स्वप्रतिरक्षित रोग

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    
   Addison's disease   interferon omega; transglutaminase; aromatic acid carboxylase; GAD; HAI; 17 hydroxylase; 21 hydroxylase    Agammaglobulinemia    IGHM; IGLL1: CD79A; CD79B; BLNK; LRRC8A
   Alopecia areata  AcceptedAmerican Academy of Dermatology, http://www.aad.org/public/publications/pamphlets/common_alopecia.html htmlThe University of Texas MD Anderson Cancer Center, http://www.mdanderson.org/patient-and-cancer-information/cancer-information/cancer-types/alopecia-areata/index.html    T-cells    Amyotrophic Lateral Sclerosis       Ankylosing Spondylitis  AcceptedHLA-B27 and Ankylosing Spondylitis, http://www.hlab27.com Inflammatory Diseases of Immune Dysregulation, http://www.idid.us   ANCA? CD8; HLA-B27
   Antiphospholipid syndrome Accepted  anti-cardiolipin;anti pyruvate dehydrogenase; β2 glycoprotein I; phosphatidylserine; anti apoH; Annexin A5 HLA-DR7, HLA-B8, HLA-DR2, HLA-DR3 
   Antisynthetase syndrome       Atopic allergy  I     Atopic dermatitis  I     Autoimmune aplastic anemia       Autoimmune cardiomyopathy  Accepted      Autoimmune enteropathy       Autoimmune hemolytic anemia  Accepted  II  complement activation
   Autoimmune hepatitis  Accepted  cell-mediatedanti-mitochondrial antibodies; ANA; anti-smooth muscle antibodies, LKM-1; soluble liver antigen    Autoimmune inner ear disease  Accepted    
   Autoimmune lymphoproliferative syndrome  Accepted   TNFRSF6; defective Fas-CD95 apoptosis
   Autoimmune peripheral neuropathy  Accepted      Autoimmune pancreatitis  Accepted   ANA; anti-lactoferrin antibodiesanti-carbonic anhydrase antibodies; rheumatoid factor   Autoimmune polyendocrine syndrome  Accepted  Unknown or multiple  APS-1 see Addison's disease
   Autoimmune progesterone dermatitis  Accepted      Autoimmune thrombocytopenic purpura  Accepted  anti gpIIb-IIIa or 1b-IX    Autoimmune urticaria  Accepted      Autoimmune uveitis  Accepted  HLAB-27?    Balo disease/Balo concentric sclerosis     
   Behçet's disease     immune-mediated systemic vasculitis; linkage to HLA-B51 (HLA-B27); very different manifestations with ulcers as common symptom; also called Morbus Adamandiades-Behçet
   Berger's disease    IgA (elevated in 50% of patients), IgA (in mesangial deposits on kidney biopsy)   Bickerstaff's encephalitis    Anti-GQ1b 2/3 patients similar to Guillain-Barré syndrome
   Blau syndrome     overlaps both sarcoidosis and granuloma annulare
   Bullous pemphigoid   IgG autoantibodies targeting the type XVII collagen component of hemidesmosomes  
   Cancer       Castleman's disease     Over expression of IL-6
   सीलिएक रोग  Accepted  IV??  Anti-tissue transglutaminase antibodies HLA-DQ8 and DQ2.5
   Chagas disease  Suspected      Chronic inflammatory demyelinating polyneuropathy    Anti-ganglioside antibodies:anti-GM1, anti-GD1a, anti-GQ1bsimilar to Guillain-Barré syndrome
   Chronic recurrent multifocal osteomyelitis    LPIN2, D18S60,similar to Majeed syndrome
   Chronic obstructive pulmonary disease  Suspected      Churg-Strauss syndrome   p-ANCA   Cicatricial pemphigoid    anti-BP-1, anti BP-2precipitates C3
   Cogan syndrome       Cold agglutinin disease  Accepted  II IgMidiopathic or secondary to leukemia or infection
   Complement component 2 deficiency       Contact dermatitis  III     Cranial arteritis    aka Temporal arteritis; involves giant cells
   CREST syndrome    Anti-centromere antibodies Anti-nuclear antibodies   Crohn's disease (one of two types of idiopathic inflammatory bowel disease "IBD")  Accepted   IV  Innate immunity; Th17; Th1; ATG16L1; CARD15;XBP1;
   Cushing's Syndrome   cortisol binding globulin?   Cutaneous leukocytoclastic angiitis    neutrophils
   Dego's disease     Vasculopathy
   Dercum's disease  Suspected   Lipoid tissue.
   Dermatitis herpetiformis    IgA; anti-epidermal transglutaminase antibodies    Dermatomyositis  Accepted   histidine-tRNA anti-signal recognition peptide Anti-Mi-2 Anti-Jo1.  B- and T-cell perivascular inflammatory infiltrate on muscle biopsy
   टाइप 1 डायबिटीज मेलेटस  Accepted   IV  Glutamic acid decarboxylase antibodies (GADA), islet cell antibodies (ICA), and insulinoma-associated autoantibodies (IA-2), anti-insulin antibodies    Diffuse cutaneous systemic sclerosis   anti-nuclear antibodies, anti-centromere and anti-scl70/anti-topoisomerase antibodies COL1A2 and TGF-β1
   Dressler's syndrome   myocardial neo-antigens formed as a result of the MI   Drug-induced lupus   anti-histone    Discoid lupus erythematosus  III  IL-2 and IFN-gamma>
   Eczema    LEKTI, SPINK5, filaggrin., Brain-derived neurotrophic factor (BDNF) and Substance P.
   Endometriosis  Suspected      Enthesitis-related arthritis   .MMP3 TRLR2, TLR4, ERAP1
   Eosinophilic fasciitis  Accepted     Eosinophilic gastroenteritis    IgE  IL-3, IL-5, GM-CSF, eotaxin
   Epidermolysis bullosa acquisita     COL7A1
   Erythema nodosum       Erythroblastosis fetalis  II  ABO, Rh, Kell antibodies mother's immune system attacks fetus
   Essential mixed cryoglobulinemia       Evan's syndrome       Fibrodysplasia ossificans progressiva     ACVR1 Lymphocytes express increased BMP4
   Fibrosing alveolitis (or Idiopathic pulmonary fibrosis)     SFTPA1, SFTPA2, TERT, and TERC.
   Gastritis   serum antiparietal and anti-IF antibodies    Gastrointestinal pemphigoid  Accepted    
   Giant cell arteritis     macrophage giant cells
   Glomerulonephritis  Sometimes   IgA  see Buerger's disease for IgA; Membranous glomerulonephritis for IgG; Membranoproliferative/mesangiocapillary GN (Complement activation); Goodpasture's syndrome; Wegener's granulomatosis 
   Goodpasture's syndrome  Accepted   II  Anti-Basement Membrane Collagen Type IV Protein    Graves' disease  Accepted   II thyroid autoantibodies (TSHR-Ab) that activate the TSH-receptor (TSHR)    Guillain-Barré syndrome (GBS)  Accepted   IV  Anti-ganglioside    Hashimoto's encephalopathy  Accepted  IV alpha-enolase    Hashimoto's thyroiditis  Accepted   IV  antibodies against thyroid peroxidase and/or thyroglobulin HLADR5, CTLA-4
   Henoch-Schonlein purpura   immunoglobulin A (IgA) and complement component 3 (C3)    Herpes gestationis aka Gestational Pemphigoid   IgG and C3 misdirected antibodies intended to protect the placenta    Hidradenitis suppurativa  Suspected      Hughes-Stovin syndrome    Hypogammaglobulinemia    IGHM, IGLL1, CD79A, BLNK, LRRC8A, CD79B
   Idiopathic inflammatory demyelinating diseases     a variant of multiple sclerosis
   Idiopathic pulmonary fibrosis    SFTPA1, SFTPA2, TERT, and TERC.
   Idiopathic thrombocytopenic purpura (See Autoimmune thrombocytopenic purpura)  Accepted   II   glycoproteins IIb-IIIa or Ib-IX, immunoglobulin G 
   IgA nephropathy  III? IgA produced from marrow rather than MALT    Inclusion body myositis     similar to polymyositis but does not respond to steroid therapy-activated T8 cells
   Chronic inflammatory demyelinating polyneuropathy    anti-ganglioside antibodies similar to Guillain–Barré syndrome
   Interstitial cystitis  Suspected   Mast cells
   Juvenile idiopathic arthritis aka Juvenile rheumatoid arthritis    inconsistent ANA Rheumatoid factor    Kawasaki's disease  Suspected    ITPKC HLA-B51
   Lambert-Eaton myasthenic syndrome    voltage-gated calcium channels; Q-type calcium channel, synaptogagmin, muscarinic acetylcholine receptor M1  HLA-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 hepatitis   ANA and SMA, LKM-1, LKM-2 or LKM-3; antibodies against soluble liver antigen(anti-SLA, anti-LP) no autoantibodies detected (~20%)    Lupus erythematosus  Accepted   III  Anti-nuclear antibodies anti-Ro. Also, they are often present in Sjögren's syndrome.   Majeed syndrome    LPIN2
   Ménière's disease  III?  major peripheral myelin protein P0   Microscopic polyangiitis   p-ANCA myeloperoxidase binds to neutrophils causing them to degranulate and damages endothelium
   Miller-Fisher syndrome see Guillain-Barre Syndrome Accepted  anti-GQ1b
   Mixed connective tissue disease  Accepted   anti-nuclear antibody anti-U1-RNP  HLA-DR4
   Morphea  Suspected     Mucha-Habermann disease aka Pityriasis lichenoides et varioliformis acuta   T-cells
   Multiple sclerosis  Suspected IV  PECAM-1 Anti-Myelin Basic Protein
   Myasthenia gravis  Accepted   II  nicotinic acetylcholine receptor MuSK proteinHA-B8 HLA-DR3 HLA-DR1
   Myositis      see Dermatomyositis and Polymyositis see Inclusion-body-myositis
    Narcolepsy  Suspected II?  hypocretin or orexin HLA-DQB1*0602 
   Neuromyelitis optica (also Devic's disease)  II? NMO-IgG aquaporin 4.   Neuromyotonia  Suspected  II?  voltage-gated potassium channels.    Occular cicatricial pemphigoid  II? BP-1, BP-2  C3 deposition
   Opsoclonus myoclonus syndrome  Suspected IV?   Lymphocyte recruitment to CSF
   Ord's thyroiditis      Palindromic rheumatism    anti-cyclic citrullinated peptide antibodies (anti-CCP) and antikeratin antibodies (AKA)
   PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcus) Suspected II?  antibodies against streptococcal infection serve as auto-antibodies 
   Paraneoplastic cerebellar degeneration  IV? II?  anti-Yo (anti-cdr-2 in purkinje fibers) anti-Hu, anti-Tr, antiglutamate receptor    Paroxysmal nocturnal hemoglobinuria (PNH)  Sometimes(?)   complement attacks RBCs
   Parry Romberg syndrome    ANA    Parsonage-Turner syndrome      Pars planitis       Pemphigus vulgaris  Accepted   II  Anti-Desmoglein 3
   Pernicious anaemia  Accepted   II  anti-parietal cell antibody    Perivenous encephalomyelitis       POEMS syndrome    interleukin 1β, interleukin 6 and TNFα. vascular endothelial growth factor (VEGF), given the .
   Polyarteritis nodosa       Polymyalgia rheumatica     
   Polymyositis  Accepted  IFN-gamma, IL-1, TNF-alpha    Primary biliary cirrhosis  Accepted    Anti-p62, Anti-sp100, Anti-Mitochondrial(M2)Anti-Ro aka SSA. Also, they are often present in Sjögren's syndrome.    Primary sclerosing cholangitis    overlap with primary biliary cirrhosis?
   Progressive inflammatory neuropathy  Suspected      Psoriasis  AcceptedNational Psoriasis Foundation, http://www.psoriasis.org/home/learn01.php    IV?   CD-8 T-cells, HLA-Cw6, IL-12b, IL-23b, TNFalpha, NF-κB
   Psoriatic arthritis  AcceptedNational Psoriasis Foundation, http://www.psoriasis.org/home/learn02.php    IV?   HLA=B27 
   Pyoderma gangrenosum     Can occur in conjunction with other immune-related disorders
   Pure red cell aplasia       Rasmussen's encephalitis    anti-NR2A antibodies    Raynaud phenomenon  Suspected   Can occur in conjunction with other immune-related disorders
   Relapsing polychondritis  Accepted      
   Reiter's syndrome       Restless leg syndrome  Suspected   May occur in Sjögren's syndrome, celiac disease, and rheumatoid arthritis or in derangements of iron metabolism
   Retroperitoneal fibrosis       Rheumatoid arthritis  Accepted   III  Rheumatoid factor (anti-IgGFc), Anti-MCV, ACPAs(Vimentin  HLA-DR4, PTPN22, depleted B cells, TNF alpha, IL-17, (also maybe IL-1, 6, and 15)
   Rheumatic fever  II  streptococcal M protein cross reacts with human myosin, anti-DNase B, ASO    Sarcoidosis  Suspected IV  BTNL2; HLA-B7-DR15; HLA DR3-DQ2.

   Schizophrenia   Suspected     Schmidt syndrome another form of APS   anti-21 hydroxylase, anti-17 hydroxylase DQ2, DQ8 and DRB1*0404
   Schnitzler syndrome    IgM?
   Scleritis       Scleroderma  Suspected IV?  Scl-70 Anti-topoisomerase dysregulated apoptosis?
   Serum Sickness  III     Sjögren's syndrome  Accepted   Anti-ro. Also, they are often present in Sjögren's syndrome.    Spondyloarthropathy     HLA-B27 
   Still's disease see Juvenile Rheumatoid Arthritis    ANA macrophage migration inhibitory factor
   Stiff person syndrome  Suspected glutamic acid decarboxylase (GAD), GLRA1 (glycine receptor
   Subacute bacterial endocarditis (SBE)  III essential mixed cryoglobulinemia    Susac's syndrome       Sweet's syndrome    GCSF
   Sydenham chorea see PANDAS       Sympathetic ophthalmia    ocular antigens following trauma    Systemic lupus erythematosis see Lupus erythematosis III     Takayasu's arteritis      Temporal arteritis (also known as "giant cell arteritis")  Accepted   IV    Thrombocytopenia  II 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  multiple mechanisms

   Tolosa-Hunt syndrome       Transverse myelitis Accepted    Transverse 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   IV    Undifferentiated connective tissue disease different from Mixed connective tissue disease  Accepted    anti-nuclear antibody  HLA-DR4
   Undifferentiated spondyloarthropathy      Urticarial vasculitis  II? anti C1q antibodies  clinically may resemble type I hypersensitivity!
   Vasculitis  Accepted  III  sometimes ANCA    Vitiligo  Suspected   NALP-1 RERE, PTPN22, LPP, IL2RA, GZMB, UBASH3A and C1QTNF6
   Wegener's granulomatosis  Accepted   Anti-neutrophil cytoplasmic(cANCA) 

 चिकित्सा-विकास 

 इन्हें भी देखें 
 स्वरोगक्षमता या स्वप्रतिरक्षा (Autoimmunity)
 प्रतिरक्षा विज्ञान (Immunology)
 प्रतिरक्षा प्रणाली (Immune System)

 अन्य पठनीय सामग्री 
 Vinay Kumar, Abul K. Abbas, Nelson Fausto, Jon Aster, Robbins and Cotran Pathologic Basis of Disease, Elsevier, 8th edition, 2010, 1464 pp., ISBN 978-1-4160-3121-5
 Handbook of Systemic Autoimmune Diseases, edited by Ronald Asherson, Elsevier, in 10 Volumes  :
 Ronald Asherson, Andrea Doria, Paolo Pauletto, The Heart in Systemic Autoimmune Diseases, Volume 1, 2004, ISBN 978-0-444-51398-4, ISBN 0-444-51398-1
 Ronald Asherson, Andrea Doria, Paolo Pauletto, Pulmonary Involvement in Systemic Autoimmune Diseases, Volume 2, 2005, ISBN 978-0-444-51652-7, ISBN 0-444-51652-2
 Ronald Asherson, Doruk Erkan, Steven Levine, The Neurologic Involvement in Systemic Autoimmune Diseases, Volume 3, 2005, ISBN 978-0-444-51651-0, ISBN 0-444-51651-4
 Michael Lockshin, Ware Branch (eds), Reproductive and Hormonal Aspects of Systemic Autoimmune Diseases, Volume 4, 2006, ISBN 978-0-444-51801-9, ISBN 0-444-51801-0
 Piercarlo Sarzi-Puttini, Ronald Asherson, Andrea Doria, Annegret Kuhn, Giampietro Girolomoni (eds), The Skin in Systemic Autoimmune Diseases, Volume Volume 5, 2006, ISBN 978-0-444-52158-3, ISBN 0-444-52158-5
 Rolando Cimaz, Ronald Asherson, Thomas Lehman (eds), Pediatrics in Systemic Autoimmune Diseases, Volume 6, 2008, ISBN 978-0-444-52971-8, ISBN 0-444-52971-3
 Justin Mason, Ronald Asherson, Charles Pusey (eds), The Kidney in Systemic Autoimmune Diseases, Volume 7, 2008, ISBN 978-0-444-52972-5, ISBN 0-444-52972-1
 Ronald Asherson, Manel Ramos-Casals, Joan Rodes, Josep Font, Digestive Involvement in Systemic Autoimmune Diseases, Volume 8, 2008, ISBN 978-0-444-53168-1, ISBN 0-444-53168-8
 Ronald Asherson, Sara Walker, Luis Jara, Endocrine Manifestations of Systemic Autoimmune Diseases, Volume 9, 2008, ISBN 978-0-444-53172-8, ISBN 0-444-53172-6
 R. Cervera, Ronald Asherson, Munther Khamashta, Joan Carles Reverter (eds), Antiphospholipid Syndrome in Systemic Autoimmune Diseases, Volume 10, 2009, ISBN 978-0-444-53169-8, ISBN 0-444-53169-6

 सन्दर्भ 

 इन्हें भी देखें 
 प्रतिरक्षा प्रणाली (immune system)
 स्वरोगक्षमता (autoimmunity)

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