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

http://www.rheumtutor.com/wp-content/uploads/2012/12/SLICC-Criteria.jpg

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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