Systemic lupus erythematous defin
Multisys AI dis. Ab produc against various Ag. Polyclonal B cell sec.
tiss dam by eg imm complex format and deposit, complem activat etc.
Feats
Remitting and relapsing.
Non specif eg malaise, fatigue, myalgia, fever.
Malar rash, discoid rash, photosensitiv, oral ulcer, non erosive arthritis, serositis, renal disord eg proteinuria, CNS disord eg seiz, blood disord eg haemolytic anaem/leukopenia/lymphopenia/thrombocytopenia, imm disord eg anti dsDNA/anti Sm/anti Plip.
Lymphadenopathy, wl, alopecia, nail fold infarct, non infec endocarditis, raynauds.
Antinuclear Ab (ANA) in 95%
Immunology and investig
95% ANA pos Anti dsDNA in 60% ENA up to 30% 40% RhF pos Anti Plip AI thyr dis in up to 10% Other investig- Low C3 and 4 Somet high ESR CRP NORMAL
Drug induced
Isoniazid Procainamide Chlorpromazine Phenytoin Quinidine
Mx
-severe flares- Cyclophosphamide and high prednisolone -cutaneous- Topical steroids, sun cream preven -maint- NSAIDs, hydroxychloroquine, low steroids, azathioprine, methotrexate, mycophenolate. -nephritis- Cycophsphamide, mycophenolate. -B cell deplet- Rituximab
Prog
80% surv at 15yr