral nervous systemUnknownProbableRare[56]
Bickerstaff's encephalitisBrainAnti-GQ1bConfirmedRare[57]
Chronic inflammatory demyelinating polyneuropathyPeripheral nerves Various, including anti-MAGConfirmed1-2 per 100,000[58]
Guillain-Barré syndromePeripheral nervesVarious, including anti-GM1, anti-GD1aConfirmed1-2 per 100,000[59]
Hashimoto's encephalopathyBrainAnti-thyroid (TPO, Tg)ProbableRare[60]
Idiopathic inflammatory demyelinating diseasesCentral nervous systemVariesProbableVaries by specific disease[61]
Lambert–Eaton myasthenic syndromeNeuromuscular junction (affecting both CNS and PNS)Anti-VGCCConfirmed0.5-2 per million[62]
Multiple sclerosisCentral nervous systemUnknown, but Oligoclonal bands often present in CSFConfirmed90 per 100,000[63]
Myasthenia gravisNeuromuscular junction (affecting both CNS and PNS)Anti-AChR, anti-MuSKConfirmed20 per 100,000[64]
Neuromyelitis optica (Devic's disease)/NMOSDOptic nerves and spinal cordAQP4-IgG (NMO-IgG)Confirmed0.5 - 4 per 100,000[65]
Restless legs syndromeCentral nervous system (thought to involve dopaminergic pathways)UnknownUncertain5-15% (more common in older adults)[66]
Stiff-person syndromeCentral nervous systemAnti-GAD, anti-amphiphysinConfirmedRare[67]
Sydenham's choreaBrainAnti-basal gangliaConfirmedRare (linked to Group A streptococcal infection)[68]
Transverse myelitisSpinal cordVarious, including anti-AQP4Probable1-8 per million[69]
Undifferentiated connective tissue disease (UCTD)VariousANA (antinuclear autoantibody) (HEp-2 cells)Confirmed2 per 100,000[70]

Endocrine system

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Addison's diseaseAdrenal glands21-hydroxylase antibodiesConfirmed0.93-1.4 per 10,000[71]
Autoimmune oophoritisOvariesAnti-ovarian antibodiesProbableRare[72]
Autoimmune orchitisTestesAnti-sperm antibodiesProbableRare[73]
Autoimmune pancreatitisPancreasIgG4, Anti-CA2 antibodiesConfirmed0.82-1.3 per 100,000[74]
Autoimmune polyendocrine syndrome type 1 (APS1)Multiple endocrine organsVarious autoantibodies depending on the organs affectedConfirmed1 in 100,000 to 200,000[75]
Autoimmune polyendocrine syndrome type 2 (APS2)Multiple endocrine organsVarious autoantibodies depending on the organs affectedConfirmed1 in 20,000[76]
Autoimmune polyendocrine syndrome type 3 (APS3)Multiple endocrine organsVarious autoantibodies depending on the organs affectedConfirmedRare
Diabetes mellitus type 1PancreasAnti-insulin, anti-IA-2, anti-GAD, anti-ZnT8 antibodiesConfirmed1.6 per 1,000[77]
EndometriosisEndometriumAnti-endometrial antibodiesProbable6-10% of women of reproductive age[78]
Graves' diseaseThyroid glandTSI, TPO, TG antibodiesConfirmed1.2% of the population[79]
Hashimoto's thyroiditisThyroid glandTPO, TG antibodiesConfirmed5% of the population[80]
Ord's thyroiditisThyroid glandTPO, TG antibodiesConfirmedRare[81]
Sjögren syndromeExocrine glands (salivary and lacrimal glands)Anti-SSA/Ro, Anti-SSB/La antibodiesConfirmed0.1-4% of the population[82]

Respiratory system

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Goodpasture syndromeConfirmed
Eosinophilic granulomatosis with polyangiitis (EGPA)Confirmed
Granulomatosis with polyangiitis (GPA)Confirmed
Idiopathic pulmonary fibrosisLungsNone specificPossible20 per 100,000 (men), 13 per 100,000 (women)[83]
Interstitial lung diseaseLungsDepends on the subtype (e.g. Anti-Jo1 in Anti-synthetase syndrome)Probable31.5 per 100,000 (men), 26.1 per 100,000 (women)[84]
Pulmonary alveolar proteinosisLungsAnti-GM-CSF antibodiesConfirmed6.2 per million[85]
Rheumatoid lung diseaseLungsRheumatoid factor, Anti-CCP antibodiesConfirmedPart of RA prevalence (about 1%)[86]
SarcoidosisLungs and other organsNone specificConfirmed10 - 40 per 100,000[87]

Blood

Autoimmune diseaseAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Autoimmune hemolytic anemiaAnti-red blood cell antibodiesConfirmed1-3 per 100,000[88]
Immune thrombocytopeniaAnti-platelet antibodiesConfirmed3.3 per 100,000 (adults), 50 per 100,000 (children)[89]
Thrombotic thrombocytopenic purpuraADAMTS13 autoantibodiesConfirmed1-2 per million[90]
Antiphospholipid syndromeAntiphospholipid antibodiesConfirmed40-50 per 100,000[91]
Paroxysmal nocturnal hemoglobinuriaNone specific, mutation causes self-cells to become susceptible to attackPossible1-2 per million[92]

Reproductive system

The reproductive system is responsible for the production and regulation of sex hormones, the formation of germ cells, and the nurturing of fertilized eggs. In women, it includes structures such as ovaries, fallopian tubes, a uterus, and a vagina, while in men, it includes testes, vas deferens, seminal vesicles, prostate, and the penis. Autoimmune diseases of the reproductive system can affect both male and female fertility and reproductive health.

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Autoimmune orchitisTestesAnti-sperm antibodiesProbableNot well established
Autoimmune oophoritisOvariesAnti-ovarian antibodiesProbableNot well established
EndometriosisUterus, ovaries, and pelvic tissueVarious, including anti-endometrial antibodiesProbableApprox. 10% of women of reproductive age[93]
Premature ovarian failureOvariesAnti-ovarian antibodies, Anti-adrenal antibodiesConfirmed1% of women under 40 years[94]

Eyes

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune diseasePrevalence rate (US)
Autoimmune retinopathyRetinaVariousConfirmed[95]
Autoimmune uveitisUveaVariousConfirmed[96]
Cogan syndromeInner ear and eyeNone specificProbable[97]
Graves' ophthalmopathyEye muscles and connective tissueTSH receptor antibodiesConfirmed[98]
Intermediate uveitisUvea (pars plana)VariousProbable[99]
Ligneous conjunctivitisConjunctivaPlasminogen deficiencyPossibleRare[100]
Mooren's ulcerCorneaNone specificProbableRare[101]
Neuromyelitis opticaOptic nerve and spinal cordAnti-AQP4Confirmed[102]
Opsoclonus myoclonus syndromeCentral nervous system, eye movement controlAnti-neuronal antibodiesPossible[103]
Optic neuritisOptic nerveVariousConfirmed[104]
ScleritisScleraVariousPossible[105]
Susac's syndromeRetina, cochlea, and brainNone specificProbable[106]
Sympathetic ophthalmiaUveal tractVariousProbableRare[107]
Tolosa–Hunt syndromeOrbitNone specificUncertainRare[108]

Muscular system

These autoimmune diseases are primarily associated with the muscles and neuromuscular function.

Autoimmune diseasePrimary organ/body part affectedAutoantibodiesAcceptance as an autoimmune disorderPrevalence rate (US)
DermatomyositisConfirmed
FibromyalgiaMusculoskeletal system, pain perceptionNone specificPossible[109]
Inclusion body myositisProximal and distal musclesNone specificProbable[110]
MyositisSkeletal musclesVarious, depends on subtypeConfirmed[111]
Myasthenia gravisVoluntary muscles, neuromuscular junctionsAnti-acetylcholine receptor, Anti-MuSKConfirmed[112]
NeuromyotoniaPeripheral nerves affecting muscle controlAnti-voltage-gated potassium channelsProbable[113]
Paraneoplastic cerebellar degenerationCentral nervous system, cerebellumAnti-Yo, Anti-Hu, Anti-Ri, othersConfirmed[114]
PolymyositisProximal skeletal musclesAnti-Jo-1, Anti-SRP, othersConfirmed[115]

Autoimmune comorbidities

This list includes conditions that are not diseases, but symptoms or syndromes common to autoimmune disease.[116]

Non-autoimmune

At this time, there is not sufficient evidence to indicate that these diseases are caused by autoimmunity.

Disease Reason not believed to be autoimmune
AgammaglobulinemiaAn immune system disorder but not an autoimmune disease.
AmyloidosisNo consistent evidence of association with autoimmunity.
Amyotrophic lateral sclerosisNo consistent evidence of association with autoimmunity.
Anti-tubular basement membrane nephritisNo consistent evidence of association with autoimmunity.
Atopic allergyA hypersensitivity.
Atopic dermatitisA hypersensitivity.
AutismNo consistent evidence of association with maternal autoimmunity.
Blau syndromeOverlaps both sarcoidosis and granuloma annulare. No evidence of association with autoimmunity.
CancerNo consistent evidence of association with autoimmunity.
Castleman's diseaseAn immune system disorder but not an autoimmune disease.
Chagas diseaseNo consistent evidence of association with autoimmunity.[119]
Chronic obstructive pulmonary diseaseNo consistent evidence of association with autoimmunity.[120] [121]
Chronic recurrent multifocal osteomyelitisLPIN2, D18S60. Synonyms: Majeed syndrome.
Complement component 2 deficiencyPossibly symptomatic of autoimmune diseases, but not a disease.
Congenital heart blockMay be related to autoimmune activity in the mother.
Contact dermatitisA hypersensitivity.
Cushing's syndromeNo consistent evidence of association with autoimmunity.
Cutaneous leukocytoclastic angiitisNo consistent evidence of association with autoimmunity.
Dego's diseaseNo consistent evidence of association with autoimmunity.
Eosinophilic gastroenteritisPossibly a hypersensitivity.
Eosinophilic pneumoniaA class of diseases, some of which may be autoimmune.
Erythroblastosis fetalisMother's immune system attacks fetus. An immune system disorder but not autoimmune.
Fibrodysplasia ossificans progressivaPossibly an immune system disorder but not autoimmune.
Gastrointestinal pemphigoidNo consistent evidence of association with autoimmunity.
HypogammaglobulinemiaAn immune system disorder but not autoimmune.
Idiopathic giant-cell myocarditisNo consistent evidence of autoimmune cause though the disease has been found comorbid with other autoimmune diseases.[122]
Idiopathic pulmonary fibrosisAutoantibodies: SFTPA1, SFTPA2, TERT, and TERC.
IgA nephropathyAn immune system disorder but not an autoimmune disease.
IPEX syndromeA genetic mutation in FOXP3 that leads to autoimmune diseases, but not an autoimmune disorder itself.
Ligneous conjunctivitisNo consistent evidence of association with autoimmunity.
Majeed syndromeNo consistent evidence of association with autoimmunity.
NarcolepsyNo evidence of association with autoimmunity. Research not reproducible.[123] [124] [125] [126]
Rasmussen's encephalitisNo consistent evidence of association with autoimmunity.
SchizophreniaNo consistent evidence of association with autoimmunity.[127] [128] [129]
Serum sicknessA hypersensitivity.
SpondyloarthropathyNo consistent evidence of association with autoimmunity.
Sweet's syndromeNo consistent evidence of association with autoimmunity.
Takayasu's arteritisNo consistent evidence of association with autoimmunity.
Undifferentiated spondyloarthropathy

See also

External links

Notes and References

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