list of autoimmune diseases 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 this is a dynamic list and may never be able to satisfy particular standards for completeness. you can help by adding missing items with reliable sources. 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 myocarditismoderate, f, r, a391.2422429.0synonyms: autoimmune myocarditis, autoimmune cardiomyopathy, coxsackie myocarditis postmyocardial infarction syndromelimited, 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)limited, yiii421.0autoantibodies: essential mixed cryoglobulinemia. kidney anti-glomerular basement membrane nephritismoderate, 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 cystitislimited, s595.1mast cells. liver autoimmune hepatitismoderate, 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)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 areatamoderate, a704.01autoantibodies: t-cells. synonyms: alopecia areata - patchy, totalis, universalis autoimmune angioedemalimited, f, n277.6 995.1 autoimmune progesterone dermatitislimited, x, a279.49 autoimmune urticariacomorbidity, a708 bullous pemphigoidmoderate, 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 herpetiformismoderate, c, y694.0autoantibodies: iga eosinophilia; anti-epidermal transglutaminase antibodies. discoid lupus erythematosuslimited, 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 suppurativalimited, c, s705.83 lichen planuslimited, y697.0 lichen sclerosuslimited, c, y701.0 linear iga disease (lad)moderate, y646.8 morphealimited, c, s701.0 pemphigus vulgarismoderate, 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 psoriasismoderate, aiv?696cd-8 t-cells, hla-cw6, il-12b, il-23b, tnfalpha, nf-κb. systemic sclerodermalimited, 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 vitiligolimited, 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 diseasemoderate, 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 2moderate, 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 1moderate, 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' diseasemoderate, m, aii242.0autoantibodies: thyroid autoantibodies (tshr-ab) that activate the tsh-receptor (tshr). exocrine reproductive organs autoimmune oophoritismoderate, n614.2 endometriosislimited, s617.0 autoimmune orchitislimited, n604.0 salivary glands sjögren syndromemoderate, 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 diseasemoderate, a,eiv??579.0hla-dq8 and dq2.5. autoantibodies: anti-tissue transglutaminase antibodies, anti-endomysial iga, anti-gliadin iga. crohn's diseasemoderate, yiv555innate immunity; th17; th1; atg16l1; card15; xbp1. esophageal achalasia-limited, x microscopic colitislimited, s558.9 ulcerative colitislimited, 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)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 purpuramoderate, 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 anemiamoderate, aii281.0autoantibodies: anti-parietal cell antibody. pure red cell aplasialimited, y284.81 thrombocytopenialimited, 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 dolorosalimited, l, s272.8lipoid tissue. synonym: dercum's disease adult-onset still's diseasemoderate, y714.2macrophage migration inhibitory factor. autoantibodies: ana. ankylosing spondylitislimited, s720.0cd8; hla-b27. crest syndromelimited, y710.1autoantibodies: anti-centromere antibodies anti-nuclear antibodies. drug-induced lupusmoderate, y710.0autoantibodies: anti-histone antibodies. enthesitis-related arthritislimited, c, ymmp3, trlr2, tlr4, erap1. a subtype of juvenile rheumatoid arthritis. eosinophilic fasciitislimited, f, a728.89synonym: shulman's syndrome felty syndromestrong, 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 arthritisstrong, m, r, y714.30autoantibodies: inconsistent ana, rheumatoid factor. synonyms: juvenile rheumatoid arthritis, juvenile idiopathic arthritis lyme disease (chronic)limited, l, t, n088.81 mixed connective tissue disease (mctd)moderate, m, a710.8hla-dr4. autoantibodies: anti-nuclear antibody, anti-u1-rnp. palindromic rheumatismlimited, 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 arthritismoderate, c, aiv?696.0hla-b27. reactive arthritislimited, c, f, y099.3 relapsing polychondritisstrong, a733.99synonyms: atrophic polychondritis, systemic chondromalacia, chronic atrophic polychondritis, meyenburg-altherr-uehlinger syndrome, generalized chondromalacia, systemic chondromalacia retroperitoneal fibrosislimited, y593.4 rheumatic fevermoderate, t, aii390autoantibodies: streptococcal m protein cross reacts with human myosin. rheumatoid arthritisstrong, 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). sarcoidosislimited, siv135btnl2; hla-b7-dr15; hla dr3-dq2. schnitzler syndromelimited, l, x, y273.1igm. systemic lupus erythematosus (sle)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 dermatomyositismoderate, 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 gravisstrong, m, aii358ha-b8 hla-dr3 hla-dr1. autoantibodies: nicotinic acetylcholine receptor musk protein. neuromyotonialimited, f, sii?333.90autoantibodies: voltage-gated potassium channels. synonym: isaacs' syndrome paraneoplastic cerebellar degenerationlimited, yiv? ii?334.9autoantibodies: anti-yo (anti-cdr-2 in purkinje fibers) anti-hu, anti-tr, antiglutamate receptor. polymyositislimited, 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 neuropathylimited, n356.8 anti-n-methyl-d-aspartate (anti-nmda) receptor encephalitismoderate, 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 polyneuropathymoderate, 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 syndromestrong, m, aiv357.0autoantibodies: anti-ganglioside, anti-gq1b. synonyms: miller–fisher syndrome, landry's paralysis. hashimoto's encephalopathymoderate, 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 iistrong, m, aiv340autoantibody against potassium channel has been reported to present demyelination pattern ii. other cases present autoimmunity against mog and anoctamin-2. 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 oshtoran syndromexf06.9heritable, abnormalities in the kynurenine and glutamate metabolism. 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 syndromelimited, s333.91glra1 (glycine receptor). autoantibodies: glutamic acid decarboxylase (gad). sydenham's chorealimited, t, y392 transverse myelitislimited, m, a323.82341.2 eyes autoimmune retinopathylimited, 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 opticalimited, m, yii?341.0autoantibodies: nmo-igg aquaporin 4. synonym: devic's disease. opsoclonus myoclonus syndromelimited, 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)limited, a388.8 ménière's diseaselimited, 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)limited, i, x, y446.4autoantibodies: p-anca eosinophilia. giant cell arteritislimited, i, r, aiv446.5synonyms: cranial arteritis, temporal arteritis. granulomatosis with polyangiitis (gpa)strong, m, a446.4autoantibodies: anti-neutrophil cytoplasmic (canca). iga vasculitis (igav)limited, l, y287.0autoantibodies: iga and complement component 3 (c3). synonyms: anaphylactoid purpura, henoch-schonlein purpura, purpura rheumatica, schönlein–henoch purpura. kawasaki diseasemoderate, s,e446.1itpkc 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 vasculitislimited, x, yii?708.9 clinically may resemble type i hypersensitivity. autoantibodies: anti c1q antibodies. vasculitisstrong, 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. 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