Rheumato

Systemic sclerosis




Intro: Autoimmune P=20/1,000,000 >M(3-5:1) 30-50y vv&skin cc=Raynaud phenomenon+
Dx : scleroderma-like condition -> diabetic chairoarthropathy( DM+no digital pitting scar),reflex sympathetic dystrophy(asymmetric),amyloidosis,mycosis fungoides,GVsH













Sign&Symptom :
Raynaud phenomenon : 95% --
Skin :skin edema-fibrosis-atrophy SSS(modified Rodnan skin score)17x0-3score(max51) if>=20risk renal crisis f/u , loss of skin crease@ face&forehead ,perioral radial
furrow ,hair follicle -> , melanin pigment hypo hyper=salt-and-pepper appearance
Joint&muscle : limited ROM, arthralgia>itis, tendon friction rubtendon skin,weakness /polymyositis,calcinosis@/ ,olecranon,pre-patellar (Calcinosis
Raynaud Esophageal dysmotility Sclerodactyly Telangectasia)
GI : 90%motility 2/3lower, (<3cm),GERD,constipation.diarrheasm bac overgrowth Rx ATB
Respi : diffuse form75% ILD ( /,fine crackles),pleural eff(pleural friction rub),PFT(restrivtive), DCLO,alveolitis(ground-glass pattern),bronc->neu&Eo=active disease
Cardio : limited form 50% PHT ,diffuse formpul fibrosis >> ,prominent a wave, lound P2, S4, PHT>20 >30 ,arrhythmiafibrosis,pericarditis,pericardial eff
Nephro : 12%diffuse risk w/ African ,preg, steroid>20-30mg/d 5 *scleroderma renal crisis (=1
st
cause of morbid/mortal) vv destruction&stenosis->ischemia-> +
renin/angiotensin/aldosterone->HT acute&severe HT+renal failure headache,blurr vision,uremia,seizure(hypertensive encep) lab=>proteinuria,microscopic hematuria,granular cast,BUN/Cr,pul
edema, MAHA
Inv : CNT CBC, UA,BUN,Cr,ESR,ANA
Evaluation : 1.diffuse or limited 2.inv organ? 3.progress grade? 4.bad prog?(diff,lim w/ PHT,rapid progress, SSS,tendon friction rub, periungual telangiectasia, limited jaw opening,vital org inv
:lung,renal,heart) 5.exacerbate?
Tx : skin thickeninginflame ->SSS->MTX,Cy,Cyclosporine AZA,mycophenolate colchicin fibrosis
Raynaud vasoconstric->digital ulcer/gg->vasodilate(CCB,alpha block,ACEI) bosentan,sildenafil,iloprost,epoprostenol
PHT vasoconstric->functional class,6-min walk test,CXR,EKG,Echo,cardiac cath-> vasodilate Tx PHT if>=class2 sidenafil+bosentan+iloprost or epoprostenol
ILD inflame/fibrosis->CXR,LFT w/ DCLO,HRCT->symptomatic Tx alveolitis pred<20mg/d + Cy
GERDGI dysmotility ->scope,manometry ->PPI,LSM
DiarrheaGI dysmotility ->stool exam,scope,H
2
breath test ->short course ATB (ciprofloxacin/metro/doxy 10-14d)
ConstipationGI dysmotility ->laxative
Scleroderma renal crisis vasoconstric ->new onset +Cr, MAHA->ACEI,dialysis
Prognosis :
scleroderma
localized
( )
morphea
(patch)
linear
scleroderma
SS
limited cutaneous
diffuse
cutaneous