EVALUARE SI MONITORIZARE

IN LUPUSUL ERITEMATOS
SISTEMIC

Codrina Ancuta
MD, PhD

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 LES
n boala autoimuna multi-sistemica, prototipul bolii prin
complexe imune
n susceptibilitate genetica & factor trigger (mediu,
medicamente, etc)
n tablou clinic complex = suma manifestarilor diferite
organe/sisteme
activitate (fenomen reversibil) &
distrugere/damage tisular & efecte
secundare medicatie (corticoterapie,
imunosupresoare) & co-morbiditati (ATS
accelerata, infectie, osteoporoza, etc)

n evolutie variabila, cu episoade remisiune & recaderi

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 CRITERII ACR (1997)
Diagnostic pozitiv = prezenta
simultana/succesiva a minim 4 din 11
criterii
Criteriu Descriere
1. RASH MALAR Butterfly, eritem in fluture

2. LUPUS DISCOID
3. FOTOSENSIBILITATE
4. ULCERATII ORALE Nedureroase, observate medic

5. ARTRITA Neeroziva, > 2 arii articulare,

simetrica
6. SEROZITA Pleurezie, pericardita

7. AFECTARE RENALA - proteinurie persistenta >0.5g/zi sau

>3+ sau
- cilindri hematici, granulosi, mixti

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 CRT. ACR Descriere
Criteriu revizuite LES-1997
8. AFECTARE - convulsii/psihoza - in absenta cauzelor
NEUROLOGICA medicamentoase, metabolice,
hidroelectrolitice;
9. AFECTARE anemie hemolitica cu reticulocitoza
HEMATOLOGICA leucopenie < 4000mmc la >2 determinari
limfopenie < 1500mmc la >2 determinari
trombocitopenie <100 000/mmc in absenta
medicatie inductoare

10. ANOMALII - ac anti-ADN dc

IMUNOLOGICE - ac anti-Sm
- ac anti-fosfolipide: anticoagulant lupic sau
test fals pozitiv pentru lues de cel putin 6
luni confirmat prin fluorescenta
11. ANA totali titru crescut in absenta medicamentelor
inductoare

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 EVALUARE SI MONITORIZARE LES

EVALUARE MONITORIZARE
DOMENII n raspuns la tratament
n activitate n profil tolerabilitate si
n distructie/damage siguranta
n functional si calitatea
vietii
articular si sistemic
comorbiditati

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 n Evaluarea initiala n Evaluari ulterioare
diagnostic pozitiv activitate boala
stabilirea activitatii bolii distructie/ damage tisular
stabilirea impactului afectarea fuctionala
functional
identificarea factori de
prognostic negativ
n ACTIVITATE = fenomen
legati de boala, co- reversibill
morbiditati (cardio- n DAMAGE/DISTRUCTIE
vascular, osteoporoza, = fenomen ireversibil
etc)

DECIZIA TERAPEUTICA OPTIMA

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 EVALUARE CLINICA
n initial - obligatorie pentru diagnosticare
n in dinamica
manifestari clinice noi
manifestari clinice persistente / agravate
efecte secundare medicamentoase
complicatii infectioase, etc
prezenta factori risc cardio-vascular, osteoporoza, etc
n examen clinic complet (inclusiv IMC, temperatura,
diureza, fanere, examen mucoase)

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 EVALUAREA PARACLINICA
Sindrom inflamator in principal VSH si CRP
n Algoritm functie de valoarea CRP
Titru normal caracteristic LES (artrita neeroziva!)
Titru crescut cauze:
infectie intercurenta (CRP>50g/l probabilitate
maxima de infectie intercurenta)
serozita
artrita eroziva (Rupus)

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 Bilant hematologic
HLG completa & reticulocite
test Coombs, bilirubina
sideremie, feritina, transferina
eventual punctie medulara

n citopenii
q anemie hemolitica, feripriva, inflamatorie,
toxicitate medicamentoasa
q leucopenie & limfopenie/neutropenie
q trombocitopenie imuna, toxica medicamentoasa

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 n Sindrom imunologic
anticorpi ANA totali, anti-ADNdc , anti-Sm, anti-Ro si
anti-La, anti-fosfolipidici (cardioliopina, anticoagulant
lupic, anti-2GPI; aPTT, VDRL), factor reumatoid,
CH50 si fractiunile C3, C4, anti-eritrocite, anti-GA,
anti-trombocite, anti-neuronali)

Utili pt diagnostic LES (anti-ADNdc, ANA totali,

etc), indica afectarea predominanta a unui
organ/sistem (anti-Sm, renal, etc)

Evaluarea in dinamica & raspuns la tratament

pt ADNdc poate evidentia evolutia favorabila,
recaderea, etc

Ac anti-Sm doar cu valoare diagnostica

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 n Teste functionale renale
Sangvin (uree, creatinina & clearance creatinina, ac
uric, ionograma)
Urinar (sumar, proteinurie /24h, Addis, biopsie renala
stadializare nefropatie)

n Teste functionale hepatice pt toxicitate

medicamentoasa vs hepatita autoimuna vs hepatita
virala
TGO, TGP, fosfataza, bilirubina, GGT, electroforeza,
explorare hepatita autoimuna: ac anti-mitocondriali,
anti-LKM1; markeri virali hepatici)

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 n Alte explorari:

Pulmonare: Rg, TFR, CT inalta rezolutie, lavaj

bronhoalveolar
Cardio-vasculare: ekg, ecocardiografie, eco
Doppler vascular;
IRM cerebral/CV
VCM
Biopsie musculara/cutanata
DXA- FR prezenti
HIV

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 EVALUARE FACTORI PROGNOSTIC
NEGATIV - RISC CARDIOVASCULAR

n Clasici - hipercolesterolemia, obezitatea, HTA,

DZ, sedentarism, fumat
n Aditivi LES= factor risc independent,
nefropatia, vasculita, corticoterapia, ac anti-
fosfolipide, homocisteina, anomalii hormonale
IDEAL
n LDL-col < 2.6mmol/l & glicemia < 7
mmol/l & TA< 130/80 mmHg & IMC<
25kg/m2

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 EVALUARE FACTORI RISC
OSTEOPOROZA
Factori majori Factori minori
Varsta > 65 ani Boli inflamatorii (PR, LES)
Fractura vertebrala Hipertiroidism in antecedente
Fractura de fragilitate >40 ani Trat anticonvulsivant cr
AHC fractura (mama sold) Dieta saraca Ca
CS p.o.> 3 luni Fumat
Sdr malabsorbtie Alcool excesiv
Hiperparatiroidism primar Cafea abuz
Tendinta la cadere G<57 Kg
Osteopoenie radiologica Scadere ponderala >10% peste 25
ani
Hipogonadism Anticoagulante cronice
Menopauza precoce (<45 ani)

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 INDICI ACTIVITATE LES
COMPUSI INDIVIDUALI
SLEDAI (Systemic Lupus
Erythematosus Disease n BILAG (British Isles
Activity Index) Lupus Assessment
Group)
SLAM (System Lupus
Activity Measure)

ECLAM (European Forma LES: activ/

Community Lupus Activity
Measure) persistent activ/inactiva

Recadere vs remisiune
LAI (Lupus Activity Index)
Raspuns la trat: fara
SIS (SLE Index Score) raspuns vs imbunatatire

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 SLEDAI

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 SLEDAI
n Permite evaluarea activitatii LES in ultimile 10 zile
n Interpretare - Forme de boala
LES inactiv SLEDAI=0
LES usor activ scor 1-5
LES moderat activ scor 6-10
LES cu activitate inalta scor 11-19
LES f. activ scor >20

Recadere = crestere scor cu mai mult de 3 pcte

Remisiunea = SLEDAI 0
Imbunatatire = reducerea scor cu peste 3 pcte
Boala persistent activa = schimbare scor cu 1-3pct

Scor > 5=initiere/schimbare tratament

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 SLAM

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 SLAM
n Evalueaza activitatea LES in ultima luna
n Cuprinde 30 intrebari - 11 organe/sisteme & 8
laborator
n Scor: 0-84

Dezavantaje
Indice subiectiv simptome
(artralgie/milagie/durere abdominala/fatigabilitate)
Interpretare egala pt manifestarile usoare si
severe

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 ECLAM

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 ECLAM

n Evalueaza activitatea bolii in ultima luna

n cuprinde 15 intrebari (clinice,
serologice)
n Scor = 0-10

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 SIS

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 BILAG

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 BILAG
n Activitatea bolii in ultima luna

n Indice de activitate individual, pe organe/sisteme:

General
Mucocutanat
Neurologic
Musculoscheletal
Cardiovascular
Renal
Respirator
hematologic

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 n Avantaje
activitatea si raspunsul la trat pe fiecare org/sistem
totodata un scor global

n Critici
absenta afect oftalmologice
Absenta afectarii gastrointestinale
Absenta criteriilor imunologice

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 n Recadere severa cresterea oricarui scor la
scor A (>9)

n Recadere moderata cresterea de la scor C

sau D sau E la scor B ( >3)

n Raspuns la tratament pierderea scorului A

si/sau B in toate sistemele, fara dezvoltarea
oricarui scor A sau B

n Raspuns partial pierderea scor A &

persistenta/ dezvoltarea unuia sau a mai multor
scoruri B
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 BILAG ECLAM SLAM SLEDAI LAI

Nr intrebari 86 30 30 24 14

Nr organe/sisteme 8 10 9 9 8

Interval de evaluare
28 28 28 10 14
(zile)
Scor Individual Global Global Global Global

Obiectiv/subiectiv Ambele ambele Subiectiv Obiectiv ambele

Ponderea variabile NU DA da Da NU

Evaluare severitate DA NU Da NU da

Teste imunelogice NU Yes NU Da da

Terapie NU NU NU NU da

Calcul retrospectiv NU Da Da da da

Modificat in sarcina NU Da Da da da

LES juvenil DA Da Da da NU

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 EVALUARE DAMAGE

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 EVALUAREA DAMAGE
modificari persisitente de minim 6 luni in
ciuda tratament (anatomice, fiziologice,
patologice, functionale) & complicatii
tratament & comorbiditati

n SLICC/ACR (Systemic Lupus International

Cooperating Clinics/American College of
Rheumatology)
evalueaza efectul cumulativ al bolii de la debut si
modificarile organice inregistrate cu o durata > 6 luni

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 EVALUAREA CALITATII VIETII

Indici nespecifici, cu aplicabilitate in toate

suferintele reumatismale
n Health Assesment Questionare (HAQ)
n Short Form 36 (SF-36)

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 EVALUAREA RASPUNSULUI LA
TRATAMENT
Indice specific RIFLE (Responder Index for
Lupus Erythematosus)
n manifestarile clinice pt diferite organe/sisteme
sunt apreciate ca:
absente

agravate
prezente/ persistente
raspuns partial
remisiune

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 EVALUAREA EFECTE
SECUNDARE TRATAMENT

n Hematologice
n Hepatice
n Osoase osteoporoza corticoid-indusa,
osteonecroza aseptica
n Infectioase
n Psihice
n Musculare
n Oculare

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 EVALUARE FRECVENTA
Anamneza & examen obiectiv (inclusiv TA, IMC) La fiecare vizita

Indici activitate - SLEDAI, BILAG La fiecare vizita

HLG completa; VSH & CRP; Creatinina; ANA, Ac
antiADN dc; CH50, C3, C4; urina & La fiecare vizita
proteinurie/24h & Addis; bilant metabolic
Baseline & anual sau
CK, TSH, imunograma
cand exista indicatie
Ac anti-La, Ro, Sm, RNP, anticoagulantul lupic Baseline si anual
Baseline & daca exista
Ac anticardiolipina indicatie clinica si/sau
annual
DXA lombar, sold baseline, la 2 ani interval

rap urinar P/creatinina, TF respiratorii, ECG, Cand exista indicatie

echocardiograma, VCN, IRM cerebral clinica
Calitatea vietii (SF-36) 36 luni

SLICC/ACR damage index

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anual