स्वप्रतिरक्षित रोग

	स्वप्रतिरक्षित रोग; वर्गीकरण एवं बाह्य साधन
आईसीडी-१०	D84.9, M35.9
आईसीडी-९	279.4
ओएमआईएम	109100
डिज़ीज़-डीबी	28805
मेडलाइन प्लस	000816
एम.ईएसएच	D001327

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 disease			interferon omega; transglutaminase; aromatic acid carboxylase; GAD; HAI; 17 hydroxylase; 21 hydroxylase
Agammaglobulinemia				IGHM; IGLL1: CD79A; CD79B; BLNK; LRRC8A
Alopecia areata	Accepted^[2]^[3]		T-cells
Amyotrophic Lateral Sclerosis
Ankylosing Spondylitis	Accepted^[4]^[5]^[6]		ANCA?	CD8; HLA-B27
Antiphospholipid syndrome	Accepted^[1]		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-mediated	anti-mitochondrial antibodies; ANA; anti-smooth muscle antibodies, LKM-1; soluble liver antigen
Autoimmune inner ear disease	Accepted		^[7]
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	^[8]
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	^[9]
Cancer
Castleman's disease				Over expression of IL-6
सीलिएक रोग	Accepted^[10]^[11]^[12]	IV??	Anti-tissue transglutaminase antibodies	HLA-DQ8 and DQ2.5
Chagas disease	Suspected^[13]
Chronic inflammatory demyelinating polyneuropathy			Anti-ganglioside antibodies:anti-GM1, anti-GD1a, anti-GQ1b	similar to Guillain-Barré syndrome
Chronic recurrent multifocal osteomyelitis				LPIN2, D18S60,similar to Majeed syndrome
Chronic obstructive pulmonary disease	Suspected^[14]^[15]
Churg-Strauss syndrome			p-ANCA
Cicatricial pemphigoid			anti-BP-1, anti BP-2	precipitates C3
Cogan syndrome
Cold agglutinin disease	Accepted	II	IgM	idiopathic 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^[1]	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.^[16]
Dermatitis herpetiformis			IgA; anti-epidermal transglutaminase antibodies
Dermatomyositis	Accepted^[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 मेलेटस	Accepted^[1]	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^[19]	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>^[20]
Eczema				LEKTI, SPINK5,^[21] filaggrin.,^[22] Brain-derived neurotrophic factor (BDNF) and Substance P.^[23]
Endometriosis	Suspected^[24]
Enthesitis-related arthritis^[25]			.	MMP3^[26] TRLR2, TLR4,^[27] ERAP1^[28]
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.^[29]
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^[1]	II	Anti-Basement Membrane Collagen Type IV Protein
Graves' disease	Accepted^[1]	II	thyroid autoantibodies (TSHR-Ab) that activate the TSH-receptor (TSHR)
Guillain-Barré syndrome (GBS)	Accepted^[1]	IV	Anti-ganglioside
Hashimoto's encephalopathy	Accepted^[1]	IV	alpha-enolase^[30]
Hashimoto's thyroiditis	Accepted^[1]	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^[31]
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.^[29]
Idiopathic thrombocytopenic purpura (See Autoimmune thrombocytopenic purpura)	Accepted^[1]	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^[32]			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,^[33] LKM-1, LKM-2 or LKM-3; antibodies against soluble liver antigen^[34]^[35](anti-SLA, anti-LP) no autoantibodies detected (~20%)^{[उद्धरण चाहिए]} \|\|
Lupus erythematosus	Accepted^[1]	III	Anti-nuclear antibodies^[36] anti-Ro.^[37] Also, they are often present in Sjögren's syndrome.^[38]^[39]
Majeed syndrome				LPIN2
Ménière's disease		III?	major peripheral myelin protein P0^[40]
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^[1]		anti-nuclear antibody anti-U1-RNP	HLA-DR4
Morphea	Suspected^[41]
Mucha-Habermann disease aka Pityriasis lichenoides et varioliformis acuta				T-cells
Multiple sclerosis	Suspected	IV		PECAM-1^[42] Anti-Myelin Basic Protein
Myasthenia gravis	Accepted^[1]	II	nicotinic acetylcholine receptor MuSK protein	HA-B8 HLA-DR3 HLA-DR1
Myositis				see 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]
Neuromyotonia	Suspected^[50]	II?	voltage-gated potassium channels.^[50]
Occular cicatricial pemphigoid		II?	BP-1, BP-2	C3 deposition
Opsoclonus myoclonus syndrome	Suspected	IV?		Lymphocyte recruitment to CSF^[51]
Ord's thyroiditis
Palindromic rheumatism			anti-cyclic citrullinated peptide antibodies (anti-CCP) and antikeratin antibodies (AKA)^[52]
PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcus)	Suspected	II?		antibodies against streptococcal infection serve as auto-antibodies
Paraneoplastic cerebellar degeneration		IV?^[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 syndrome			ANA
Parsonage-Turner syndrome
Pars planitis
Pemphigus vulgaris	Accepted^[1]	II	Anti-Desmoglein 3
Pernicious anaemia	Accepted^[56]	II	anti-parietal cell antibody
Perivenous encephalomyelitis
POEMS syndrome				interleukin 1β, interleukin 6 and TNFα. vascular endothelial growth factor (VEGF), given the .^[57]
Polyarteritis nodosa
Polymyalgia rheumatica
Polymyositis	Accepted^[17]		IFN-gamma, IL-1, TNF-alpha
Primary biliary cirrhosis	Accepted^[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 cholangitis				overlap with primary biliary cirrhosis?
Progressive inflammatory neuropathy	Suspected
Psoriasis	Accepted^[59]	IV?		CD-8 T-cells, HLA-Cw6, IL-12b, IL-23b, TNFalpha, NF-κB
Psoriatic arthritis	Accepted^[60]	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^[61]
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^[1]	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,^[62] anti-DNase B, ASO
Sarcoidosis	Suspected	IV^[63]^[64]		BTNL2; HLA-B7-DR15; HLA DR3-DQ2.^[65]
Schizophrenia	Suspected^[66]^[67]^[68]
Schmidt syndrome another form of APS			anti-21 hydroxylase, anti-17 hydroxylase^[69]	DQ2, DQ8 and DRB1*0404
Schnitzler syndrome				IgM?
Scleritis
Scleroderma	Suspected^[41]	IV?	Scl-70 Anti-topoisomerase	dysregulated apoptosis?
Serum Sickness		III
Sjögren's syndrome	Accepted^[1]		Anti-ro.^[37] Also, they are often present in Sjögren's syndrome.^[38]^[39]
Spondyloarthropathy				HLA-B27
Still's disease see Juvenile Rheumatoid Arthritis			ANA	macrophage migration inhibitory factor^[70]
Stiff person syndrome	Suspected		glutamic acid decarboxylase (GAD),^[71]	GLRA1 (glycine receptor
Subacute bacterial endocarditis (SBE)		III^[72]	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^[1]	IV
Thrombocytopenia		II	glycoproteins 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 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^[1]	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^[75]	clinically may resemble type I hypersensitivity!
Vasculitis	Accepted^[9]	III	sometimes ANCA
Vitiligo	Suspected^[76]^[77]			NALP-1 RERE, PTPN22, LPP, IL2RA, GZMB, UBASH3A and C1QTNF6
Wegener's granulomatosis	Accepted^[78]		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 [1] :
- 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

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प्रतिरक्षा प्रणाली (immune system)
स्वरोगक्षमता (autoimmunity)

बाहरी कड़ियाँ

साँचा:स्वप्रतिरक्षित रोग

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स्वप्रतिरक्षित रोग

अनुक्रम

मानदण्ड (क्राइटेरिया)

वर्गीकरण

चिकित्सा-विकास

इन्हें भी देखें

अन्य पठनीय सामग्री

सन्दर्भ

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बाहरी कड़ियाँ

स्वप्रतिरक्षित रोग वर्गीकरण एवं बाह्य साधन
आईसीडी-१०	D84.9, M35.9
आईसीडी-९	279.4
ओएमआईएम	109100
डिज़ीज़-डीबी	28805
मेडलाइन प्लस	000816
एम.ईएसएच	D001327