prezentare de caz clinic - umfiasi.ro de medicina... · 2015- imuran 100 mg si plaquenil 400 mg...
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Prezentare de caz clinic
Medic rezident: Rusu Sonia
Indrumator: Dr Mioara Tigau
Coordonator activitate didactica: Conf Dr Elena Rezus
L.E. 37 ani ,sex feminin, mediu rural
MI : dureri cu caracter inflamator la nivelul artic mici maini,picioare,umeri,coate,genunchi bilateral
fatigabilitate marcata
fotosensibilitate
prurit generalizat
AHC : tata --boala Parkinson
mama –HTAE
APF: PM=14 ani , UM=martie 2016 , N=1, A=1, S=2
APP:2002 - NCB C5 bilaterala algoparestezica
Fibromatoza uterina
Tiroidita autoimuna
Hipotirodie secundara
Steatoza hepatica
Obezitate gradul 1
Comportamente : fosta fumatoare 10 PA –abstinenta din 2005 , 1 cafea/zi,neaga consumul de alcool
CVM : pensionata pe caz de boala
Medicatie administrate inaintea internarii :
Imuran 50 mg 2cp/zi , Plaquenil 200 mg 2cp/zi , Prednison 5 mg 1cp/zi , Euthyrox 25 microgr 1cp/zi , Omeran 20 mg 1cp/zi , Movalis 15 mg 1cp/zi , Ketotifen 1mg 1cp/zi
Istoricul bolii :
Boala a debutat in 2002 prin dureri cu caracter inflamator la nivelul artic mici maini si picioare insotite de tumefactie,fotosensibilitate,rash malar,tulburare depresiva cu agravari anxioase si titrul crescut al Ac anti ADNdc,pentru care a urmat tratament cu Plaquenil 400mg/zi si Prednison 10 mg/zi,ulterior Ciclofosfamida pulsterapie 3200 mg in 6 luni cu evolutie favorabila episodica a bolii.
2006 – examen oftalmologic –modificari –se instituie tratament cu Imuran 100 mg/zi
2009 –puseu (CRP >45 mg/l)–Plaquenil 400mg/zi seara
Oct 2009 –Plaquenil +Imuran
2010 – leucopenie --doar Imuran
2011-SLEDAI-12 –boala activa se administreaza Plaquenil +Imuran
2012 – AND dc crescuti- pac nu tolereaza doze mari de PDN sau Plaquenil se mareste doza de Imuran la 150 mg/zi
2015- Imuran 100 mg si Plaquenil 400 mg
04.04.2016 –revine pentru dureri cu caracter inflamator la nivelul articulatiilor mici maini,picioare,umeri,coate,genunchi bilateral insotite de fatigabilitate marcata, fotosensibilitate si prurit generalizat.
Examen clinic obiectiv
Stare generala : relativ buna , T=1,61 m , G=87 kg ,IMC=33,5kg/m2
Facies :rash malar
Tegumente si mucoase : palide, vergeturi sidefii abdomen si coapse,
hemoragii gingivale , purpura gambe bilateral
Tesut conjunctivo-adipos – bine reprezentat
Sistem ggl : superficial nepalpabil
Aparat respirator si cardiovascular- in limite normale
Aparat digestiv,uro-genital,SN,endocrine,organe de simt – in limite normale
Aparat locomotor :durere si redoare la nivelul coloanei cervicale,umeri durerosi
la palpare pe stg>dr , retroplusia si rotatiile limitate algic pe ultimele grade,coate
dureroase la palpare,RCC cu extensia limitata pe ultimele grade,genunchi cu
durere la palparea pct topografice mai intens pe dr, cu usoara tumefactie la
nivelul patelei drepte,cursa rotulei limitata in sens latero-lateral si cranio-caudal
bilateral,glezne sensibile la palpare cu mobilitate pastrata.
Analize uzuale
HLG –
Formula leucocitara -normala
Hb=12,6 g/dl
VSH=39 mm/h
Biochimie –
CRP=20,41 mg/l
Colesterol = 118,15 mg/dl
Ex sumar urina –normal
Ag Hbs,Ac anti HVC -negativi
Imunologie
ANA 9 Profil
ADN mc =94
ADN dc=62
Sm=103
Cromatina=116
Scl-70=15
Centromer=80
RNP/Sm=13
SSA(Ro)=0
SSB(La)=0
Complement C3 =90,75 mg/dl
AC anti cardiolipina (Ig M,Ig
G) =44,46 U/ml
Ac anti beta 2-glicoproteina
1-Ig M=7,4 U/ml
Ac anti beta 2-glicoproteina
1-Ig G=60,4U/ml
Anticogulantul lupic = pozitiv
Investigatii paraclinice
Rx pulmonara si maini bilateral plaman fara leziuni focale evolutive si cord cu o configuratie
normal radiologic,discrete osteoporoza juxta-articulara,cateva microgeode in stiloidele cubitale.
Ecografie de maini sinovita extensori maini bilateral
Ecografie abdomino-pelvina
-Ficat marit LHD -160 mm,LHS-80 mm,moderat steatozic difuz,contine in segmentul VII o calcificare
de 6 mm ;
-Uter –contine cervico-istmic un nodul fibromatos de 65/60 mm
Diagnostic pozitiv
Lupus eritematos sistemic
Vasculita limfocitara secundara
Sindrom antifosfolipidic secundar
Tiroidita autoimuna
Hipotiroidie secundara
Fibrom uterin
Steatoza hepatica
Diagnostic diferential
Boala mixta de
tesut conjunctiv
Diagnostic
diferential
Dermatomiozita
Sdr
Felty(PR +
citopenie)
Poliartrita
reumatoida
Ac anti CCP +
Fazele tardive de boala-rx-leziuni distructive
din PR
Ac anti ADNdc ,Ac anti
Sm –specifici pt LES Leucopenie+
neutropenie
Anemie/Trombo
citopenie
+
splenomegalie
Leziuni
cutanate(eritem
facial,fotosensibilita
te)
CK,Aldolaza,TGO,LDH-
crescute
Model EMG miopatic
Biopsie musculara
LES+sclerodermie+poli
miozita+artrita
eroziva
Ac anti U1RNP
crescuti
Tratament
Obiective :
-reducerea inflamatiei sistemice
-scaderea activitatii bolii +/- obtinerea remisiunii
-prevalenta recaderilor
-contracararea riscului de aparitie a complicatiilor
-tratarea corecta a comorbiditatilor
Tratament non-farmacologic:
-evitarea frigului,umezelii,efortului fizic intens
-evitarea expunerii la soare si folosirea de crème cu SPF 30-50
-evitarea medicatiei ce poate determina fenomene de lupus –like si a celor care cresc fotosensibilitatea
-evita vaccinarea cu vaccinuri vii
-evita si trateaza correct infectiile intercurente
-consult oftalmologic la 6 luni
Tratament farmacologic:
LES:
-AINS-Movalis 15 mg/zi 10 zile/luna
-GC –Prednison 5mg/zi
-Imuran 100mg/zi si Plaquenil 400 mg/zi
-Ciclofosfamida – pulsterapie in formele severe de LES(vasculita)
SAFL : anticoagulante – heparina in tromboze acute
-- warfarina,acenocumarol ce se pot asocia cu aspirina in doze de 75-100 mg in
profilaxia trombozelor
Tratamentul comorbiditatilor si complicatiilor :
-Infectii -- mai frecvent respiratorii(bacil Koch,CMV,Virus varicelo-zosterian,Pneumocystis carinii)si urinare-
necesita ATB-pie prompta
-Complicatii secundare corticoterapiei(osteoporoza,osteonecroza
aseptica,HTA,DZ,cataracta,psihoza,infectii) –tratament adjuvant
-Comorbiditati cardiovasculare (ATS accelerata) –statine,antiagregant plachetar,IECA
-Boala cronica de rinichi stadiul terminal --transplant/dializa si tratamentul complicatiilor acesteia.
-Neoplazii —carcinom de vezica urinara(sec admin de Ciclofosfamida),neo col uterin(displazie de cervix in
infectia HPV-screening anual cu efectuarea testului Papanicolau)
Monitorizarea bolii –SLEDAI score=14 pct(activitate inalta a bolii)
Scor SLEDAI
Permite evaluarea activitatii LES in ultimile 10 zile
Interpretare - Forme de boala:
LES inactiv SLEDAI=0
LES usor activ = 1-5
LES moderat activ = 6-10
LES cu activitate inalta =11-19
LES f. activ >20
Recadere = crestere scor cu mai mult de 3 pct
Remisiunea = SLEDAI 0
Imbunatatire = reducerea scor cu peste 3 pcte
Boala persistent activa = schimbare scor cu 1-3pct
Particularitatea cazului
Asocierea LES cu sdr antifosfolipidic la o pacienta tanara cu evolutie ondulanta a bolii
Prezenta tuturor Ac(anti cardiolipina,anti beta 2 glicoproteina 1,anticoagulant lupic) asociati cu
LES
Prognostic
nefavorabil
Afectari
trombotice
Lupus
neurologic
Vasculita
Nefrita
lupica
Studii The American Journal of Medicine
Comparison of the primary and secondary antiphospholipid syndrome: A
European multicenter study of 114 patients ☆
Author links open the overlay panel. Numbers correspond to the affiliation
list which can be exposed by using the show more link.Joao L. Vianna,
M.D. a, Munther A. Khamashta, M.D., Ph.D. ∗, a, Jose Ordi-Ros, M.D.,
Ph.D. b, Jose Font, M.D., Ph.D. c, Ricard Cervera, M.D., Ph.D. c, Alfonso
Lopez-Soto, M.D., Ph.D. c, Carlos Tolosa, M.D., Ph.D. b, Juliane Franz,
M.D. c, Alberto Selva, M.D., Ph.D. b, Miguel Ingelmo, M.D., Ph.D. c, Miguel
Vilardell, M.D., Ph.D. b, Graham R.V. Hughes, M.D., F.R.C.P. To determine whether the features of the antiphospholipid syndrome
(APS) are in any way influenced by the presence or absence of systemic
lupus erythematosus (SLE). We followed up patients with ‘primary’ APS
(PAPS) and APS secondary to SLE (APS plus SLE) with the objective of
comparing laboratory and clinical events and of determining whether
patients with PAPS would have evolution to SLE.
PATIENTS AND METHODS: A total of 114 patients from 3 European referral
centers were included in this study. Fifty-six had APS plus SLE and 58
had PAPS. Laboratory and clinical data were collected during an
average 2-year period.
RESULTS: Patients with PAPS and patients with APS plus SLE had similar
clinical and laboratory profiles, with the exceptions of autoimmune
hemolytic anemia, endocardial valve disease, neutropenia, and low C4
levels, all of which occurred more frequently in patients with APS plus
SLE (p values: <0.05, <0.005, <0.01, and <0.001, respectively). On
follow-up, 10 thrombotic episodes occurred in 10 patients, 8 of whom
were receiving anticoagulant therapy. No patient with PAPS had either
anti-DNA or anti-extractable nuclear antigen antibodies, and these
patients had a significantly lower prevalence of antinuclear antibodies
(41%) than patients with APS plus SLE (89%).
CONCLUSIONS: Patients with APS plus SLE and PAPS have similar
clinical profiles, although heart valve disease, hemolytic anemia, low C4
levels, and neutropenia seem to be more common in patients with APS
plus SLE. Patients with APS may develop further thrombotic events
despite anticoagulation therapy
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