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स्वप्रतिरक्षित रोग

स्वप्रतिरक्षित रोग
वर्गीकरण एवं बाह्य साधन
आईसीडी-१०D84.9, M35.9
आईसीडी-279.4
ओएमआईएम109100
डिज़ीज़-डीबी28805
मेडलाइन प्लस000816
एम.ईएसएचD001327

वे रोग स्वप्रतिरक्षित रोग (Autoimmune diseases) कहलाते हैं जिनके होने पर किसी जीव की प्रतिरक्षा प्रणाली अपने ही ऊतकों या शरीर में उपस्थित अन्य पदार्थों को रोगजनक (pathogen) समझने की गलती कर बैठती है और उन्हें समाप्त करने के लिये उन पर हमला कर देती है। इस प्रकार का रोग शरीर के किसी एक अंग में सीमित हो सकता है (जैसे स्वप्रतिरक्षित थॉयराय शोथ (autoimmune thyroiditis) या शरीर के विभिन्न स्थानों पर एक विशेष प्रकार के ऊतक को प्रभावित कर सकता है।

स्वप्रतिरक्षित रोगों की चिकित्सा में प्रायः दवाओं का उपयोग करके प्रतिरक्षा प्रणाली की सक्रियता को कम किया जाता है जिसे प्रतिरक्षादमन (immunosuppression) कहते हैं।

मानदण्ड (क्राइटेरिया)

वर्गीकरण

नाम Accepted/
suspected
Hypersensitivity

I, II, III, IV

आटोएन्टीबॉडी टिप्पणी
Acute disseminated encephalomyelitis (ADEM)Accepted[1]
Addison's diseaseinterferon omega; transglutaminase; aromatic acid carboxylase; GAD; HAI; 17 hydroxylase; 21 hydroxylase
AgammaglobulinemiaIGHM; IGLL1: CD79A; CD79B; BLNK; LRRC8A
Alopecia areataAccepted[2][3]T-cells
Amyotrophic Lateral Sclerosis
Ankylosing SpondylitisAccepted[4][5][6]ANCA?CD8; HLA-B27
Antiphospholipid syndromeAccepted[1]anti-cardiolipin;anti pyruvate dehydrogenase; β2 glycoprotein I; phosphatidylserine; anti apoH; Annexin A5HLA-DR7, HLA-B8, HLA-DR2, HLA-DR3
Antisynthetase syndrome
Atopic allergyI
Atopic dermatitisI
Autoimmune aplastic anemia
Autoimmune cardiomyopathyAccepted
Autoimmune enteropathy
Autoimmune hemolytic anemiaAcceptedIIcomplement activation
Autoimmune hepatitisAcceptedcell-mediatedanti-mitochondrial antibodies; ANA; anti-smooth muscle antibodies, LKM-1; soluble liver antigen
Autoimmune inner ear diseaseAccepted[7]
Autoimmune lymphoproliferative syndromeAcceptedTNFRSF6; defective Fas-CD95 apoptosis
Autoimmune peripheral neuropathyAccepted
Autoimmune pancreatitisAcceptedANA; anti-lactoferrin antibodiesanti-carbonic anhydrase antibodies; rheumatoid factor
Autoimmune polyendocrine syndromeAcceptedUnknown or multipleAPS-1 see Addison's disease
Autoimmune progesterone dermatitisAccepted
Autoimmune thrombocytopenic purpuraAcceptedanti gpIIb-IIIa or 1b-IX
Autoimmune urticariaAccepted[8]
Autoimmune uveitisAcceptedHLAB-27?
Balo disease/Balo concentric sclerosis
Behçet's diseaseimmune-mediated systemic vasculitis; linkage to HLA-B51 (HLA-B27); very different manifestations with ulcers as common symptom; also called Morbus Adamandiades-Behçet
Berger's diseaseIgA (elevated in 50% of patients), IgA (in mesangial deposits on kidney biopsy)
Bickerstaff's encephalitisAnti-GQ1b 2/3 patientssimilar to Guillain-Barré syndrome
Blau syndromeoverlaps both sarcoidosis and granuloma annulare
Bullous pemphigoidIgG autoantibodies targeting the type XVII collagen component of hemidesmosomes[9]
Cancer
Castleman's diseaseOver expression of IL-6
सीलिएक रोगAccepted[10][11][12]IV??Anti-tissue transglutaminase antibodiesHLA-DQ8 and DQ2.5
Chagas diseaseSuspected[13]
Chronic inflammatory demyelinating polyneuropathyAnti-ganglioside antibodies:anti-GM1, anti-GD1a, anti-GQ1bsimilar to Guillain-Barré syndrome
Chronic recurrent multifocal osteomyelitisLPIN2, D18S60,similar to Majeed syndrome
Chronic obstructive pulmonary diseaseSuspected[14][15]
Churg-Strauss syndromep-ANCA
Cicatricial pemphigoidanti-BP-1, anti BP-2precipitates C3
Cogan syndrome
Cold agglutinin diseaseAcceptedIIIgMidiopathic or secondary to leukemia or infection
Complement component 2 deficiency
Contact dermatitisIII
Cranial arteritisaka Temporal arteritis; involves giant cells
CREST syndromeAnti-centromere antibodies Anti-nuclear antibodies
Crohn's disease (one of two types of idiopathic inflammatory bowel disease "IBD")Accepted[1]IVInnate immunity; Th17; Th1; ATG16L1; CARD15;XBP1;
Cushing's Syndromecortisol binding globulin?
Cutaneous leukocytoclastic angiitisneutrophils
Dego's diseaseVasculopathy
Dercum's diseaseSuspectedLipoid tissue.[16]
Dermatitis herpetiformisIgA; anti-epidermal transglutaminase antibodies
DermatomyositisAccepted[17]histidine-tRNA anti-signal recognition peptide Anti-Mi-2 Anti-Jo1.[18]B- and T-cell perivascular inflammatory infiltrate on muscle biopsy
टाइप 1 डायबिटीज मेलेटसAccepted[1]IVGlutamic acid decarboxylase antibodies (GADA), islet cell antibodies (ICA), and insulinoma-associated autoantibodies (IA-2), anti-insulin antibodies
Diffuse cutaneous systemic sclerosisanti-nuclear antibodies, anti-centromere and anti-scl70/anti-topoisomerase antibodies[19]COL1A2 and TGF-β1
Dressler's syndromemyocardial neo-antigens formed as a result of the MI
Drug-induced lupusanti-histone
Discoid lupus erythematosusIIIIL-2 and IFN-gamma>[20]
EczemaLEKTI, SPINK5,[21] filaggrin.,[22] Brain-derived neurotrophic factor (BDNF) and Substance P.[23]
EndometriosisSuspected[24]
Enthesitis-related arthritis[25].MMP3[26] TRLR2, TLR4,[27] ERAP1[28]
Eosinophilic fasciitisAccepted
Eosinophilic gastroenteritisIgEIL-3, IL-5, GM-CSF, eotaxin
Epidermolysis bullosa acquisitaCOL7A1
Erythema nodosum
Erythroblastosis fetalisIIABO, Rh, Kell antibodiesmother's immune system attacks fetus
Essential mixed cryoglobulinemia
Evan's syndrome
Fibrodysplasia ossificans progressivaACVR1 Lymphocytes express increased BMP4
Fibrosing alveolitis (or Idiopathic pulmonary fibrosis)SFTPA1, SFTPA2, TERT, and TERC.[29]
Gastritisserum antiparietal and anti-IF antibodies
Gastrointestinal pemphigoidAccepted
Giant cell arteritismacrophage giant cells
GlomerulonephritisSometimesIgAsee Buerger's disease for IgA; Membranous glomerulonephritis for IgG; Membranoproliferative/mesangiocapillary GN (Complement activation); Goodpasture's syndrome; Wegener's granulomatosis
Goodpasture's syndromeAccepted[1]IIAnti-Basement Membrane Collagen Type IV Protein
Graves' diseaseAccepted[1]IIthyroid autoantibodies (TSHR-Ab) that activate the TSH-receptor (TSHR)
Guillain-Barré syndrome (GBS)Accepted[1]IVAnti-ganglioside
Hashimoto's encephalopathyAccepted[1]IValpha-enolase[30]
Hashimoto's thyroiditisAccepted[1]IVantibodies against thyroid peroxidase and/or thyroglobulinHLADR5, CTLA-4
Henoch-Schonlein purpuraimmunoglobulin A (IgA) and complement component 3 (C3)
Herpes gestationis aka Gestational PemphigoidIgG and C3 misdirected antibodies intended to protect the placenta
Hidradenitis suppurativaSuspected[31]
Hughes-Stovin syndrome
HypogammaglobulinemiaIGHM, IGLL1, CD79A, BLNK, LRRC8A, CD79B
Idiopathic inflammatory demyelinating diseasesa variant of multiple sclerosis
Idiopathic pulmonary fibrosisSFTPA1, SFTPA2, TERT, and TERC.[29]
Idiopathic thrombocytopenic purpura (See Autoimmune thrombocytopenic purpura)Accepted[1]IIglycoproteins IIb-IIIa or Ib-IX, immunoglobulin G
IgA nephropathyIII?IgA produced from marrow rather than MALT
Inclusion body myositissimilar to polymyositis but does not respond to steroid therapy-activated T8 cells
Chronic inflammatory demyelinating polyneuropathyanti-ganglioside antibodiessimilar to Guillain–Barré syndrome
Interstitial cystitisSuspected[32]Mast cells
Juvenile idiopathic arthritis aka Juvenile rheumatoid arthritisinconsistent ANA Rheumatoid factor
Kawasaki's diseaseSuspectedITPKC HLA-B51
Lambert-Eaton myasthenic syndromevoltage-gated calcium channels; Q-type calcium channel, synaptogagmin, muscarinic acetylcholine receptor M1HLA-DR3-B8
Leukocytoclastic vasculitis
Lichen planus
Lichen sclerosus
Linear IgA disease (LAD)
Lou Gehrig's disease (Also Amyotrophic lateral sclerosis)VCP, ATXN2, OPTN, FIG4, TARDBP, ANG, VAPB, FUS, SETX, ALS2, SOD1
Lupoid hepatitis aka Autoimmune hepatitisANA and SMA,[33] LKM-1, LKM-2 or LKM-3; antibodies against soluble liver antigen[34][35](anti-SLA, anti-LP) no autoantibodies detected (~20%)[] ||
Lupus erythematosusAccepted[1]IIIAnti-nuclear antibodies[36] anti-Ro.[37] Also, they are often present in Sjögren's syndrome.[38][39]
Majeed syndromeLPIN2
Ménière's diseaseIII?major peripheral myelin protein P0[40]
Microscopic polyangiitisp-ANCA myeloperoxidasebinds to neutrophils causing them to degranulate and damages endothelium
Miller-Fisher syndrome see Guillain-Barre SyndromeAcceptedanti-GQ1b
Mixed connective tissue diseaseAccepted[1]anti-nuclear antibody anti-U1-RNPHLA-DR4
MorpheaSuspected[41]
Mucha-Habermann disease aka Pityriasis lichenoides et varioliformis acutaT-cells
Multiple sclerosisSuspectedIVPECAM-1[42] Anti-Myelin Basic Protein
Myasthenia gravisAccepted[1]IInicotinic acetylcholine receptor MuSK proteinHA-B8 HLA-DR3 HLA-DR1
Myositissee Dermatomyositis and Polymyositis see Inclusion-body-myositis
Narcolepsy[43][44]Suspected[45]II?hypocretin or orexin[46]HLA-DQB1*0602[47]
Neuromyelitis optica (also Devic's disease)II?NMO-IgG aquaporin 4.[48][49]
NeuromyotoniaSuspected[50]II?voltage-gated potassium channels.[50]
Occular cicatricial pemphigoidII?BP-1, BP-2C3 deposition
Opsoclonus myoclonus syndromeSuspectedIV?Lymphocyte recruitment to CSF[51]
Ord's thyroiditis
Palindromic rheumatismanti-cyclic citrullinated peptide antibodies (anti-CCP) and antikeratin antibodies (AKA)[52]
PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcus)SuspectedII?antibodies against streptococcal infection serve as auto-antibodies
Paraneoplastic cerebellar degenerationIV?[53] II?anti-Yo[54] (anti-cdr-2[55] in purkinje fibers) anti-Hu, anti-Tr, antiglutamate receptor
Paroxysmal nocturnal hemoglobinuria (PNH)Sometimes(?)complement attacks RBCs
Parry Romberg syndromeANA
Parsonage-Turner syndrome
Pars planitis
Pemphigus vulgarisAccepted[1]IIAnti-Desmoglein 3
Pernicious anaemiaAccepted[56]IIanti-parietal cell antibody
Perivenous encephalomyelitis
POEMS syndromeinterleukin 1β, interleukin 6 and TNFα. vascular endothelial growth factor (VEGF), given the .[57]
Polyarteritis nodosa
Polymyalgia rheumatica
PolymyositisAccepted[17]IFN-gamma, IL-1, TNF-alpha
Primary biliary cirrhosisAccepted[58]Anti-p62, Anti-sp100, Anti-Mitochondrial(M2)Anti-Ro aka SSA.[37] Also, they are often present in Sjögren's syndrome.[38][39]
Primary sclerosing cholangitisoverlap with primary biliary cirrhosis?
Progressive inflammatory neuropathySuspected
PsoriasisAccepted[59]IV? CD-8 T-cells, HLA-Cw6, IL-12b, IL-23b, TNFalpha, NF-κB
Psoriatic arthritisAccepted[60]IV?HLA=B27
Pyoderma gangrenosumCan occur in conjunction with other immune-related disorders
Pure red cell aplasia
Rasmussen's encephalitisanti-NR2A antibodies
Raynaud phenomenonSuspectedCan occur in conjunction with other immune-related disorders
Relapsing polychondritisAccepted[61]
Reiter's syndrome
Restless leg syndromeSuspectedMay occur in Sjögren's syndrome, celiac disease, and rheumatoid arthritis or in derangements of iron metabolism
Retroperitoneal fibrosis
Rheumatoid arthritisAccepted[1]IIIRheumatoid factor (anti-IgGFc), Anti-MCV, ACPAs(VimentinHLA-DR4, PTPN22, depleted B cells, TNF alpha, IL-17, (also maybe IL-1, 6, and 15)
Rheumatic feverIIstreptococcal M protein cross reacts with human myosin,[62] anti-DNase B, ASO
SarcoidosisSuspectedIV[63][64]BTNL2; HLA-B7-DR15; HLA DR3-DQ2.[65]
SchizophreniaSuspected[66][67][68]
Schmidt syndrome another form of APSanti-21 hydroxylase, anti-17 hydroxylase[69]DQ2, DQ8 and DRB1*0404
Schnitzler syndromeIgM?
Scleritis
SclerodermaSuspected[41]IV?Scl-70 Anti-topoisomerasedysregulated apoptosis?
Serum SicknessIII
Sjögren's syndromeAccepted[1]Anti-ro.[37] Also, they are often present in Sjögren's syndrome.[38][39]
SpondyloarthropathyHLA-B27
Still's disease see Juvenile Rheumatoid ArthritisANAmacrophage migration inhibitory factor[70]
Stiff person syndromeSuspectedglutamic acid decarboxylase (GAD),[71]GLRA1 (glycine receptor
Subacute bacterial endocarditis (SBE)III[72]essential mixed cryoglobulinemia
Susac's syndrome
Sweet's syndromeGCSF
Sydenham chorea see PANDAS
Sympathetic ophthalmiaocular antigens following trauma
Systemic lupus erythematosis see Lupus erythematosisIII
Takayasu's arteritis
Temporal arteritis (also known as "giant cell arteritis")Accepted[1]IV
ThrombocytopeniaIIglycoproteins IIb-IIIa or Ib-IX in ITP anti-ADAMTS13 in TTP.[73] and HUS anti-cardiolipin (anti-cardiolipin antibodies) and β2 glycoprotein I in Antiphospholipid syndrome anti-HPA-1a, anti-HPA-5b, and others[74] in NAITmultiple mechanisms
Tolosa-Hunt syndrome
Transverse myelitisAcceptedTransverse Myelitis is a rare neurological disorder that is part of a spectrum of neuroimmunologic diseases of the central nervous system. https://web.archive.org/web/20120623100725/http://www.myelitis.org/
Ulcerative colitis (one of two types of idiopathic inflammatory bowel disease "IBD")Accepted[1]IV
Undifferentiated connective tissue disease different from Mixed connective tissue diseaseAcceptedanti-nuclear antibodyHLA-DR4
Undifferentiated spondyloarthropathy
Urticarial vasculitisII?anti C1q antibodies[75]clinically may resemble type I hypersensitivity!
VasculitisAccepted[9]IIIsometimes ANCA
VitiligoSuspected[76][77]NALP-1 RERE, PTPN22, LPP, IL2RA, GZMB, UBASH3A and C1QTNF6
Wegener's granulomatosisAccepted[78]Anti-neutrophil cytoplasmic(cANCA)

चिकित्सा-विकास

इन्हें भी देखें

अन्य पठनीय सामग्री

सन्दर्भ

  1. MeSH Autoimmune+Diseases
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