Entry 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.
Class Human Diseases; Immune diseaseBRITE hierarchy
Pathway map ko05322 Systemic lupus erythematosus
Ortholog table Disease H00080 Systemic lupus erythematosus
Orthology K03986 complement C1q subcomponent subunit A
K03987 complement C1q subcomponent subunit B
K03988 complement C1q subcomponent subunit C
K11086 small nuclear ribonucleoprotein B and B'
K11087 small nuclear ribonucleoprotein D1
K11088 small nuclear ribonucleoprotein D3
K05209 glutamate receptor ionotropic, NMDA 2A
K05210 glutamate receptor ionotropic, NMDA 2B
K11089 60 kDa SS-A/Ro ribonucleoprotein
K10784 T cell receptor alpha chain V region
K10785 T-cell receptor beta chain V region
K03161 tumor necrosis factor ligand superfamily member 5
K03160 tumor necrosis factor receptor superfamily member 5
K03156 tumor necrosis factor superfamily, member 2
K06856 immunoglobulin heavy chain
K03997 complement component 8 subunit alpha
K03998 complement component 8 subunit beta
K03999 complement component 8 subunit gamma
K06498 high affinity immunoglobulin gamma Fc receptor I
K06472 low affinity immunoglobulin gamma Fc receptor II-a
K06463 low affinity immunoglobulin gamma Fc receptor III
Compound Reference Authors Rahman A, Isenberg DA.
Title Systemic lupus erythematosus.
Journal Reference Authors Mok CC, Lau CS.
Title Pathogenesis of systemic lupus erythematosus.
Journal Reference Authors Clatworthy MR, Smith KG.
Title B cells in glomerulonephritis: focus on lupus nephritis.
Journal Reference Authors Couser WG.
Title New insights into mechanisms of immune glomerular injury.
Journal West J Med 160:440-6 (1994)
Reference Authors Cook HT, Botto M.
Title Mechanisms of Disease: the complement system and the pathogenesis of systemic lupus erythematosus.
Journal Reference Authors Carroll MC.
Title A protective role for innate immunity in systemic lupus erythematosus.
Journal Reference Authors Sturfelt G, Truedsson L.
Title Complement and its breakdown products in SLE.
Journal Reference Authors Lu L, Kaliyaperumal A, Boumpas DT, Datta SK.
Title Major peptide autoepitopes for nucleosome-specific T cells of human lupus.
Journal Reference Authors Zieve GW, Khusial PR.
Title The anti-Sm immune response in autoimmunity and cell biology.
Journal Reference Authors Scofield RH, Farris AD, Horsfall AC, Harley JB.
Title Fine specificity of the autoimmune response to the Ro/SSA and La/SSB ribonucleoproteins.
Journal Reference Authors Hoffman RW.
Title T cells in the pathogenesis of systemic lupus erythematosus.
Journal Related ko04060 Cytokine-cytokine receptor interaction
ko04610 Complement and coagulation cascades
ko04612 Antigen processing and presentation
ko04660 T cell receptor signaling pathway
ko04662 B cell receptor signaling pathway
ko04670 Leukocyte transendothelial migration
LinkDB All DBs
