colagenoze curs
TRANSCRIPT
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COLAGENOZE CU DETERMINARE PULMONARA
COLAGENOZE Klemperer 1941
COLAGEN = tesut conjunctiv = tesut conectiv
a) substanta fundamentala b) celule- lichid interstitial - fibrocite - polimorfo
nucleare- glicoproteine - fibroblasti - eozinofile- mucopolizaharide - cel. grase - monocite- proteine necolagene - mastocite - limfocite
- histocite
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c) fibre- colagen- reticulina FIBROBLASTE - fibre de reticulina + ciment - elastice
FIBRE DE COLAGEN
COLAGENOZE CU DETERMINARE PULMONARA
-continuare-
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LEZIUNI PRINCIPALE
1.Necroza fibrelor (necroza fibrinoida)2. Scleroza de colagen
Bolile tesutului conectiv au trasatura comuna Necroza fibrinoida + dezordini imunologice
Boli autoimune (VASCULARITA AUTOIMUNA)
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PREDILECTIA UNOR COLAGENOZE PENTRU DIVERSE ORGANE SAU SISTEME (dupa CORTEZ SI PIMENTAL)
Poliartritanodoasa
Boala lupica Sclerodermiedifuza
Polimiozita Poliartritareumatoida
Vase ++++ ++ + + +Piele + ++ +++ ++ +
Muschi ++ + ++ +++ ++
Articulatii + ++ + + +++
Nervi ++ +
Seroase +++ + + +
Inima +++ +++ ++ +
Rinichi +++ +++ + +
Tub digestiv +++ + ++
Plaman + + +++ + +
SNC + ++
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LUPUSUL ERITEMATOS DISEMINAT SAU SISTEMIC
(LES)
DEFINITIE: Boala determinata dedegenerescenta fibrinoida a tes.conjunctiv care afecteaza:
- pielea, membranele, articulatiile, pleura, pericardul, inima, rinichiul
- ganglionii, ficatul splina, sistemul hemato poetic si plamanul
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LUPUSUL ERITEMATOS DISEMINAT SAU SISTEMIC
(LES)-continuare-
EPIDEMIOLOGIE
- afecteaza in special femeile intre 15-45 ani cu o incidenta cuprinsa intre 1/2000
si 1/250 (SUA, Indiile de Vest, Africa de Sud)- M/F = 1/8
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ETIOPATOGENIE
= CAUZA NECUNOSCUTA =
Anomalia principala:- capacitatea de producere AUTOANTICORPI impotriva - ADN - limfocite- constituenti nucleari - fact. coagularii- ARN - neuroni- citoplasma - cord- eritrocite - colagen- trombocite
+
Fagocit material Celula LE
nuclear (Hargraves) alterat
ADN + Ac. Anti ADN Complexe imune+ Complement
Depunere la nivelul rinichiului si pielii
L.E.S = BOALA COMPLEXELOR IMUNE
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FACTORI PREDISPOZANTI(PATOGENICI)
• Predispozitie genetica - pozitia cromozomului X
• Stimuli de mediu - virusuri- bacterii- U.V. (raze)- medicamente- procainamida- hidralazina- clorpromazina- izoniazida- hidantoina
• Factori hormonali - preponderenta la femei• raspuns imun anormal - scaderea nr. limf. T
- cresterea activitatii limf. B
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FACTORI PREDISPOZANTI(PATOGENICI)
-continuare-
= Sinteza de anticorpi impotriva propriilor nucleoproteineEste perturbata cea mai importanta functie a sistemului imunitar:
DISCRIMINAREA DINTRE “SELF” SI “NONSELF”Limf. B ----> exces de Ig.G = ac. fata de haptene, elemente proteice
tisulare, celule proprii, limfocite.Limf. T----->redus numeric, defect in geneza T supresor
L.E.S.= Boala cu componenta autoimuna
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MANIFESTARI CLINICE (1)
- 85 % din C apare la femei intre 15-45 ani- 10 % din C dupa medicamente• Caractere : 1. cronicitate
2. Atingere pluriviscerala• Elemente de suspiciune
- Sindrom febril- Sindrom reumatoid- glomenilonefrita- pleurezie - poliserozita- Sindrom cutanat- purpura- Sindrom Raynaud - flebite migratorii- Sindrom miastenic
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MANIFESTARI CLINICE (1)-continuare-
• Modalitati de debut- acut- insidios - (ani de zile)- asimptomatic (numai serologie +)- invazia unor organe- multisistemica - letala
• LEZIUNI CUTANATE- frecvente - aspectul clinic de rash “in fluture” este rar.- eritem facial- leziuni discoide- eritem periunghialFOTOSENSIBILITATE = FENOMEN patognomonic 1/3 din C- sindrom Raynand 15-20% din C- alopecie frontalaulceratii bucale si sept nazal
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MANIFESTARI CLINICE(2)
• ARTRALGII =cele mai frecvente 90% din C - rare eroziuni sau deformari
• NEFRITA = peste 50% din C - cauza majora de deces
• NEUROLOGIC - 50% din C - dezordini n. cranieni - coree - manifestari neurologice de focar - psihoza
• CARDIOVASCULARE - pericardite 10-20% din C - endocardita verucoasa atipica (Libman - Saks)
• LIMFADENOPATII - in formele active• SPLENOMEGALIE - 10% din C
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MANIFESTARI CLINICE (3)
• PULMONAR1- Pneumopatii infectioase2- Pleurezii: 1/3 din C.
+ pachipleurite- uni sau bilaterale- minime sau masive- exudat cu Nr. de limfocite- Complement- celule LE prezente- glucoza norfmal
3. - Pneumonia lupica = RARA DAR SEVERA- tahipnee - tahicardie- dispnee - absenta germenilor- cianoza- febra
Radiologic: Condensare difuza de aspect infiltrativ predominant bazal
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MANIFESTARI CLINICE (3)-continuare-
4. - Pneumopatie interstitiala difuza 3% din C.a) aspect radiologic de fibroza densa cu desen reticulomicronodular (noduli submiliari)b) absenta semnelor stetacustice sau discretec) disfunctie ventilatorie restrictivad) Biopsie = depozite de complexe imunee) Corticoterfapie raspuns favorabil
5. - Pneumonia atelectaticaa) infiltratie pulmonara cu obliterarea spatiului alveolarb) topografie bazalac) Nu raspunde la antibioticed) caracter recidivante) raspuns pozitiv la corticoterapie
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MANIFESTARI CLINICE(4)
6) Hipertensiune in artera pulmonara7)Hemoragii intrapulmonare uni sau bilaterale
DIAGNOSTIC DE LABORATOR
ELEMENTE CARACTERISTICE DAR NESPECIFICE- anemie feripriva autoimuna- VSH- leucopenie si trombocitopenie- hematurie - proteinurie
ELEMENTE SPECIFICE- Ac. AN - pozitiv in 90% din C- celule LE- factorul LE Haserick- aspect anatomo patologic
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TRATAMENT
- FARA TRATAMENT CORTIZONIC - SUPRAVIETUIRE PINA LA 2 ANI- CU TRATAMENT 10-15 ANI
1. ANTIMALARICE DE SINTEZA- indicate in asociere cu corticoterapia permit uneori reducerea
dozei de glucocorticoizi- in fazele de acalmie pot inlocui cortizonicele
Medicamente doze:- Hidroxiclorochin 200-400 mg/ 24 h (tb. 200 mg)- Nivaquin 4 mg/ Kg c/ zi (100-300 mg)- durata 5-6 luni
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TRATAMENT-continuare-
2.GLUCOCORTICOIZI- echivalent in prednison peste 1mg /Kg c/ zi in cazul atingerilor nervoase si
renale- determinarile pleuropulmonare 40-50 mg/ zi
DOZA DE INTRETINERE dupa 3-4 luni . 10-15-20 mg /zi.
3.CITOSTATICE- imunosupresive- ciclophosphamida (fiole 200 mg, tb.50 mg) alternant cu corticoterapie sau
asociat - 400 mg la 2 zile apoi 100 mg la 2 zile.- azathioprina (tb. 50 mg. , 300mg /zi fractionat)- metotrexat 40 mg. Doza unica saptamanala
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SCLERODERMIA(SCLEROZA SISTEMICA PROGRESIVA)
• DEFINITIE - dezordine generalizata a tesutului conj. - cauza necunoscuta .- umflarea si proliferarea firelor de colagen . condensare in masa comuna- stari inflamatoriiPielea - ingrosata Precedate de
- inflexibila sindr. - aderenta la tes. subcutanat. Raynaud
Localizari: articulatii, muschi,tub digestiv, plamani, rinichi, inima, etc.Diagnostic facil Sindrom Raynaud
Manifestari cutanate50 % din C . AAN pozitiv. Factor reumatoid poz 33% din CComplement normal
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MANIFESTARI CLINICE
• DEBUT Disconfort g.intestinal, astenie, poliartrita, mialgii Sindrom Raynaud + modificari cutanate ale extremitatilor
• LEZIUNILE CUTANATE - Localizare - pe arii mici- generalizate pe trunchi si membre - fata, maini, picioare- Evolutie- modificari atrofice cu ulceratii- brazdarea pielii in jurul gurii- telangietazii, pigmentari, - calcificari in partile moi
• LEZIUNI CARDICE - insuficienta cardiaca - cardiomegalie
- tulb. de conducere-aritmie
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MANIFESTARI CLINICE-continuare-
• GASTROINTESTINAL - esofag in bagheta de sticla - disfagie, malnutritie
• RENAL - uremie cu HT maligna
• SINDROM CREST• -calcinoza, s.Raynaud, disfunctie esofagiana, scleroza cut.
discreta, telangiectazii
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MANIFESTARI PULMONARE
• CLINIC:- Dispnee progresiva- Rar dureri toracice - hemoptizii
• RADIOLOGIG:- Aspecte normale- Fagure de miere - predominant bazal- Rar aspect granulic- calcificari si pleurezii
• FUNCTIONAL:
-Sindrom de rigiditate pulmonara
• PROGNOSTIC:Afectare cord, rinichi, plamani, deces in
cativa aniCORTICOTERAPIA -ameliorare
• TRATAMENT:CORTICOSTEROIZI-doze si durata ca la
LESD penicilamina-stabilizare a colagenuluiFactorul XIII inpiedica polimerizarea
colagenuluiColcichina (ALARCON SEGOVIA)- blocare transf. procolagen colagenCaptopril - antihipertensiv
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ARTRITA REUMATOIDA(POLIARTRITA REUMATOIDA)
- Boala de etiologie necunoscuta- Raportul M/F 1/3- Apare intre 40-50 ani mai frecvent
IMUNOLOGIC - AcAN 10-30% din cazuri- Fact. Reumatoid 70-80 % din cazuri - nespecific- ( si
in LES, sclerodermie polimiosita, FID, hepatita cr., etc)
ANATOMO-PATOLOGIC - Proces inflamator al suprafetelor epiteliale in special seroase.
- focare de degenerare, proliferarea tesutului conj.
- hipertrofie tes.limfoid, vasculita
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ARTRITA REUMATOIDA(POLIARTRITA REUMATOIDA)
-continuare-
NODUL REUMATOID - Procese de degenerare cu necroza centrala- proliferare de celule mezemchimale in
palisada - inflamatie cu tesut fibros si celule
inflamatorii
-Necroza fibrinoida la vasele mici
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MANIFESTARI CLINICE
• DEBUT: - Astenie - Febra - Scadere ponderala - transpiratii - parestezii (maini si picioare) - POLIARTLARGII
- EVOLUTIE IN PUSEE -
• FAZA DUREROASA: TUMEFIERE DEFORMARE
”CASEXIA REUMATICA”
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MANIFESTARI CLINICE-continuare-
• MANIFESTARI EXTRAARTICULARE:- Noduli subcutanati - limfadenopatii- Leziuni de vasculita - episclerite, sclerite- Infarcte digitale - pericardite- Arterite viscerale necrozante - neuropatie periferica- Ulceratii cutanate - Sjögren Sindrom
Rinichiul este crutat de artrita reumatoida !
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MANIFESTARI PULMONARE
• PLEUREZIA REUMATOIDA
- 2-5% din cazuri- poate apare inaintea afectarii articulare - de obicei tardiv- obisnuit bilaterale - abundente
- PROTEINE - in jur de 50 gr 0
~ lichid lactescent (Colesterol )- hipergamaglob.- glucoza sub 0,30 gr 0
- LDH
- fact reumatoidBiopsia pleurala nodul reumatoidCorticoterapie resorbtie
• LEZIUNI PULMONARE
- INFILTRATIVE- NODULARE - F.I.D. cea mai frecventa- Apar la barbatii in jur de 60 ani
CLINIC: tuse, durere toracica, raluri crepitante, dispnee
RADIOLOGIC: leziuni - nodulare- infiltrative- reticulonodulare
- Mai frecvente la barbat
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MANIFESTARI PULMONARE (2)
• Hipertensiune pulmonara + Sind Reynaud + FR -POZITIV
• Boala obstructiva cu evolutie letala rapida• Bronsectazii si bronsita cr. Frecvent
TRATAMENT: similar cu L.E.S.
SINDROMUL CAPLAN
- varianta a plamanului reumatoid la bolnavii cu P.R. expusi la praf (minerii din bazinele carbonifere).
RADIOLOGIC:
- opacitati multiple cu 0,5-5 cm, rotunde, bine conturate, bilateral periferic. - imagini de silicoza
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POLIARTERITA NODOASA (P.N.)(Periarterita Nodoasa)
DEFINITIE: Boala rara de cauza necunoscuta, caracterizata prin: - focare de arterita necrozanta
ale arterelor mici si mijlocii.
POLIMORFISM CLINIC MARCAT
- 1/3 din cazuri - determinare pulmonara IMUNOLOGIC
- Hipersensibilitate de tip III: - fata de
medicamente - fata de alti
alergeni
NU S-A PUTUT STABILI CAUZALITATE CERTA
ANATOMO PATOLOGIC:
- Afectarea A. mici focare de tromboza- ficat - rinichi- splina - miocard
infarct de organproliferare fibroblastica- anevrisme
PULMONAR: - necroza cu cazeificare- infarct- bronsectazii
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MANIFESTARI CLINICE
- Incidenta crescuta peste 60 ani- predominanta la barbati
DEBUT: Simptome generale: - febra ARTICULATII:febra - astenie reumatoida - pnderala artrita
RENAL: Glomerulo nefrita acuta CORD: - infarct H.T.A. sec. - insuf. coronariana- vasculita vezicala - insuf. cardiaca - disurie - pericardita - hematurie
.
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MANIFESTARI CLINICE-continuare-
DIGESTIV: - sangerari CUTANAT: - eruptii - crampe abdominale - noduli subcutanati - perforatii - glosite ulcerative - steatoree
SISTEMUL NERVOS: afecteaza nervii cranieni
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MANIFESTARI PULMONARE
ASTM BRONSIC PNEUMONII PLEUREZII- aparitie tardiva - tuse - predominenta- grav - hemoptizii limfocitelor- dispnee continua - dureri toracice- febril - unele: INFARCT PULMONAR
- cu infiltrate pulmonare - eozinofile in sputa- eozinofile marcata- granuloame alergicein plamani
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MANIFESTARI PULMONARE-continuare-
Radiologic: Aspect pneumonic, aspect granulic, caverne (tbc?)
DIAGNOSTIC: Leucocitoza, Eozinofile + lez. Pulmonare, Eozinofile + astm bronsic, VSH mult marit. Fact. A.N. si Cel. L.E. ~prezente BIOPSIA musculara - aspecte tipice
TRATAMENT: CORTIZONICE
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GRANULOMATOZA WEGENER
DEFINITIE : - GRANULOM NECROTIC AL CAILOR RESPIRATORII SUPERIOARE SI/ SAU INF. - LEZIUNI DISEMINATE ARTERIAlE SI VENOASE. - GLOMERULO-NEFRITA ACUTA-SEVERA
- VARIANTA A PN ?
ANATOMOPATOLOGIC: - angeita necrozanta- formatiuni granulomatoase- in rinichi - glomerulo nefrita focala, necroza fibrinoida- infarctizari
CLINIC: DEBUT Rinoree si obstructie nazala 2/3 din C sinuzita, epistaxis, ulceratii gingivale
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GRANULOMATOZA WEGENER-continuare-
• PULMONAR: - tuse, hemoptizii rebele, pleurezii, febra de tip septic,- infiltrate uneori cu tendinta la necrozare sau noduli cu tendinta la ulcerare.
CAND EZINOFILA SANG. este prezenta
Granulomatoza alergica• RENAL GLOMERULONEFRITA ACUTA - SEVERA
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TRATAMENT
• Fara - deces pana la 5 luni rar 5 ani– Corticoizi - prelungiri pana la 13-15 ani
IMUNOSUPRESOARE:Azathioprina 150-200 mg/ziClorambucil 0,2 mg/kc/ziCiclophosphamida 600 mg/sapt. (200 mgx3)Metotrexat initial 60 mg/sapt. i.v. apoi 15-25
mgp.o./sapt
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SINDROMUL SJOGREN
DEFINITIE: - Afectiune cronica a glandelor lacrimale si salivare caracterizata prin keratoconjunctivita si
xerostomie (gura uscata)
Se asociaza frecvent cu: P.R, LES, sclerodermia sau polimiozita.Factor reumatid prezent 95-100 % din C.Ac. A.N. prezenti 70 % din C.PULMONAR: - F.I.D. (pneumonie limfocitara)
- pleurezii
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DERMATOMIOZITA SI POLIMIOZITA
DEFINITIE: sindrom caracterizat prin:
- astenie- contracturi musculare- atrofia tesutului conjunctiv
= rezultatul unui proces inflamator
Localizari: piele, muschi striati si tesut conjunctiv
CLINIC: Astenie musculara progresiva cu atrofie progresiva
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CLINIC• CUTANAT - eruptie rosie-liliachie + edem (nas, obraz,unghii)
- PATOGNOMONIC - edem al pleoapei superioare - liliachiu
- eruptii buloase, hiperkeratoze, induratii sclerodermice
• ARTICULAR - artrita acuta tranzitorie- artrita reumatoida
• PULMONAR - infiltrate in lobii inferiori- pneumonii de aspiratie