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
Human diseases [BR:br08402]
 Immune system diseases
  Allergies and autoimmune diseases
   H00080  Systemic lupus erythematosus
Human diseases in ICD-10 classification [BR:br08403]
 13. Diseases of the musculoskeletal system and connective tissue (M00-M99)
  M30-M36  Systemic connective tissue disorders
   M32  Systemic lupus erythematosus
    H00080  Systemic lupus erythematosus
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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