BRONSIECTAZIILE

CURS STUDENTI AN IVMODUL RESPIRATOR

DEFINITIE

• Dilatarea uneia/ mai multe bronhii>2mm permanenta (CT toracic)

• Deteriorea peretelui bronsic -componenta elastica - componenta musculara• Localizata/generalizata

DEFINITIA CLINICA

• Tuse cronica productiva• Sputa semnificativa cantitativ• Purulenta/piohemoptoica/hemoragica• +/- Dispnee, cianoza• NB forme uscate-lobi superiori, drenaj bun,

post TBC

EPIDEMIOLOGIE

ETIOPATOGENIE• OBSTRUCTII BRONSICE DOBANDITE -Aspiratii corp strain-alune, dinti, etc - Tumori - -papilomatoza laringiana, -teratom endobronsic -Adenopatii hil pulmonar-TBC, sarcoidoza, histoplasmoza -BPOC- bronsita cronica -Boala reumatica – policondrita, amiloidoza traheobronsica - Afectare secretie mucus- aspergilozabronhopulmonara, granulomatoza

bronhocentrica, postoperator

• ANOMALII CE CAUZEAZA OBSTRUCTIE BRONSICA= DOBANDITE- Traheobronsice –bronhomalacia, chistul bronsic, deficiente cartilaj( Sd

Williams-Campbell), traheobronhomegalia(sd Mounier-Kuhn), bronchii ectopice, fistula transesofagiana

-Vasculare- sechestrare pulmonara intralobara, anevrism artera pulmonara-Limfatice- sd unghii galbene

• IMUNODEFICIENTE-Deficite Ig G –agamaglobulinemie congenitala, deficite selective subclase Ig G, deficite

dobandite etc-Deficite de Ig A- deficit selectiv IgA cu/ fara sindrom ataxie-teleangiectazie-Disfunctie leucocitara- boala cronica granulomtoasa (NADPH oxidaza disfunctie)-Imunodeficiente umorale rare deficite e CD40, deficit de ligand C40 si hipergamaglo M• ANOMALII CLEARENCE SECRETIE BRONSICA-Defecte ciliare-sd Kartagener, diskinesia ciliara-Fibroza chistica-mucoviscidoza- tipic, se manifesta in copilarie. Prezentarea tardiva-adult-Sd Young- azoospermie si infectii sinopulmonare• CAUZE DIVERSE-Deficit alpha 1 antitripsina-absenta sau cu anomalie de funsctie-Pneumonie aspiratie recurenta- alcoolism, dezordini neurologice, pneumonie lipoidica-Boala reumatica- PR, sd Syogren-Boala Inflamatorie intestinala-b. Crohn, RUH-Inhalare toxice-amoniac, oxid nitric, inhalare pulberi, fum-(talc, silicati)-Rejet cronic transplant organ-transplant maduva osoasa, transplant cord-pulmon• INFECTII-Bacteriene- stafilococ auriu, Klebsiella, Pseudomonas Aeruginosa-Virale -adenovirusuri (7 si 21),-Infectii fungice (histoplasmoza)

ETIOPATOGENIE-FRCVENTA IN LOTURI STUDIATE

• IDIOPATICA• POSTINFECTIOASE• DEFICITE IMUNE (IgG, Ig A)• ABPA (aspergiloza bronhopulmonara)• ASPIRATIE/BRGE• SD YOUNG• RA • CF (cystic fibrosys)• DISFUNCTIA CILIARA

FORME ANATOMOCLINICE• FOCALE• Cicatrici postinfectioase• Obstructii cai aeriene-

tumori,corpi straini• Aspiratie-tulburari

neurologice, antecedente de neo cerebral sau laringian

• DIFUZE• Boli pulmonare-infectii

antecedente,injurie de aspiratie sau de inhalatie

• Boli sinopulmonare etiologie congenitala imunodeficienta• Alte boli sistemice• Idiopatic

MORFOPATOLOGIE• Dilatare lumen bronhii mijlocii extinse spre

bronhii periferice• Inflamatia cronica perete bronsic-distrugere

structura musculara si elastica• Depozite mucus intrabronsic• Mucoasa inflamata cu ulceratii la nivelul

mucoasei• Tesutul pulmonar distal-afectat prin infectii

repetate pneumonie post• Dilatare cale aeriana secundara leziunii perete

Clasificare REID• Cilindrice• Varicoase/moniliforme• Chistice/sacciforme

EXEMPLE RADIOLOGIC

Cylindrical bronchiectasis with signet-ring appearance. Note that the luminal airway diameter is greater than the diameter of the adjacent vessel.

Cystic and cylindrical bronchiectasis of the right lower lobe on a posterior-anterior chest radiograph.

3.Varicose bronchiectasis with alternating areas of bronchial dilatation and constriction

4.This CT scan depicts areas of both cystic bronchiectasis and varicose bronchiectasis.

TABLOUL CLINIC• ISTORIC-infectii pulmonare repetate la adult -un episod infectios sever in copilarie

(tuberculoza, pertusis, pneumonie severa) - Fibroza chistica azi cea mai frecv cauza la

adulti si copii• SIMPTOME• Tuse cronica, de obicei productiva (toaleta bronsica

matinala)• Sputa mucoasa, cantitatea crescuta in exacerbari

infectioase(<10 ml-usoare; 10-150ml/24h medii;>150ml-severe)

• Sputa poate deveni purulenta, hemopurulenta, hemoptoica, sangerare din vase bronsice ectatice

• Dispnee• Wheesing• Fatigabilitate• Durere pleuritica• Fatigabilitate• Scadere ponderala• Febra• Incontinenta urinara (femei)

EXAMEN FIZIC• EXAMEN FIZIC GENERAL nespecificIn stadii avansate- degete hipocratice -cianoza calda -pletora -semne cord pulmonar cronicPulmonar-nespecific -raluri crepitante in exacerbari -wheezingSemne si simptome suferinta sinusala cronica

DIAGNOSTIC DIFERENTIAL• Alpha1-antitrypsin (AAT) deficit• Astm bronsic• Bronsita acuta, cronica• BPOC• Fibroza chistica (CF)• Emfizem pulmonar• Empiem pleuropulmonar• Boala reflux gastroesofagean• Pneumonia de aspiratie• Pneumonia bacteriana• Tuberculoza

DIAGNOSTIC POZITIV• CT pulmonar –HRCT (high resolution CT)/radiografie standard• Examen sputa-biochimic, macroscopic, bacteriologic

(Pseudomonas Aeruginosa si E colii in infectii cronice)-frotiu si culturi, inclusiv pentru fungi; celularitate (atentie la eozinofile)

• HLG-anemie, leococitoza cu neutrofilie sau eozinofilie (aspergiloza alergica BP)

• Dozare imunoglobuline cantitativ• Dozare nivel AAT• Testul sudorii sau testare genetica pt fibroza chistica• Testarea precipitine la Aspergillus si determinare Ig E

serice(>1000UI) sau crestere de 2ori • Teste boala autoimuna ( factor reumatoid, anicorpi antinucleari)

High-resolution computed tomography scan in a 75-year-old man with cystic bronchiectasis

High-resolution computed tomography scan in a 13-year-old female adolescent shows left lower-lobe bronchiectasis, which is secondary to tuberculosis

The high-resolution computed tomography scan demonstrates findings of fluid-filled dilated bronchi in a 65-year-old man with bronchiectasis in the left lower lobe

TESTE FUNCTIONALE PULMONARE• Teste ventilatorii-frecvent model obstructiv, nereversibil la

bronhodilatator-scaderea FEV1-rar model restrictiv, in boala avansata si in

prezenta bronsiectazii prin tractiune, cicatriciale di fibroza pulmonara

TESTE SPECIALE• Microscopie electronica- evaluare structura si functie

epiteliu/cili (diskynesia ciliara primara) +/- examen sperma

• Bronhografie-material contrast prin cateter sau bronhoscop, apoi radiografie

-risc de spasm bronsic –• Bronhoscopie- nu de rutina, pentru evidentiere Tu

sau corpi starini• Lavaj bronhoalveolar-celularitate si culturi,

evidentierea infectiei primare sau secundare• Teste functie ciliara in vivo

COMPLICATII

TRATAMENT(non CF)

• Ameliorare simptome• Reducerea frecventei complicatiilor• Prevenirea exacerbarilor• Reducerea morbiditatii si mortalitatii• Depistarea precoce conditii asociate (AAT)

OBIECTIVELE TRATAMENTULUI

• 1.tratament exacerbari infectioase• 2.suprimarea inacarcarii microbiene• 3.reducerea inflamatiei• 4. promovarea igienei bronsice• 5. tratamentul conditiilor subiacente• 6.controlul sangerarii bronsice• 7.tratament chirurgicalPasteur et all, Thorax 2010

Tratment exacerbari infectie• Frecvent-cauza este H influenzae, Pseudomnas

aeruginosa tip mucoid, mai rar Streptococ Pneumoniae (difera de br. cronica), in SUA Micobacterium aviarum complex MAC(la cei cu HIV)

• Prelevare sputa pt examen bacteriologic• FLUOROQUINOLONA per os ( ciprofloxacin 500

mgx2/zi) 7-10 zile minim ambulator-nu se confirma beneficii la Tobramicina inhalator asociata (alternativa Amoxicilina 500 mg x3/zi 9doze mAIi mari la H influenzae)

• Reconsiderare daca fac recaderi frecvente si ex sputa• Stare genera• la grava-se poate asocia antibiotic IV• Up To Date, Thorax 2010 guidelines

Thorax 2010

Thorax, 2010, British guidelines

• PREVENIREA RECADERILOR1.ciprofloxacin 500-1500 mg/zi 2 sau 3 doze, 7-14 zile lunarsau2 .macrolid zilnic sau de 3 ori /saptamana in fibroza chistica-eritromicina 500mg x2/zi sau azithromycina 50 mg de 3

ori/saptamana 6 luni-este necesar sa excludem infectia cu mycobacterium nontuberculos

Sau3.Antibiotic aerosoli (nu au intrat inca in ghiduri)-tobramicina in aerosoli, gentamicina –studii mici-aztreonam-ciprofloxacina liposomal-ciprofloxacina pudra uscata-colistin aerosli (prep IV)-gentamicina aerosoli (prep IV)UpToDate, MedscAPE, Thorax 2010 Guidelines Pasteur et al

IGIENA BRONSICA• A.MASURI MECANICE• Tapotaj toracic• Drenaj postural• Tuse• Presiune expiratorie pozitiva• B. NEBULIZARE• Solutii saline hipertone• Discutabil alte mucolitice inhalator sau per os• Bronhodilatatoare aerosol la cei care au raspuns bronhodilatator la spirometrie-

initial bronhodiltator raspuns scurt( beta agonist), ulterior cu actiune de lunga durata

• C. ANTIINFLAMATOR• Glucocorticoizi sistemic doar in cazuri cu wheesing• Inhalator-fluticasone-controverse risc/beneficiu

• CHIRURGICAL-rezectie pt bronsiectazii localizate, cura hemoptiziilor ce nu raspund la terapie interventionala endobronsica(bronhoscopie)

• TRANSPLANTUL PULMONAR

• Albastru=medscape ultima• Rosu-uptodate • VERDE Bronchiectasis Workup• Author: Ethan E Emmons, MD; Chief Editor:

Zab Mosenifar, MD more. mEDSCAPE

PROGNOSTICLeicester cough questionnaire

SUPURATII PULMONARE

• Supuratiile pulmonare = afectiuni caracterizate prin etiologie infectioasa, inflamatie supurativa a parenchimuluipulmonar si /sau conducte aerice + bronhoree purulenta

CLASIFICAREA SUPURATII PULMONARE

• 1. Dupa sediu- pulmonare/-bronsice• 2.Etiologie- bacteriene/fungice/parazitare• 3.Evolutie ; acute/cronice• 4. Patogeneza-bronhogene(aspiratie)-hematogene(septicemii, embolii septice)-contiguitate transdiafragmatica• 5. Pe plaman initial indemn (primitive) sau

leziune preexistenta (secundare)

ABCESUL PULMONAR

• DEFINIRE =Necroza a parenchimului pulmonar cauzat de infectie microbiana

• “pneumonie necrotizanta” sau “gangrena pulmonara” –termeni ce definesc multiple leziuni mici , spre deosebire de o cavitate mare ( termenii sunt de fapt ai unui proces continuu)

FIZIOPATOLOGIE1.Evolutia unei pneumonii de aspiratie• Anaerobi mucoasa bucala gingivala• Predispozitie la aspirare- alcoolici, toxicomani,

anestezia generala, disfagia• Germenii trebuie sa ajunga in cai aeriene inferioare• Pneumonita si la 7-14 zile- necroza tisulara si

abcedare(de obicei, asociere germeni)2.Sd. Lemierre-tromboflebita supurativa vena jugulara-

punct de pornire la nivelul amigdale faringiene sau periamigdalian- invazie vase gat-tromboflebita septica-embol septic pulmonar

3. Endocardita Tricuspida- (Stafilococ auriu) –determinari secundare in ambii plamani

MICROBIOLOGIE

• Flora gingivala-Peptostreptococi, Prevotella, Bacteroides, Fusobacterium spp

• Germeni non-anaerobi- Streptococcus millieri, streptococi microaerophili(sunt frecvent asociati cu anaerobi)

• Alti germeni-S.aureus, K. pneumoniae, Spcoc pyogenes, Burkholderia pseudomallei, Haemophilus influenzae tip B, Nocardia, Acrtynomyces

• PARAZITI-Paragonimus westermani, Entamoeba hystolitica

• FUNGI- Aspergillus, Cryptoccocus neoformans, Histoplasma Capsulatum, Blastomyces dermatidis, Coccidioides immitis)

TABLOUL CLINIC

• ANAEROBI• Simptomatologie initial torpida, pentru saptamani, in

special la anaerobi• -febra• -tuse• -expectoratie • Semne de boala cronica- transpiratii nocturne, scadere in

greutate, anemie. • Posibil-hemoptizie sau pleurezie• Nu au frisoane• Sputa are miros fetid (exceptie S millieri) sau gust neplacut

• EX fizic- Boala gingivala- conditiile favorizante aspirare (tulb neurologice,

disfagie, alcoolism, etc)- -febra- -sindrom condensare sau/si sindrom lichidian pleural• Radiografie- infiltrat pulmonar cu cavitate in teritorii

dependente de pozitia culcat ( segment superiorlob inferior sau segment posterior lob superior)

GERMENI NONANAEROBI• Stafilococ auriu- boala cu tablou fulminant, la

adulti tineri sau adolescenti cu infectie gripala subiacenta

• -pot fi tulpini MRS+• Soc, neutropenie, necroza pulmonara

extensiva, mortalitate crescuta• Klebsiella Pneumoniae –raspuns dificil la

antibiotice, necroza , bacteriemie importanta• Nocardia- determina abces la

imunocompromisi, corticosteroizi de durata

DIAGNOSTIC DIFERENTIAL

UpToDate, 2012

LEZIUNI CAVITARE PULMONARE• 1.BACTERIENE-bcese anaerobi, abcese aerobi, bule

infectate, infarct pulmonar infectat, empiem, tuberculoza

• 2. Infectii fungice-Coccidioidomicoza, histoplasmoza, blastomicoza, aspergiloza

• 3.Infestari parazitare-Echinococoza, Amibiaza, • 4. Neoplazii –carcinom bronsic, carcinom metastatic,

limfom nonH, limfom H• 5. Leziuni inflamatorii pulmonare-granulomatoza

Wegener, sarcoidoza• Gherasim et al, 2010

DIAGNOSTICFORMA CLINICA CLASICA• Diagnostic bacteriologic –pt anaerobi, aspirat

transtraheal, sau transtoracic, sau pleural, sau hemoculturi ( nu bronhoscopie)

- Confirmarea bacteriologica in infectii cu anaerobi este rareori posibila

- Culturile din sputa, chiar daca au alti germeni-terapie antianaerobi in cazul prezentarii tipice.

• Diagnostic radiologic (Rx si CT)

• FORMA CLINICA NEOBISNUITA• -dg diferential leziuni cavitare-atentie TBC,

neoplasm, • -evidentiere conditii locale favorizante-corp

strain, neoplasm pulmonar, bronhostenoza(bronhoscopie, BAL, biopsii)

RADIOLOGIE

• 1. faza formare-opacitati neomogene, sferice, sau opacitati sistematizate uni sau plurisegmentare

• 2. faza supuratie deschisa-imagine hidroaerica intrapulmonara, nivel lichid comunica bronsia de drenaj\3.imagine cavitara cu contur gros

• + complicatii-piotorax, piopneumotorax

TRATAMENT anaerobi

• Clindamicina 600 mg IV la 8h apoi 150 mg/300 mg de 4 ori/zi superioara Penicilinei

(exista anaerobi ce secreta betalactamase)• Combinatie

inhibitorbetalactamasa/betalactam (clavulanat, sulbactam, aztreonam cu ampicilina, ticarcilina, piperacilin)

• Ampicilia-sulbactam 3g iv la 6 ore Carbapenem• Metronidazol in combinatie cu peniciline ( nu

are actiune pe streptococi microaerofili)

TRATAMENT alti germeni

• Gram negativi-functie de testare antibiograma,sunt sensibili la aceleasi regimuri

• Stafilococ MRS+ Linezolid 600 mg iv la 12h, apoi oral cand este

afebril, aceeasi dozaVancomycina- 15 mg/kgc/12ore-se determina

concentratia plasmatica, 15-20 mcg/ml-ajustar la creatinina

• Cel putin 3 saptamani• Pana la remiterea imaginii radiologice la

leziune mica, stabila; poate dura luni

• TRATAMENT CHIRURGICAL-doar rareori in abces anaerobi.

- Abcese peste 6 cm- -lipsa de raspuns la tratment medical- -complicatii locale

PROGNOSTIC

• Vindecare• Recidiva• Cronicizare-chirurgie