![Page 1: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/1.jpg)
Caz clinic
Student: Andreea Bara, gr 58Asistent: Dr Andreea Borangiu
![Page 2: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/2.jpg)
LC (F), 33 ani
MI Reevaluare clinico-biologica Tuse seaca Stare febrila la domiciliu (38˚C) Fatigabilitate
![Page 3: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/3.jpg)
APP
Scleroza sistemica(1998)
Chist ovarian stang operat (2007)
Proteza de sold ptr necroza cap femural (oct 2008)
TB latenta (2009)
![Page 4: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/4.jpg)
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
![Page 5: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/5.jpg)
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
![Page 6: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/6.jpg)
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;
![Page 7: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/7.jpg)
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%
![Page 8: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/8.jpg)
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
![Page 9: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/9.jpg)
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
![Page 10: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/10.jpg)
![Page 11: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/11.jpg)
![Page 12: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/12.jpg)
![Page 13: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/13.jpg)
![Page 14: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/14.jpg)
![Page 15: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/15.jpg)
![Page 16: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/16.jpg)
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
![Page 17: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/17.jpg)
Examen paraclinic
![Page 18: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/18.jpg)
Examen paraclinic
![Page 19: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/19.jpg)
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
![Page 20: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/20.jpg)
![Page 21: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/21.jpg)
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
![Page 22: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/22.jpg)
Evolutia DLCO 2006-2011
![Page 23: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/23.jpg)
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
![Page 24: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/24.jpg)
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
![Page 25: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/25.jpg)
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)
![Page 26: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/26.jpg)
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
![Page 27: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/27.jpg)
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
![Page 28: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/28.jpg)
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
![Page 29: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/29.jpg)
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
![Page 30: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/30.jpg)
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
![Page 31: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/31.jpg)
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
![Page 32: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/32.jpg)
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)
![Page 33: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/33.jpg)
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
![Page 34: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/34.jpg)
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
![Page 35: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/35.jpg)
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
![Page 36: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/36.jpg)
Recomandari
INR la 2 sapt, mentinere 2-3
Interzis injectii im, extractii dentare, interventii chirurgicale
Revine la control 7 martie
Quantiferon, TIBC, CTLF
![Page 37: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/37.jpg)
Complicatii ale bolii
Scleroza cutanata, Ulceratii cutanate, Acroosteoliza
Alveolita fibrozanta, HTP BRGE Sindrom Sicca Pericardita, Pleurezie Fatigabilitate, pierdere ponderala, depresie
![Page 38: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/38.jpg)
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
![Page 39: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/39.jpg)
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
![Page 40: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/40.jpg)
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
![Page 41: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/41.jpg)
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
![Page 42: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/42.jpg)
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
![Page 43: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/43.jpg)
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
![Page 44: Caz Clinic Sclerodermie 2011 Seria Vii Umf Carol Davila1](https://reader035.vdocuments.pub/reader035/viewer/2022082202/55cf918c550346f57b8e5767/html5/thumbnails/44.jpg)