KEGG   DISEASE: H00080Help
Entry
H00080                      Disease                                

Name
Systemic lupus erythematosus
Description
Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterised by the production of IgG autoantibodies that are specific for self-antigens, such as DNA, nuclear proteins and certain cytoplasmic components, in association with a diverse array of clinical manifestations. The primary pathological findings in patients with SLE are those of inflammation, vasculitis, immune complex deposition, and vasculopathy. Immune complexes comprising autoantibody and self-antigen is deposited particulary in the renal glomeruli and mediate a systemic inflammatory response by activating complement or via Fc{gamma}R-mediated neutrophil and macrophage activation. Activation of complement (C5) leads to injury both through formation of the membrane attack complex (C5b-9) or by generation of the anaphylatoxin and cell activator C5a. Neutrophils and macrophages cause tissue injury by the release of oxidants and proteases.
Category
Immune system disease
BRITE hierarchy
Pathway
Systemic lupus erythematosus
Gene
HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752]
HLA-DQA1 (polymorphism) [HSA:3117] [KO:K06752]
HLA-DQB1 (polymorphism) [HSA:3119] [KO:K06752]
C2 (deletion) [HSA:717] [KO:K01332]
C4 (null alleles) [HSA:720] [KO:K03989]
TNF-alpha (polymorphism) [HSA:7124] [KO:K03156]
Fc gamma RIIA (polymorphism) [HSA:2212] [KO:K06472]
Fc gamma RIIB (polymorphism) [HSA:2213] [KO:K12560]
Fc gamma RIIIA (polymorphism) [HSA:2214] [KO:K06463]
Fc gamma RIIIB (polymorphism) [HSA:2215] [KO:K06463]
PARP (polymorphism)
CRP (polymorphism) [HSA:1401] [KO:K16143]
TLR5 (polymorphism) [HSA:7100] [KO:K10168]
PDCD1 (polymorphism) [HSA:5133] [KO:K06744]
OAZ (polymorphism) [HSA:23090]
Env factor
Hydrazines
Procainamide [DR:D00477]
Chlorpromazine [DR:D00270 D00789]
Isoniazid [DR:D00346 ]
Phenytoin [DR:D00512 ]
Penicillamine [DR:D00496]
Tobacco smoke
Hair dyes
Ultraviolet light
Epstein-Barr virus
Marker
Anti-C1q
Anti-dsDNA
C3 [HSA:718]
C4d
CR1/E [HSA:1378]
CRP [HSA:1401]
CD27 [HSA:939]
IL-6 [HSA:3569]
IL-10 [HSA:3586]
IL-12 [HSA:3594 3565]
IL-13 [HSA:3596]
IL-16 [HSA:3603]
IL-18 [HSA:3606]
INF-alpha
TNF-alpha [HSA:7124]
Urine MCP-1
IL2R [HSA:3559 3560 3561]
TNFR
IL1Ra (IL-1R antagonist) [HSA:3557]
CD27 [HSA:939]
CD154 [HSA:959]
ICAM-1 [HSA:3383]
sVCAM-1 [HSA:7412]
Thrombomodulin [HSA:7056]
Drug
NSAID
Hydroxychloroquine [DG:DG01015]
Methylprednisolone [DG:DG00410]
Prednisone [DR:D00473]
Cyclophosphamide [DG:DG00675]
Mycophenolate mofetil [DR:D00752 D05094]
Azathioprine [DG:DG00743]
Rituximab [DR:D02994]
Epatuzumab
Abetimus sodium [DR:D03204]
CTLA-4-Ig
Comment
Autoantigen:
Double-stranded DNA [CPD:C00434]
H2A [HSA:474382]
H2B [HSA:8346]
H3 [HSA:3020]
H4 [HSA:8359]
Ro/SS-A [HSA:6737]
La/SS-B [HSA:6741]
Sm-B [HSA:6628]
Sm-D [HSA:6632]
NR2A [HSA:2903]
NR2B [HSA:2904]
Phosphatidyl-serine [CPD:C02737]
C1q [HSA:712]
Other DBs
ICD-10: 
MeSH: 
OMIM: 
Reference
PMID:12835292 (description, gene, env_factor, autoantigen)
  Authors
Mok CC, Lau CS.
  Title
Pathogenesis of systemic lupus erythematosus.
  Journal
J Clin Pathol 56:481-90 (2003)
Reference
PMID:18305268 (gene, autoantigen)
  Authors
Rahman A, Isenberg DA.
  Title
Systemic lupus erythematosus.
  Journal
N Engl J Med 358:929-39 (2008)
Reference
PMID:18075789 (gene)
  Authors
Rhodes B, Vyse TJ.
  Title
General aspects of the genetics of SLE.
  Journal
Autoimmunity 40:550-9 (2007)
Reference
PMID:16941108 (gene)
  Authors
Wong M, Tsao BP.
  Title
Current topics in human SLE genetics.
  Journal
Springer Semin Immunopathol 28:97-107 (2006)
Reference
PMID:18028758 (env_factor)
  Authors
Albilia JB, Lam DK, Clokie CM, Sandor GK.
  Title
Systemic lupus erythematosus: a review for dentists.
  Journal
J Can Dent Assoc 73:823-8 (2007)
Reference
PMID:16373251 (env_factor)
  Authors
Sarzi-Puttini P, Atzeni F, Iaccarino L, Doria A.
  Title
Environment and systemic lupus erythematosus: an overview.
  Journal
Autoimmunity 38:465-72 (2005)
Reference
PMID:17307106 (env_factor)
  Authors
D'Cruz DP, Khamashta MA, Hughes GR.
  Title
Systemic lupus erythematosus.
  Journal
Lancet 369:587-96 (2007)
Reference
PMID:16168709 (marker)
  Authors
Croker JA, Kimberly RP.
  Title
SLE: challenges and candidates in human disease.
  Journal
Trends Immunol 26:580-6 (2005)
Reference
PMID:16093831 (marker)
  Authors
Liu CC, Manzi S, Ahearn JM.
  Title
Biomarkers for systemic lupus erythematosus: a review and perspective.
  Journal
Curr Opin Rheumatol 17:543-9 (2005)
Reference
PMID:15248202 (marker)
  Authors
Illei GG, Tackey E, Lapteva L, Lipsky PE.
  Title
Biomarkers in systemic lupus erythematosus: II. Markers of disease activity.
  Journal
Arthritis Rheum 50:2048-65 (2004)
Reference
PMID:15355751 (marker)
  Authors
Illei GG, Lipsky PE.
  Title
Biomarkers in systemic lupus erythematosus.
  Journal
Curr Rheumatol Rep 6:382-90 (2004)
Reference
(drug)
  Authors
McPhee SJ, Papadakis MA, Tierney LM (ed).
  Title
Current Medical Diagnosis & Treatment 2007, Forty-Sixth Edition
  Journal
The McGraw-Hill Companies, Inc. (2007)
Reference
PMID:18068856 (drug)
  Authors
King JK, Hahn BH.
  Title
Systemic lupus erythematosus: modern strategies for management: a moving target.
  Journal
Best Pract Res Clin Rheumatol 21:971-87 (2007)
Reference
PMID:17699298 (drug)
  Authors
Ponticelli C.
  Title
New therapies for lupus nephritis.
  Journal
Clin J Am Soc Nephrol 1:863-8 (2006)
Reference
PMID:15380523 (autoantigen)
  Authors
Hoffman RW.
  Title
T cells in the pathogenesis of systemic lupus erythematosus.
  Journal
Clin Immunol 113:4-13 (2004)
Reference
PMID:16932712 (autoantigen)
  Authors
Cook HT, Botto M.
  Title
Mechanisms of Disease: the complement system and the pathogenesis of systemic lupus erythematosus.
  Journal
Nat Clin Pract Rheumatol 2:330-7 (2006)

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