Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1

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Caz clinic Student: Andreea Bara, g Asistent: Dr Andreea Bor

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Transcript of Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1

Page 1: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1

Caz clinic

Student: Andreea Bara, gr 58Asistent: Dr Andreea Borangiu

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LC (F), 33 ani

MI Reevaluare clinico-biologica Tuse seaca Stare febrila la domiciliu (38˚C) Fatigabilitate

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APP

Scleroza sistemica(1998)

Chist ovarian stang operat (2007)

Proteza de sold ptr necroza cap femural (oct 2008)

TB latenta (2009)

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Istoricul bolii

1998: Debut-sindrom Raynaud, poliartralgii difuze,

subfebrilitate, ulceratii digitale, calcificari subcutanate

Punctie biopsie renala: GN membrano-proliferativa

Azatioprina (100mg/zi), Colchicina (0.5g/zi), Prednison(60mg/zi), Antiagregante, Vasodilatatoare

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Istoricul bolii

1999: Sindrom inflamator moderat, Sindrom retentie azotata

Ac antiSCL70 pozitivi

Ciclofosfamida puls terapie (0,6g/m2/luna) 6 luni Prednison (7.5mg/zi)

Metotrexat (max 7.5mg/sapt) pana in 2006, cu ameliorarea manifestarilor cutanate

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2006-Sp ”Sf Maria”

Ac anticentromer negativ, Ac anti SCL70: 121,7U/L

Capilaroscopie: hemoragii subunghiale, megacapilare, fara zone avasculare

Disfunctie restrictiva usoara, DLCO:44%, PAPs:29mmHg

Ciclofosfamida puls terapie (0,6g/m2/luna) 6 luni;

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Istoricul bolii

2008:

Ulceratii digitale multiple Hipotensiune CT: honey combing, bazal bilateral- Fibroza pulmonara

Ciclofosfamida puls terapie(0.6g/m2/luna) 12 luni

DLCO: 54%

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Istoricul bolii

2009:Februarie: Necroza ischemica deget II MD brusc instalata ACO sub control INR, Sildenafil (60mg/zi)

IDR la PPD 20 mm; Infectie TB latenta; Izoniazida 9 luni

Martie: Epistaxis-uri repetate, HTA (160/90mmHg) Creatinina 1,5 mg/dL Se opreste Sildenafil, se initiaza Enap (5mg/zi) Ac antiSCL70: 143U/mL

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Istoricul bolii

Iulie 2009 Revarsat pleural, pericardic persistent- Serozita PAPm: 32mmHg DLCO: 47% Sildenafil (60mg/zi)

2010 DLCO: 48% Episod digestiv: epigastralgii, scaune diareice, probe malabsorbtie

negative Omeprazol, Trimebutina (Debridad), Eritromicina, Smecta

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Scor Rodnan

0=fara modificari 1=ingrosare usoara 2=ingrosare moderata 3=ingrosare severa

17 regiuni Valori :20, 51 Scor pacienta:

2

2

0

1 1

2 222 2

3 30 0

3 3

0 0

2 2

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Examen paraclinic

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Examen paraclinic

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Examen paraclinic

Rx toraco-pulmonaraSechele fibronodulare hilar si parahilar stang, Desen peribronhovascular accentuat perihilarsi bazal bilateral, predominant stang

Rx sinusuri: Voalare sinusuri maxilare (Sinuzita)Azitromicina 500mg/zi 3 zile

Probe functionale respiratoriiDisfunctie ventilatorie restrictiva moderata (CV 64%)DLCO moderat scazut (47%)

Test Quantiferon in lucru

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Examen paraclinic

Rx toraco-pulmonaraSechele fibronodulare hilar si parahilar stang, Desen peribronhovascular accentuat perihilarsi bazal bilateral, predominant stang

Rx sinusuri: Voalare sinusuri maxilare (Sinuzita)Azitromicina 500mg/zi 3 zile

Probe functionale respiratoriiDisfunctie ventilatorie restrictiva moderata (CV 64%)DLCO moderat scazut (47%)

Test Quantiferon in lucru

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Evolutia DLCO 2006-2011

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Criterii ACR ptr diag SC

Criteriu majorScleredem proximal de MCF sau MTF

Criteriu minorSclerodactilieCicatrici stelate pulpa degeteFibroza pulmonara bibazala

Diagnostic= criteriu major + 2 criterii minore

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Diagnostic pozitiv

Scleroza sistemica cu afectare cutanata difuza, pulmonara (alveolita fibrozanta, in urmarire pentru HTP), renala si serozitica

HTA secundara afectarii renale

Sindrom inflamator, in urmarire pentru o reactivare tuberculoasa

Sinuzita

Osteoporoza cortizonica

Proteza sold drept ONACF

Chist ovarian drept operat

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Diagnostic diferentialScleredem

Pro: Ingrosarea dermului

Contra: Respecta degetele mainii si piciorului Nu se insoteste de anomalii serologice (Ac anti SCL

70 pozitivi) Nu afectari organe interne (pulmonara, renala, tract

digestiv, seroase)

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Diagnostic diferential: PR

Pro: Varsta tanara a pacientei Prezenta poliatralgii difuze Prezenta sindroamelor anemic si inflamator Afectarea pulmonara si renala

Contra: Caracterul deformant si distructiv al leziunilor

articulare in cazul PR, limitarea miscarii prin fibroza tegumentelor si modificari tip acroosteoliza in SC

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Diagnostic diferential: LES

Pro: Sindrom inflamator si punctie biopsie renala:

GN membrano-proliferativa (1998)

Contra: Imobilizarea in flexie a articulatiilor mainii,

spre deosebire de artrita Jacqoud din LES

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Diagnostic diferential:Overlap Sindrom

Pro: Elementele pro prezente la oricare din

diagnosticele diferentiale cu PR, LES

Contra: Elementele contra corespunzatoare prezente

la diagnosticele diferentiale cu PR, LES

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Diagnostic diferential: BMTC

Pro: Unul sau mai multe elemente prezente la

argumentele pro prezente la diagnosticele diferentiale cu PR, LES

Contra: Ac anti U1RNP negativi

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Diagnostic diferentialProces infectios

Pro: Stare generala alterata, febra, tuse Rx sinusuri (voalare), !!! Posibilitatea reactivarii TB latente; Quantiferon in

lucru

Contra: Hemocultura, urocultura, ex sputa negative Prognosticul prost al pacientilor cu forma cutanata

difuza, Ischemia deget III MS-sdr inflamator Quantiferon in lucru

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Tratament igieno-dietetic

Evitare frig, manusi calduroase, nu fumat

Mentinerea curata si uscata a leziunilor tegumentare

Consum lichide cel putin 2L/zi

Evitarea consumului suplimentar vit C

Dormit pe perna inalta pentru a evita refluxul gastro-esofagian

Terapie fizica si ocupationala

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Tratament patogenic

Ameliorarea inflamatiei: Prednison (7,5mg/zi)

Ameliorarea fibrozei pulmonare: Piascledin (300mg/zi)

Contracararea lezarii endotelialeAspenter (75mg/zi), Sintrom (sub control INR 2-3)

Vasodilatatoare si tratamentul HTPSildenafil (60mg/zi), Diltiazem (90mg/zi),Pentoxifilin (100mg PEV lent 500mL SF)

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Tratament patogenic

Tratamenetul HTA secundareEnap (5mg/zi)

Corectarea anemieiVenofer fl 100mg PEV 500mL SF, dupa 3 zile dezvolta reactie adversa cutanata Pacienta trece pe Tardyferon 2cp/zi, tratament pe care il va urma 3 luni

Tratamentul sinuziteiAzitromicina cp 500mgx3/zi, 3 zile urmat de Claritromicina cp 500mgx2/zi 5 zile,Paracetamol sinus cp 500mgx3/zi saptRhinocort 2 pufuri fiecare nara x2/zi 2 sapt, urmat 1 puf fiecare nara x2/zi pana

la 3 luni

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Tratament simptomatic

Tratamentul afectarii digestive (simptomatologie)Omeprazol 20mg/zi,Trimebutina(Debridad) 200mg/zi, Metoclopramid 15mg/zi

Tratamentul osteoporozeiVit D3 (Vigantoletten)1000ui/zi,Acidum risedronicum (Actonel) 35mg/sapt

Prevenirea hipokaliemiei Aspacardin 3 cp/zi

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Evolutie parametri paraclinici de laborator de-a lungul internarii

CRP: 31.38mg/dL 14.33mg/dL

INR: 4.59 2.64

Creatinina: 1.3mg/dL 1mg/dL

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Recomandari

INR la 2 sapt, mentinere 2-3

Interzis injectii im, extractii dentare, interventii chirurgicale

Revine la control 7 martie

Quantiferon, TIBC, CTLF

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Complicatii ale bolii

Scleroza cutanata, Ulceratii cutanate, Acroosteoliza

Alveolita fibrozanta, HTP BRGE Sindrom Sicca Pericardita, Pleurezie Fatigabilitate, pierdere ponderala, depresie

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Complicatii ale tratamentului

Ateromatoza accentuata Osteoporoza cortizonica Hipokaliemie Susceptibilitate crescuta la infectii Anemie, leucopenie Cistita hemoragica Miastenia gravis secundara CFS Insuficienta ovariana hTA Risc crescut malignizari

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Prognostic

Forma difuza-mai prost decat CREST

Viteza de extensie a leziunilor cutanate

Sc afectare cutanata limitata: 71% supravietuire la 10 ani Sc afectare cutanata difuza: 21% supravietuire la 10 ani HTP: factor major prognostic

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Particularitatea cazului

Pacienta prezinta stare generala alterata, stare febrila la domiciliu si tuse seaca.

Suspecta de reactivarea unei tuberculoze latente, Quantiferon in asteptare

In functie de rezultat, Temporizare cura ciclofosfamida si tratament

antituberculos Administrare cura ciclofosfamida

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In loc de sfarsit

J Reumatology sept 2010APRIL (A proliferation inducing ligand, TNF, Mo)20 pacienti SSc, 14 lot control

Afectare cutanata difuza, fibroza pulmonara, vasculopatie periferica, afectare capilara accentuata, prezenta Ac anti topoizomeraza I

Ac anti SCL70 sunt singurul factor predictiv independent ptr sinteza crescuta de APRIL de catre mononuclearele circulante.

Posibilitate terapeutica

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Halofuginone

Alkaloid extras din Dichroa febrifuga Apartine clasei Orphan drugs Inhiba TH17, fara a avea efect pe Treg Inhiba sinteza colagenului I (migrare si proliferare celulara,

angiogeneza) Inhiba sinteza colagenazei

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Bibliografie

Esentialul in Reumatologie, Coord Prof Dr Ruxandra Ionescu, Catedra de Medicina Interna si Reumatologie, UMF “Carol Davila” 2006, Ed Medicala Almateea

http://www.ncbi.nlm.nih.gov/pubmed/20810514

http://emedicine.medscape.com/article/331864-overview

http://www.sclerodermatt.org/community/212

http://archives.focus.hms.harvard.edu/2009/071009/discoveries_whitman.shtml

MemoMed 2010, Editia 16, Editura Minesan, Editura Universitara, Bucuresti

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