42. SYSTEMIC LUPUS ERYTHEMATOSUS Flashcards
Genetic predisposition:
HLA-DRB1, HLA-DQB, HLA-DR2, HLA-DR3
+ higher risk in those with c1q, c2, c4 deficiency
Serology:
ANA or anti-dsDNA antibodies
Positive anti-Smith nuclear antigen
Positive lupus anticoagulant
False-positive serologic test for Treponema pallidum
Antiphospholipid antibodies
Drug-induced lupus serology:
Antihistone antibodies
High ANA titer
Negative anti-DNA antibodies
Normal complement
Drug-induced lupus etiology:
Anticonvulsants
Sulfonamides
Antiarrhythmic drugs
Treatment:
Diffuse:
Glucocorticosteroids in high doses and cyclophosphamide (500-800mg/m2)
+ immunosuppressant - methotrexate, leflunomide, azathioprine, myclophenolate mofetil
Hydroxychloroquine 5-7mg/kg/day to 400mg/day
Pathogenesis:
SLE = multisystem autoimmune disorder with impaired adaptive and innate immunity. characterised by production of large amounts of circulating autoantibodies and hypergammaglobulinemia
🡪This antibody production may be due to loss of T-lymphocyte control on B-lymphocyte activity, leading to hyperactivity of B lymphocytes, which leads to nonspecific and specific antibody and autoantibody production
🡪 autoantibodies form immune complexes that deposit on vessel walls, and inadequate clearance of these complexes results in immune response with tissue inflammation and ischaemia manifested as lupus nephritis and vasculitis