# 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.

## 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.
| Disease | Primary organ/body part affected | Autoantibodies | Acceptance as an autoimmune disease | Prevalence rate (US) |
| Alopecia areata | Hair follicles | None specific | Confirmed | 2.1% |
| Autoimmune angioedema | Skin | C1 inhibitor | Probable | Less than 5,000 |
| Autoimmune progesterone dermatitis | Skin | Progesterone | Probable | Extremely rare |
| Autoimmune urticaria | Skin | IgG against IgE or IgE receptor | Probable | Not well established |
| Bullous pemphigoid | Skin | Anti-BP180, Anti-BP230 | Confirmed | 6-30 per 100,000 (mostly older adults) |
| Cicatricial pemphigoid | Mucous membranes, sometimes skin | Anti-BP180, Anti-BP230 | Confirmed | Rare |
| Dermatitis herpetiformis | Skin | Anti-tissue transglutaminase | Confirmed | 10 per 100,000 |
| Dermatomyositis | Skin and muscles | Anti-Jo1, Anti-Mi2, Anti-SRP, Anti-TIF1 | Confirmed | 9 in 1,000,000 |
| Discoid lupus erythematosus | Skin | ANA, Anti-dsDNA, Anti-Sm | Confirmed | Part of SLE prevalence (20-150 per 100,000) |
| Epidermolysis bullosa acquisita | Skin | Anti-type VII collagen | Confirmed | Extremely rare |
| Erythema nodosum | Skin | None specific | Possible | Not well established |
| Gestational pemphigoid | Skin | Anti-BP180, Anti-BP230 | Confirmed | Rare |
| Hidradenitis suppurativa | Skin | None specific | Uncertain | 1-4% |
| Lichen planus | Skin, mucous membranes | None specific | Probable | 1% |
| Lichen sclerosus | Skin | None specific | Probable | Rare |
| Linear IgA disease | Skin | Anti-epidermal basement membrane IgA | Confirmed | Extremely rare |
| Morphea | Skin | None specific | Probable | Not well established |
| Psoriasis | Skin | Various, not specific | Confirmed | 2-3% |
| Pemphigus vulgaris | Skin and mucous membranes | Anti-desmoglein 3, Anti-desmoglein 1 | Confirmed | 1-5 per 100,000 |
| Scleroderma (systemic sclerosis) | Skin, organs | ANA, anti-Scl-70, anti-centromere | Confirmed | 240 per 1,000,000 |
| Sjögren syndrome | Exocrine glands (salivary and lacrimal glands) | Anti-SSA/Ro, Anti-SSB/La antibodies, anti-centromere | Confirmed | 0.1-4% of the population |
| Vitiligo | Skin | Various, not specific | Confirmed | 1% |

## Digestive system

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

## Heart and vascular system

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

## Urinary system

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

## Nervous system

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

## Endocrine system

| Autoimmune disease | Primary organ/body part affected | Autoantibodies | Acceptance as an autoimmune disease | Prevalence rate (US) |
| Addison's disease | Adrenal glands | 21-hydroxylase antibodies | Confirmed | 0.93-1.4 per 10,000 |
| Autoimmune oophoritis | Ovaries | Anti-ovarian antibodies | Probable | Rare |
| Autoimmune orchitis | Testes | Anti-sperm antibodies | Probable | Rare |
| Autoimmune pancreatitis | Pancreas | IgG4, Anti-CA2 antibodies | Confirmed | 0.82-1.3 per 100,000 |
| Autoimmune polyendocrine syndrome type 1 (APS1) | Multiple endocrine organs | Various autoantibodies depending on the organs affected | Confirmed | 1 in 100,000 to 200,000 |
| Autoimmune polyendocrine syndrome type 2 (APS2) | Multiple endocrine organs | Various autoantibodies depending on the organs affected | Confirmed | 1 in 20,000 |
| Autoimmune polyendocrine syndrome type 3 (APS3) | Multiple endocrine organs | Various autoantibodies depending on the organs affected | Confirmed | Rare |
| Diabetes mellitus type 1 | Pancreas | Anti-insulin, anti-IA-2, anti-GAD, anti-ZnT8 antibodies | Confirmed | 1.6 per 1,000 |
| Endometriosis | Endometrium | Anti-endometrial antibodies | Probable | 6-10% of women of reproductive age |
| Graves' disease | Thyroid gland | TSI, TPO, TG antibodies | Confirmed | 1.2% of the population |
| Hashimoto's thyroiditis | Thyroid gland | TPO, TG antibodies | Confirmed | 5% of the population |
| Ord's thyroiditis | Thyroid gland | TPO, TG antibodies | Confirmed | Rare |

## Respiratory system

| Autoimmune disease | Primary organ/body part affected | Autoantibodies | Acceptance as an autoimmune disease | Prevalence rate (US) | Cit. |
| Goodpasture syndrome | See urinary system | See urinary system | Confirmed | See urinary system | — |
| Eosinophilic granulomatosis with polyangiitis (EGPA) | See vascular system | See vascular system | Confirmed | See vascular system | — |
| Granulomatosis with polyangiitis (GPA) | See vascular system | See vascular system | Confirmed | See vascular system | — |
| Idiopathic pulmonary fibrosis | Lungs | None specific | Possible | 20 per 100,000 (men), 13 per 100,000 (women) | |
| 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) | |
| Pulmonary alveolar proteinosis | Lungs | Anti-GM-CSF antibodies | Confirmed | 6.2 per million | |
| Rheumatoid lung disease | Lungs | Rheumatoid factor, Anti-CCP antibodies | Confirmed | Part of RA prevalence (about 1%) | |
| Sarcoidosis | Lungs and other organs | None specific | Confirmed | 10 - 40 per 100,000 | |

## Blood

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

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

## Eyes

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

## Musculoskeletal system

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 | See integumentary system | Confirmed | See integumentary system | — |
| Fibromyalgia | Musculoskeletal system, pain perception | None specific | Possible | | |
| Inclusion body myositis | Proximal and distal muscles | None specific | Probable | | |
| Myositis | Skeletal muscles | Various, depends on subtype | Confirmed | | |
| Myasthenia gravis | Voluntary muscles, neuromuscular junctions | Anti-acetylcholine receptor, Anti-MuSK | Confirmed | | |
| Neuromyotonia | Peripheral nerves affecting muscle control | Anti-voltage-gated potassium channels | Probable | | |
| Paraneoplastic cerebellar degeneration | Central nervous system, cerebellum | Anti-Yo, Anti-Hu, Anti-Ri, others | Confirmed | | |
| 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 | | |

## Autoimmune comorbidities

This list includes conditions that are not diseases, but symptoms or syndromes common to autoimmune disease.
* Chronic fatigue syndrome
* Complex regional pain syndrome
* Eosinophilic esophagitis
* Gastritis
* POEMS syndrome
* Raynaud's phenomenon
* Primary immunodeficiency
* Pyoderma gangrenosum

## 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 |
| 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. |
| Chronic obstructive pulmonary disease | No consistent evidence of association with autoimmunity. |
| 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. |
| 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. |
| Rasmussen's encephalitis | No consistent evidence of association with autoimmunity. |
| Schizophrenia | No consistent evidence of association with autoimmunity. |
| 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 | |