aspecte radiologice in sarcoidoza mediastino pulmonara
TRANSCRIPT
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Aspecte radiologice in sarcoidoza mediastino-
pulmonara
Moldoveanu Elena Cristina Moldoveanu Bogdan Constantin
Spitalul Clinic de Pneumologie,Iasi
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SARCOIDOZABoala multisistemica de etiologie necunoscuta care afecteaza in special persoanele tinere si de varsta medie
60-70%din pacienti au modificari radiologice pe radiografia toracica cel mai frecvent adenopatii hilare bilaterale ( +/-leziuni in parenchim de tip micronoduli si infiltrate ); adesea sunt asociate leziuni oculare si cutanate.
Morbiditatea si mortalitatea cea mai mare este rezultatul afectarii pulmonare.
Diagnosticul este stabilit pe baza constatarilor clinico radiologice fiind sustinut de prezenta histologica a granulomului de celule epitelioide fara cazeificare.
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Aspecte radiologice
Tipic
1. Limfadenopatie simetrica bilaterala hilara si mediastinala.
2. Cu sau fara modificari parenchimatoase (noduli, infiltrate, fibroza).
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In functie de anomaliile radiografiei toracice
Stadiul 0-radiografie toracica normala
Stadiul 1-adenopatie hilara/mediastinala
Stadiul 2-adenopatie plus infiltrate parenchimatoase nonfibrotice
Stadiul 3-numai infiltrate parenchimatoase nonfibrotice (fara adenopatie)
Stadiul 4-semne radiologice de fibroza/modificari chistice
•Alte clasificari folosesc 3 stadii cu modificari infiltrative non fibrotice si fibrotice grupate in stadiul III (Hansell, 2005)
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Stadiul 1
Frecvent : adenopatii hilar, paratraheal, subcarinar, in fereastra aortopulmonara (vizibile radiologic)
Mai rar: mediastinal anterior, mediastinal posterior, ganglioni mamari interni, retrocrurali, paracardiaci, axilari(vizibili prin computer-tomografie)
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Radiografia PA demonstreaza limfadenopatie hilara si paratraheala bilaterala simetrica
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Caracteristici adenopatii
Adenomegalie moderata sau voluminoasa- asimptomatica sau compresie pe cai aeriene si esofag (disfagie)
Contur ganglionar net/sau tendinta la confluare in mase ganglionare
Pot prezenta calcificari (rar comparativ cu TBC, silicoza, infectii fungice)
Pot da mediastinita cronica cu sindrom de VCS
Pot genera obstructie vasculara
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Simetria: criteriu important in DD (adenopatii asimetrice metastatice, limfoame, TBC)
Bilateralitatea :rar, nu exclus adenopatii unilaterale dar impun DD cu malignitati,TBC, infectii fungice)
Asocierea adenopatii si infiltrate(cand apar modificari in parenchim adenopatiile se micsoreaza/DD cu boli maligne sau limfoame unde modificarile pulmonare se coreleaza cu adenopatiile
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Evolutia adenopatiilor
Rezolutie completa fara tratament( poate sa evolueze spontan favorabil)
Adenopatii si modificari parenchimatoase (de obicei adenopatiile se micsoreaza cand apar leziuni in parenchim)
Aspecte rezolutive dupa corticoterapie
Episoade de recurenta
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Aspecte radiologice in stadiul I
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Stadiul I de sarcoidoza arata distributia caracteristica a adenopatiilor bilateral paratraheal, hilar, subcarinar,
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Adenopatie hilara bilaterala
Adenopatii hilare si paratraheale
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Aspecte CT in stadiul I
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Caz 1Adenopatii localizate paratraheal drept si stang,asimetrice mai voluminoase pe dreapta
Adenopatii paratraheal drept superior,stang superior si in fereastra aortopulmonara
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Adenopatii hilare, interlobare subcarinare si paraesofagiene
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Caz 2
Adenopatii bilateral mediastinal
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Caz 3
Adenopatii bilateral mediastinal
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Caz 4
Adenopatii retroperitoneale, frenic inferior si paracardiac
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Caz 4
Adenopatii bilateral
mediastinal
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Diagnostic diferential stadiul I
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Cauze de adenopatii mediastinale si hilare
TBC, infectii fungice (histoplasmoza)
Silicoza,antracoza, expunere la azbest, boli colagen vasculare, granulomatoza Wegener
Limfoame, leucemie
Adenopatii metastatice (cancer plaman, san, melanom, neoplasm renal,neoplasm testicular)
Alte cauze (hiperplazie reactiva, boala Castleman, imunodeficiente,etc
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Simetria:
In sarcoidoza de obicei adenopatiile sunt simetrice
adenopatii asimetrice in limfoame,TBC, metastaze
Unilateralitatea
rar in sarcoidoza
obisnuit malignitati,TBC,infectii fungice
Asociere adenopatii si infiltrate
sarcoidoza:cand apar modificari in parenchim adenopatiile se micsoreaza
modificarile pulmonare se coreleaza cu adenopatiile-in boli maligne, limfoame
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Adenopatii in limfoame maligne
Mase adenopatice in contiguitate de la un situs la
altul, pornind de la o localizare cervicala
primitiva ,interesarea lanturilor prevasculare,
adenopatii voluminoase cu aspect de mase conglomerate
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Adenomegalie in limfoame
Multiplicitate de situsuri ganglionare,sunt intersati ganglionii din mediastinul
anterior,mijlociu si posterior
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Stadiul 2Adenopatii si imagini pulmonare frecvent
bilaterale si simetrice si care intereseaza zonele centrale: perihilar si lobii superiori segmente posterioare si apicale
Micronoduli/noduli mai mari
Aspecte reticulare/reticulonodulare
Infiltrate segmentare
Mase conglomerate(>3cm)perihilar
Atelectazii situate distal de obstructii bronsice(prin leziuni granulomatoase endobronsice)
Rar pattern in ,,geam mat, miliara difuza, forme alveolare confluente, aspecte nodulare net circumscrise
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NODULIIimaginea radiologica de micronoduli apare ca
urmare a coalescentei leziunilor granulomatoase
distributie perilimfatica in stadii precoce si difuza in stadii avansate
peribronhovascular adiacent vaselor si bronhiilor perihilare (inalt sugestiv) /in campurile pulmonare superioare si mijlocii
in relatie cu scizurile(in lungul scizurilor)
regiunile subpleurale costale (localizare tipica de boala)
septurile interlobulare
regiunile centrilobulare
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noduli mici, bine definiti uni/ bilaterali mare variabilitate de aspecte individuale
redusi numeric, localizati focal / numar mare si tendinta la confluenta
noduli mai mari (1-4 cm ) solitari sau multipli pot excava au margini nete sau pot fi inconjurati de alti
noduli sateliti mai mici (semnul galaxiei) nu calcifica opacitati mari imprecis definite sau chiar
arii de condensare cu bronhograma
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Aspecte radiologice stadiul II
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• Adenopatii hilare, pattern reticulonodular peribronhovascular, perihilar
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Infiltrate si pattern reticulonodular
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Infiltrate bilaterale perihilare si micronoduli perihilari
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Infiltrate reticulonodulare, adenopatii hilare si paratraheale
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Opacitati imprecis definite perihilar,adenopatii Micronoduli perihilari
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Adenopatii ,infiltrate si micronoduli
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Aspecte CT in stadiul II
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Caz 1
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Caz 2
Micronoduli perilimfatici
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Caz 3
Micronoduli,infiltrate,arii de fibroza si adenopatii
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Caz 3
Adenopatii, condensari si noduli
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Caz 4
Adenopatii si interesare minima parenchimatoasa
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Caz 5
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Caz 6
Micronoduli, contur net, perilimfatici,predominant perihilar, lobi superiori,campuri mijlocii
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Adenopatii mediastinale asociate cu micronoduli
subpleurali,peribronhovasculari si condensari subpleurale
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Micronoduli si ingrosari
nodulare peribronhovasculare
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Diagnostic diferential in stadiul II
Un diagnostic diferential pentru fiecare tip de modificare parenchimatoasa dar in particular pentru leziunile nodulare •Limfangita carcinomatoasa•Silicoza•Antracoza•Beriloza•Tuberculoza pulmonara•In toate aceste afectiuni se respecta principiul de dispozitie perilimfatica cu noduli situati in interstitiul peribronhovascular perihilar, in relatie cu septurile interlobulare cu regiunile subpleurale si centrilobulare
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Stadiul 3
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•Infiltrate parenchimatoase fara adenopatii
•Infiltratele se localizeaza mai ales perihilar
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Stadiul 4
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Fibroza in sarcoidoza• semn predominant: ingrosari de septuri netede
sau nodulare
• bilateral pe arii focale predominant in lobii superiori
• fibroza asociaza distorsiuni bronsice si bronsiectazii de tractiune- deplasarea posterioara a traheei si bronhiilor principale(reducere de volum pulmonar in segmentele posterioare ale lobilor superiori)
• dispozitie pulmonara :predominant central, segmente posterioare
• mase conglomerate in lobii superiori, perihilar(similar fibrozei masive progresive)
• chisturi subpleurale de dimensiuni variabile si aspecte sporadice in fagure (campuri mijlocii si superioare,nu in baza ca la FPI)
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Leziuni de fibroza extensive,dispozitie
centrala,deplasare posterioara a carenei
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Alte aspecte intilnite in sarcoidoza
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Interesarea cailor aeriene in sarcoidoza
• Stenoze
• Distorsiuni
• Plaman in mozaic cu ,,air trapping’’ (arii mici de hipoatenuare de hipertransparenta mai evidente in expir ) semnifica interesarea cailor aeriene mici
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Stenoza bronsica
• secundara fibrozei si tractiunilor extrinseci(mai ales in cazul maselor fibrotice conglomerate localizate parahilar)
• endobronsica prin granuloamele bronsice
• rar prin compresiune extrinseca secundara adenopatiilor
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• Stenoza poate fi unica sau multipla
• Afecteaza bronhiile lobare sau diviziunile segmentare proximale
• Granuloamele(mase endobronsice) pot da obstructii localizate cu episoade de colaps segmentar sau lobar (mai frecvent lobul mediu)
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AspergilozaUneori in sarcoidoza in urma unor procese de necroza pulmonara pot apare cavitati in care se produce colonizarea cu
Aspergillus
Micetom intracavitar
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Sarcoidoza pleurala • Granuloame la nivelul pleurei viscerale si
parietale care nu genereaza semne sau simptome
• Rar pleurita sau pleurezie
• Pleurezia apare in context de boala extensiva pulmonara sau interesare multisistemica
• Uni/bilaterala /cantitate variabila
• Dispare spontan /poate lasa ingrosare pleurala
• Deoarece apare incidental impune un diagnostic diferential in special cu TBC ( se recomanda biopsie pleurala)
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Pneumotorax
• in faze avansate de fibroza
• sau consecinta unor bule subpleurale sau granuloame pleurale multiple subpleurale
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Manifestari osoase • Afectarea scheletului este focala sau generalizata
• Cel mai frecvent sunt afectate oasele mici de la nivelul mainilor si picioarelor – leziuni chistice care confera osului un aspect trabecular sau in ,,fagure” rar eroziuni extensive
• Coloana vertebrala toracala si mai rar sternul si coastele
Aspecte :
• Leziuni distructive si tasari vertebrale
• Leziuni osteosclerotice vertebrale
• Spondilita
• Leziuni litice costale cu fractura pe os patologic(rar leziuni sclerotice)
• Deoarece leziunile osoase nu sunt specifice pot fi atribuite sarcoidozei doar in contextul satisfacerii criteriilor de diagnostic ( DD MTS osoase, mielom multiplu,etc)
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Manifestari vasculare
• Interesarea vaselor mari este rara, secundara compresiunilor extrinseci prin adenopatii sau tractiuni exercitate de procesele fibroase
• HTAP apare in caz de fibroza avansata
• Rar sindrom de VCS
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Rolul CT /HRCT
• Exista o mare variabilitate de aspecte imagistice intalnite in sarcoidoza
• Cunoasterea si recunoasterea unor imagini caracteristice ajuta si limiteaza DD imagistic si ofera un diagnostic cert daca exista argumente clinice si paraclinice
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CT evalueaza :
modificarile parenchimatoase
limfadenopatiile
extensia adenopatiilor(statii care nu sunt vizibile radiologic)
CT evidentiaza:
• anomalii parenchimatoase la pacienti cu radiografie aparent normala sau numai cu adenopatii
• utila la cei cu modificari parenchimatoase inaparente sau atipice (exista aspecte semiologice CT inalt sugestive, distributia acestora in raport cu elementele anatomice ale lobului pulmonar secundar)
CT ghideaza:
• manopere diagnostice(biopsie ganglionara/parenchimatoasa,transbronsica )
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CT –urmareste evolutia afectiunii
• Urmarirea afectiunii,evolutia sub tratament corelata cu parametrii biologici,complicatii( bronsiectazii, aspergiloza, fibroza, emfizem, infectii)
• Aspecte CT potential reversibile sub tratament micronoduli,aspecte in ,,geam mat’’de alveolita, condensari alveolare ( aspecte care caracterizeaza si boala activa)
• Aspecte CT ireversibile care nu au raspuns favorabil la terapie (,,fagure’’, chisturi, reticulatie grosiera, distorsiuni bronhovasculare, bronsiectazii de tractiune)
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Pentru diagnosticul de sarcoidoza este necesara concordanta intre elemente
clinice, radiologice si histologice
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BIBLIOGRAFIE • W.Richard Webb,Nestor l.Muller,David P.Naidich-High –
Resolution CTof the Lung Third edition Lippincot Williams&Wilkins 2000,285-302
• A .Cancellieri,G.Dalpiaz,M. Maffessanti-Diffuse Lung Diseases,Clinical features,Pathology,HRCT,2004, 94-97
• David M Hansell,David A.Linch,Peter Armstrong,H.Page Mc Adams-Imaging of the diseases of the chest Elsevier Mosby 2005 ;631-652
• Sebastian Lange-Radiology of Chest Diseases Thieme 2007; 91-94
• The spectrum of pulmonary sarcoidosis-variation of HRCT findings and clues for specific diagnosis European Journal of Radiology 2009
• Eric.J.Stern,Stephen J. Swensen-High Resolution CT of the Chest, Lippincot Williams&Wilkins,2010, 233-246.
• W.Richard Webb.Charles B.Higgins-Thoracic Imaging, Pulmonary and Cardiovascular Radiology,second edition Lippincot Williams&Wilkins, 2011,466-477