Rheumato

Systemic lupus erythematosus

Intro: Autoimmune P=15-80/100,000 >M(9:1) 20-40y FH 20x
Sign &Symptom : CC->50%rheumato 20%skin
Skin : 1 ACLE(malar) / SCLE( scar) / CCLE(DLE=erythematosus plaque / ->follicular plugging&central
atrophic scar @face, scalp,ear,V-shaped,arm)
2 photosens,allopacia,oral ulcer,livedo reticularis,vasculitis
Bone&muscle : arthritis>1 poly~RA not persistant,no deformity(Jaccoud,s arthropathy=deformitym tendon&tissue)
Urinary :albuminuria all, microscopic hematuria80% ,nephritis45%(risk->,<30y,not Europe)LN classV
Neuro : cognitive defect,seizure,behave ch,psychosis myelopathy,peripheral neuropathy,headache,stroke,chorea ddx-drug/inf/E-lite /hypertensive encep/uremia
Cardio :pericarditis18%myocarditis15% cardiac temponade pulse , arrhythmia, cardiomegaly ,pulHTN
Hemato :anemia(ACD50%AIHA10%micro spherocyte,reti count),plt clinical Txplt<500,000/cm
3
,wc Tx,*APS clinical
thromboembolism/+ALP Ab >3mo(LA,anticardilipin IgG/M,anti
2
glycoprotein)Tx:anticoag life long
General :( ifx)
Dx&Inv : CBC,UA,Cr,sediment,24hr urine pro(spot UPCR),Alb,Glob,choresterol,TG,LDL,E-lyte,CXR,sero[ANA,anti-DNA,(anti-Sm,anti-phopholipid Ab<clinical
thrombosis/abortion APS>) => anti-DNA,serum C]
(LN) Indication (2012) Cr rising/ proteinuria>=1g/24hr / proteinuria>=0.5g/24hr+hematuria>5/HPF or proteinuria>=0.5g/24hr+cellular cast 2
SLE chill+leucopenia ,C, anti-DNA,respond to pred20-40mg/d 24-48hr
Aggravating factors:sunlight/ifx/stress/preg/abortion/drug(E)/compliance
Tx : 1)mild form [fever,arthritis,mild serositis+sm effusion,rash,fatigue,headach,leucopenia/lymphopenia] -> antimararial/NSAID/local Corticosteroid
ASA/NSAID for arthritis, serositis,vasculitis
Antimararial(Chloroquine<=4MKD/Hydroxychloroquine<=6.5MKD) for fatigue,skin, arthritis
Local Corticosteroid for skin lesion low dose Systemic Corticosteroid(<0.5MKD)
2)severe [massive eff,myocarditis,lupus pneumonitis,lung hem,orrhage,renal,AIHA/thrombocytopenia,neuropsychiatric lupus,vasculitis,] -> hi dose Corticosteroid/immunosupp drug
Corticosteroid(pred/dexa1MKD)
Immunosupp drug :Cyclophosphamide Indication hi dose steroid>2mo,steroid,side eff
Standard Tx LN II/IV/V : 1)Induction ph -> pred1MKD+pulse Cy500-750mg/BSA q mox6 ->maintainace ph q3mo18mo(NIH regimen)long term side effectrisk CA,
2)Maintainance ->dose pred&pulse Cy sequential Tx AZA50-100mg/d or mycofenolate mofetil 3mo18mo
If suspected RPGN : pulse methyl pred1g/d (3d)->pred0.5MKD
Additive Tx: Antimararial exacerbate renal inv thrombosis
proteinuria>=0.5g/24hr ACEI or ARB
Other Tx: keep BP<130/80mmHg ,LDL<100mg/dl,>=6mo preg