Wikipedia

List of autoimmune diseases

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.

  • Confirmed - Used for conditions that have strong, well-established evidence of autoimmune etiology.
  • Probable - Used for conditions where there is substantial evidence of autoimmune involvement, but the scientific consensus may not be as strong as for those in the 'confirmed' category.
  • Possible - Used for conditions that have some evidence pointing towards autoimmune involvement, but it's not yet clear or there is ongoing debate.
  • Uncertain - Used for conditions where the evidence of autoimmune involvement is limited or contested.
Dermatosis in Crohn's disease
Demyelination in MS
PAS stain of lupus nephritis
Autoimmune urticaria
Proptosis in Graves' disease

Integumentary system

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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.

Disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Alopecia areata Hair follicles None specific Confirmed 2.1% [1][2]
Autoimmune angioedema Skin C1 inhibitor Probable Less than 5,000 [3]
Autoimmune progesterone dermatitis Skin Progesterone Probable Extremely rare [4]
Autoimmune urticaria Skin IgG against IgE or IgE receptor Probable Not well established [5][6]
Bullous pemphigoid Skin Anti-BP180, Anti-BP230 Confirmed 6-30 per 100,000 (mostly older adults) [7]
Cicatricial pemphigoid Mucous membranes, sometimes skin Anti-BP180, Anti-BP230 Confirmed Rare [8]
Dermatitis herpetiformis Skin Anti-tissue transglutaminase Confirmed 10 per 100,000 [9]
Dermatomyositis Skin and muscles Anti-Jo1, Anti-Mi2, Anti-SRP, Anti-TIF1 Confirmed 9 in 1,000,000 [10]
Discoid lupus erythematosus Skin ANA, Anti-dsDNA, Anti-Sm Confirmed Part of SLE prevalence (20-150 per 100,000) [11]
Epidermolysis bullosa acquisita Skin Anti-type VII collagen Confirmed Extremely rare [12]
Erythema nodosum Skin None specific Possible Not well established [13]
Gestational pemphigoid Skin Anti-BP180, Anti-BP230 Confirmed Rare [14]
Hidradenitis suppurativa Skin None specific Uncertain 1-4% [15]
Lichen planus Skin, mucous membranes None specific Probable 1% [16]
Lichen sclerosus Skin None specific Probable Rare [17]
Linear IgA disease Skin Anti-epidermal basement membrane IgA Confirmed Extremely rare [18]
Morphea Skin None specific Probable Not well established [19]
Psoriasis Skin Various, not specific Confirmed 2-3% [20]
Pemphigus vulgaris Skin and mucous membranes Anti-desmoglein 3, Anti-desmoglein 1 Confirmed 1-5 per 100,000 [21]
Scleroderma (systemic sclerosis) Skin, organs ANA, anti-Scl-70, anti-centromere Confirmed 240 per 1,000,000 [22]
Vitiligo Skin Various, not specific Confirmed 1% [23]

Digestive system

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Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Autoimmune enteropathy Small intestine Anti-enterocyte antibodies Probable Rare [24]
Autoimmune hepatitis Liver ANA, ASMA, anti-LKM1 Confirmed 1 in 10,000 to 1 in 50,000 [25]
Celiac disease Small intestine Anti-tissue transglutaminase antibodies (tTG), Endomysial antibody (EMA), Deamidated gliadin peptide (DGP) Confirmed 1 in 100 [26]
Crohn's disease Digestive tract ASCA, Anti-OmpC, Anti-CBir1, ANCA Probable 201 per 100,000 adults [27]
Pernicious anemia Stomach Anti-IF, Anti-parietal cell Confirmed 0.1% [28]
Ulcerative colitis Colon and rectum pANCA, ASCA Probable 249 per 100,000 adults [29]

Heart and vascular system

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Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Rheumatic heart disease Heart valves Anti-streptolysin O (ASO), anti-DNase B Confirmed Declining due to improved treatment of strep throat [30]
Kawasaki disease Coronary arteries Unknown Probable 20 per 100,000 children under age 5 [31]
Giant cell arteritis Large and medium arteries, can affect coronary arteries None specific Confirmed 200 per 100,000 (over age 50) [32][33]
Takayasu's arteritis Large arteries, including the aorta None specific Confirmed Rare, more common in East Asia [33][34]
Behçet's disease Small to large vessels in mouth, genitals, eyes, skin None specific Probable [35]
Eosinophilic granulomatosis with polyangiitis (EGPA) Small to medium vessels in respiratory tract, skin, heart, kidneys, nerves ANCA Confirmed [36]
Granulomatosis with polyangiitis (GPA) Small to medium vessels in respiratory tract, kidneys c-ANCA/PR3-ANCA Confirmed [36]
IgA vasculitis (IgAV) Small vessels in skin, joints, kidneys, gastrointestinal tract IgA immune complexes Probable [37]
Leukocytoclastic vasculitis Small vessels in skin Various immune complexes Probable [38]
Lupus vasculitis Small to medium vessels in multiple organs ANA, anti-dsDNA, anti-Smith, others Confirmed [39]
Rheumatoid vasculitis Small to medium vessels in skin, nerves, eyes, heart Rheumatoid factor, ACPA Probable [40]
Microscopic polyangiitis (MPA) Small vessels in kidneys, lungs, nerves, skin p-ANCA/MPO-ANCA Confirmed [41]
Polyarteritis nodosa (PAN) Medium to small vessels in kidneys, skin, muscles, joints, nerves None specific Probable [42]
Polymyalgia rheumatica Large to medium vessels in shoulders, hips None specific Possible [43]
Urticarial vasculitis Small vessels in skin C1q antibodies Probable [44]
Vasculitis All vessel sizes in multiple organs Depends on specific type Uncertain [45]

Urinary system

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Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Goodpasture syndrome Kidneys, lungs Anti-GBM antibodies Confirmed 1 per million people [46]
IgA nephropathy Kidneys IgA autoantibodies Confirmed 3.5 per 100,000 [47]
Membranous nephropathy Kidneys Anti-PLA2R antibodies Confirmed 10 per 100,000 [48]
Lupus nephritis Kidneys Anti-dsDNA, Anti-Sm, Anti-nuclear antibodies Confirmed Up to 60% of those with Lupus [49]
Interstitial nephritis Kidneys Various autoantibodies Probable Varies widely, often drug-induced [50]
Interstitial cystitis Bladder Anti-urothelial and anti-nuclear antibodies Probable 100-450 per 100,000 women, less common in men [51]
Primary sclerosing cholangitis Bile ducts, can affect gallbladder P-ANCA, Anti Smooth Muscle Antibodies (ASMA) Confirmed 1 per 100,000 [52]

Nervous system

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Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Acute disseminated encephalomyelitis Central nervous system Unknown Confirmed 0.8 per 100,000 [53]
Acute motor axonal neuropathy Peripheral nerves Anti-GM1 Confirmed Part of Guillain-Barré syndrome prevalence [54]
Anti-NMDA receptor encephalitis Brain Anti-NMDA receptor Confirmed 1.5 per million [55]
Autoimmune encephalitis Brain Various, depending on subtype (e.g., NMDA receptor antibodies, LGI1 antibodies) Confirmed Rare [56]
Balo concentric sclerosis Central nervous system Unknown Probable Rare [57]
Bickerstaff's encephalitis Brain Anti-GQ1b Confirmed Rare [58]
Chronic inflammatory demyelinating polyneuropathy Peripheral nerves Various, including anti-MAG Confirmed 1-2 per 100,000 [59]
Guillain-Barré syndrome Peripheral nerves Various, including anti-GM1, anti-GD1a Confirmed 1-2 per 100,000 [60]
Hashimoto's encephalopathy Brain Anti-thyroid (TPO, Tg) Probable Rare [61]
Idiopathic inflammatory demyelinating diseases Central nervous system Varies Probable Varies by specific disease [62]
Lambert–Eaton myasthenic syndrome Neuromuscular junction (affecting both CNS and PNS) Anti-VGCC Confirmed 0.5-2 per million [63]
Multiple sclerosis Central nervous system Unknown, but Oligoclonal bands often present in CSF Confirmed 90 per 100,000 [64]
Myasthenia gravis Neuromuscular junction (affecting both CNS and PNS) Anti-AChR, anti-MuSK Confirmed 20 per 100,000 [65]
Neuromyelitis optica (Devic's disease)/NMOSD Optic nerves and spinal cord AQP4-IgG (NMO-IgG) Confirmed 0.5 - 4 per 100,000 [66]
Restless legs syndrome Central nervous system (thought to involve dopaminergic pathways) Unknown Uncertain 5-15% (more common in older adults) [67]
Stiff-person syndrome Central nervous system Anti-GAD, anti-amphiphysin Confirmed Rare [68]
Sydenham's chorea Brain Anti-basal ganglia Confirmed Rare (linked to Group A streptococcal infection) [69]
Transverse myelitis Spinal cord Various, including anti-AQP4 Probable 1-8 per million [70]
Undifferentiated connective tissue disease (UCTD) Various ANA (antinuclear autoantibody) (HEp-2 cells) Confirmed 2 per 100,000 [71]

Endocrine system

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Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Addison's disease Adrenal glands 21-hydroxylase antibodies Confirmed 0.93-1.4 per 10,000 [72]
Autoimmune oophoritis Ovaries Anti-ovarian antibodies Probable Rare [73]
Autoimmune orchitis Testes Anti-sperm antibodies Probable Rare [74]
Autoimmune pancreatitis Pancreas IgG4, Anti-CA2 antibodies Confirmed 0.82-1.3 per 100,000 [75]
Autoimmune polyendocrine syndrome type 1 (APS1) Multiple endocrine organs Various autoantibodies depending on the organs affected Confirmed 1 in 100,000 to 200,000 [76]
Autoimmune polyendocrine syndrome type 2 (APS2) Multiple endocrine organs Various autoantibodies depending on the organs affected Confirmed 1 in 20,000 [77]
Autoimmune polyendocrine syndrome type 3 (APS3) Multiple endocrine organs Various autoantibodies depending on the organs affected Confirmed Rare [77]
Diabetes mellitus type 1 Pancreas Anti-insulin, anti-IA-2, anti-GAD, anti-ZnT8 antibodies Confirmed 1.6 per 1,000 [78]
Endometriosis Endometrium Anti-endometrial antibodies Probable 6-10% of women of reproductive age [79]
Graves' disease Thyroid gland TSI, TPO, TG antibodies Confirmed 1.2% of the population [80]
Hashimoto's thyroiditis Thyroid gland TPO, TG antibodies Confirmed 5% of the population [81]
Ord's thyroiditis Thyroid gland TPO, TG antibodies Confirmed Rare [82]
Sjögren syndrome Exocrine glands (salivary and lacrimal glands) Anti-SSA/Ro, Anti-SSB/La antibodies Confirmed 0.1-4% of the population [83]

Respiratory system

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Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Goodpasture syndrome See urinary system [a] See urinary system Confirmed See urinary system —
Eosinophilic granulomatosis with polyangiitis (EGPA) See vascular system [b] See vascular system Confirmed See vascular system —
Granulomatosis with polyangiitis (GPA) See vascular system [c] See vascular system Confirmed See vascular system —
Idiopathic pulmonary fibrosis Lungs None specific Possible 20 per 100,000 (men), 13 per 100,000 (women) [84]
Interstitial lung disease Lungs Depends on the subtype (e.g. Anti-Jo1 in Anti-synthetase syndrome) Probable 31.5 per 100,000 (men), 26.1 per 100,000 (women) [85]
Pulmonary alveolar proteinosis Lungs Anti-GM-CSF antibodies Confirmed 6.2 per million [86]
Rheumatoid lung disease Lungs Rheumatoid factor, Anti-CCP antibodies Confirmed Part of RA prevalence (about 1%) [87]
Sarcoidosis Lungs and other organs None specific Confirmed 10 - 40 per 100,000 [88]

Blood

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Autoimmune disease Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Autoimmune hemolytic anemia Anti-red blood cell antibodies Confirmed 1-3 per 100,000 [89]
Immune thrombocytopenia Anti-platelet antibodies Confirmed 3.3 per 100,000 (adults), 50 per 100,000 (children) [90]
Thrombotic thrombocytopenic purpura ADAMTS13 autoantibodies Confirmed 1-2 per million [91]
Antiphospholipid syndrome Antiphospholipid antibodies Confirmed 40-50 per 100,000 [92]
Paroxysmal nocturnal hemoglobinuria None specific, mutation causes self-cells to become susceptible to attack Possible 1-2 per million [93]

Reproductive system

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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 disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Autoimmune orchitis Testes Anti-sperm antibodies Probable Not well established [94]
Autoimmune oophoritis Ovaries Anti-ovarian antibodies Probable Not well established [94]
Endometriosis Uterus, ovaries, and pelvic tissue Various, including anti-endometrial antibodies Probable Approx. 10% of women of reproductive age [95]
Premature ovarian failure Ovaries Anti-ovarian antibodies, Anti-adrenal antibodies Confirmed 1% of women under 40 years [96]

Eyes

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Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disease Prevalence rate (US) Cit.
Autoimmune retinopathy Retina Various Confirmed [97]
Autoimmune uveitis Uvea Various Confirmed [98]
Cogan syndrome Inner ear and eye None specific Probable [99]
Graves' ophthalmopathy Eye muscles and connective tissue TSH receptor antibodies Confirmed [100]
Intermediate uveitis Uvea (pars plana) Various Probable [101]
Ligneous conjunctivitis Conjunctiva Plasminogen deficiency Possible Rare [102]
Mooren's ulcer Cornea None specific Probable Rare [103]
Neuromyelitis optica Optic nerve and spinal cord Anti-AQP4 Confirmed [104]
Opsoclonus myoclonus syndrome Central nervous system, eye movement control Anti-neuronal antibodies Possible [105]
Optic neuritis Optic nerve Various Confirmed [106]
Scleritis Sclera Various Possible [107]
Susac's syndrome Retina, cochlea, and brain None specific Probable [108]
Sympathetic ophthalmia Uveal tract Various Probable Rare [109]
Tolosa–Hunt syndrome Orbit None specific Uncertain Rare [110]

Musculoskeletal system

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

Autoimmune disease Primary organ/body part affected Autoantibodies Acceptance as an autoimmune disorder Prevalence rate (US) Cit.
Dermatomyositis See integumentary system [d] See integumentary system Confirmed See integumentary system —
Fibromyalgia Musculoskeletal system, pain perception None specific Possible [111]
Inclusion body myositis Proximal and distal muscles None specific Probable [112]
Myositis Skeletal muscles Various, depends on subtype Confirmed [113]
Myasthenia gravis Voluntary muscles, neuromuscular junctions Anti-acetylcholine receptor, Anti-MuSK Confirmed [114]
Neuromyotonia Peripheral nerves affecting muscle control Anti-voltage-gated potassium channels Probable [115]
Paraneoplastic cerebellar degeneration Central nervous system, cerebellum Anti-Yo, Anti-Hu, Anti-Ri, others Confirmed [116]
Rheumatoid arthritis Big and small joints of extremities, temporomandibular joints Citrullinated proteins Confirmed 0.5 - 1%
Polymyositis Proximal skeletal muscles Anti-Jo-1, Anti-SRP, others Confirmed [117]

Autoimmune comorbidities

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This list includes conditions that are not diseases, but symptoms or syndromes common to autoimmune disease.[118]

Non-autoimmune

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

Disease Reason not believed to be autoimmune Cit.
Agammaglobulinemia An immune system disorder but not an autoimmune disease.
Amyloidosis No consistent evidence of association with autoimmunity.
Amyotrophic lateral sclerosis No consistent evidence of association with autoimmunity.
Anti-tubular basement membrane nephritis No consistent evidence of association with autoimmunity.
Atopic allergy A hypersensitivity.
Atopic dermatitis A hypersensitivity.
Autism No consistent evidence of association with maternal autoimmunity.
Blau syndrome Overlaps both sarcoidosis and granuloma annulare. No evidence of association with autoimmunity.
Cancer No consistent evidence of association with autoimmunity.
Castleman's disease An immune system disorder but not an autoimmune disease.
Chagas disease No consistent evidence of association with autoimmunity. [121]
Chronic obstructive pulmonary disease No consistent evidence of association with autoimmunity. [122][123]
Chronic recurrent multifocal osteomyelitis LPIN2, D18S60. Synonyms: Majeed syndrome.
Complement component 2 deficiency Possibly symptomatic of autoimmune diseases, but not a disease.
Congenital heart block May be related to autoimmune activity in the mother.
Contact dermatitis A hypersensitivity.
Cushing's syndrome No consistent evidence of association with autoimmunity.
Cutaneous leukocytoclastic angiitis No consistent evidence of association with autoimmunity.
Dego's disease No consistent evidence of association with autoimmunity.
Eosinophilic gastroenteritis Possibly a hypersensitivity.
Eosinophilic pneumonia A class of diseases, some of which may be autoimmune.
Erythroblastosis fetalis Mother's immune system attacks fetus. An immune system disorder but not autoimmune.
Fibrodysplasia ossificans progressiva Possibly an immune system disorder but not autoimmune.
Gastrointestinal pemphigoid No consistent evidence of association with autoimmunity.
Hypogammaglobulinemia An immune system disorder but not autoimmune.
Idiopathic giant-cell myocarditis No consistent evidence of autoimmune cause though the disease has been found comorbid with other autoimmune diseases. [124]
Idiopathic pulmonary fibrosis Autoantibodies: SFTPA1, SFTPA2, TERT, and TERC.
IgA nephropathy An immune system disorder but not an autoimmune disease.
IPEX syndrome A genetic mutation in FOXP3 that leads to autoimmune diseases, but not an autoimmune disorder itself.
Ligneous conjunctivitis No consistent evidence of association with autoimmunity.
Majeed syndrome No consistent evidence of association with autoimmunity.
Narcolepsy No evidence of association with autoimmunity. Research not reproducible.[needs update] [125][126][127][128]
Rasmussen's encephalitis No consistent evidence of association with autoimmunity.
Schizophrenia No consistent evidence of association with autoimmunity. [129][130][131]
Serum sickness A hypersensitivity.
Spondyloarthropathy No consistent evidence of association with autoimmunity.
Sweet's syndrome No consistent evidence of association with autoimmunity.
Takayasu's arteritis No consistent evidence of association with autoimmunity.
Undifferentiated spondyloarthropathy

See also

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Notes

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  1. ^ For detailed information on Goodpasture syndrome, including its impact on the respiratory system, refer to its entry in the urinary system section.
  2. ^ For detailed information on EGPA, including its impact on the respiratory system, refer to its entry in the vascular system section.
  3. ^ For detailed information on GPA, including its impact on the respiratory system, refer to its entry in the vascular system section.
  4. ^ For detailed information on dermatomyositis, including its impact on the musclar system, refer to its entry in the integumentary system section.

References

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