Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2804_Библиотеки_им_академика_М_И_Перельмана
.pdf
Pulmonaryfunctiontests(PFTs)
Bloodtesting
Lungsamplingincludingbronchoscopyandvideo-assistedthoracicsurgery(VATS)
CHESTIMAGING
Review of old CXRs is often very helpful in assessing both the rate and extent of change of lung
diseaseovertime.
Upto10%ofCXRsmaybenormalinpatientswithILD;asaresult,anormalCXRmaynotexclude
ILDinsettingswheretheclinicalsuspicionofILDisveryhigh.
AsaresultofthewidevarietyofILDs,CXRsmayhaveahighlyvariableappearance.
The mostcommonabnormality onCXRinILD is a reticular patternoflinear opacities that may be
localizedorformanetworkinvolvingthelungsdiffusely.
However,nodularopacities, alveolaropacities,mixedalveolaropacities, andreticularopacitiesand
occasionallycysticchangesarealsoseen.
Extensivefibrosisofthelungsmayleadtovolumelossinoneorbothlungs.
Patients with knownor suspectedILDs shouldundergo CT scanningas thediagnosis of many ILDs
reliesonHRCT.
60
HRCTis a scanningtechniquethatusesthinslice (usually1-mm thick)images thatare obtainedand
processedusingahigh-frequencyreconstructionalgorithm.60Scansare obtainedwith thepatient ina
supinepositionduringabreathholdatmaximal inspirationandthenduringabreathholdatmaximal
expiration.
Proneimagingmaybeperformedincaseswhereatelectasisobscurestheposteriorlungbases.
The pattern on HRCT is important in determining the differential diagnosis of ILD (Table 10-6).
Notably,publishedguidelinesexistforthedefinitiveradiologicdiagnosisofcertainILDs,specifically
IPF.
60
TABLE10-6
CLINICALANDRADIOLOGICFEATURESOFINTERSTITIALLUNGDISEASES
ClinicalFeatures HRCTFindings
UIP
Insidious onset and progressive
dyspnea
Drycough
Poorly responsive to treatment, poor
long-termsurvival
Variable course punctuated by
intermittentexacerbations
Pattern can be associated with
connectivetissuedisease
Olderpatients>50y
Malepredominance
UIPonCTorbiopsyformsthe
radiologic basis for diagnosing
IPF in the absence of
underlyingcause
Subpleural, basal predominant
reticulation/interstitial
thickening
Honeycombing with/without
tractionbronchiectasis
Heterogeneous (geographic)
distribution
Absence of ground glass,
consolidation, micronodules,
cysts,orairtrapping
https://t.me/med1917
May have atypical distribution
infamilialcases
NSIP
Associatedwithyoungerpatients
Morecommoninfemales
Commonly associated with collagen
vascular diseases, including
scleroderma, rheumatoid arthritis, and
antisynthetasesyndrome
Response to therapy is variable
dependingonetiology
Interstitialthickening,oftenwith
peripheralsubpleuralsparing
Ground-glassinfiltrates
Tractionbronchiectasis
Homogeneousdistribution
In end-stage disease, may
develop fibrotic changes and
“bronchiolectasis” that
resemblesUIP
RB-ILD
Associatedwithcigarettesmoking
Generally responsive to smoking
cessation
Bronchiolocentric ground-glass
nodules with an upper lobe
predominance
DIP
Associated with cigarette smoking and
occupationalexposures
Generally responsive to smoking
cessation
Maybetreatedwithcorticosteroids
Peripheral ground-glass
opacitiesorconsolidation
May have small, well-defined
cysts
COP
Subacute course, often presents as
multiple outpatient treatment failures of
bronchitis/pneumonia
Oftenassociatedwithinfectionsordrug
exposures
Responsive to prolonged courses of
corticosteroids
Often recurs if steroids are withdrawn
toorapidly
Multifocal ground-glass
opacitiesandconsolidations
Usuallylowerlobepredominant
Infiltratesmaybemigratory on
serialimaging
May have “reverse halo” or
atollsign
Sarcoidosis
Dyspnea, cough, and chest pain are
commonpresentingsymptoms
Systemicsymptomsmaybeprominent
Approximately 1 in 20 cases are
asymptomatic and incidentally detected
onCXR
Almost any organ system may be
affected
Perilymphaticnodules
Patchyground-glassopacities
Reticularinfiltrates
Tractionbronchiectasis
Progressivemassivefibrosis
Hilar or mediastinal
lymphadenopathy
Fibrotic
HP/fibroticHP
with
honeycombing
PresentsinasimilarfashiontoUIP/IPF
There may be a history of systemic
symptoms(fever,myalgias)
Associated with environmental
Reticular abnormality with an
upper or mid-lung
predominance
Micronodules
https://t.me/med1917
exposures (birds, molds, hot tubs) but
theseareidentifiedin<50%ofcases
Mosaicattenuation/airtrapping
Peribronchovascular
predominance
Honeycombingmaybepresent
inmoreadvanceddisease
BAL,bronchoalveolarlavage;COP,cryptogenicorganizingpneumonia;DIP,desquamativeinterstitialpneumonia;HP,
hypersensitivitypneumonitis;HRCT,high-resolutionCT;ILD,interstitiallungdisease;IPF,idiopathicpulmonaryfibrosis;NSIP,
nonspecificinterstitialpneumonia;RB-ILD,respiratorybronchiolitis–associatedinterstitiallungdisease;UIP,usualinterstitial
pneumonia.
DatafromKadochMA,ChamMD,BeasleyMB,etal.Idiopathicinterstitialpneumonias:aradiology-pathologycorrelationbasedon
therevised2013AmericanThoracicSociety-EuropeanRespiratorySocietyclassificationsystem.CurrProblDiagnRadiol.
2015;44:15-25;RaghuG,CollardHR,EganJJ,etal.AnofficialATS/ERS/JRS/ALATstatement:idiopathicpulmonaryfibrosis–
evidence-basedguidelinesfordiagnosisandmanagement.AmJRespirCritCareMed.2011;183:788-824;WebbRW,Higgins
CB.ThoracicImaging:PulmonaryandCardiovascularRadiology.LippincottWilliams&Wilkins;2005.
PULMONARYFUNCTIONTESTING
PFTsareanoninvasivesetofteststhatallowforevaluationoflungfunction,stratificationofdisease
severity,andformonitoringofdiseaseprogressionovertime.
Complete pulmonary function testing involves spirometry, lung volumes, and diffusing capacity
(DLCO),alongwithrestingandexercisepulseoximetry.
PFTsallowustodeterminewhetherpatientshaverestrictivelungdisease,obstructivelungdisease,or
amixedpatternofdisease.ThisaidsinthedifferentialdiagnosisofILD.
WhilemostfibroticILDsdemonstratearestrictivepatternonPFTs,anumberofILDsmaydemonstrate
obstruction,includingsmoking-relatedlungdiseases(RB-ILDandPLCH),cysticlungdiseases(LAM
andBHD),andsarcoidosis.
DLCOiscommonlydecreasedinpatientswithILDbutisanonspecificfinding.
Insomecases,thepresenceofcoexistingCOPDinpatientswithILDsmayleadtoamixedpatternof
lungdisease onPFTs.Inothercases,pseudonormalizationofPFTsoccurswherethecombinationof
restrictionandobstructionleadstothefindingof“normal”testing.Inthesecases,alowDLCOmaybe
the only cluethat thepatienthas significantunderlying lungdisease whenthe PFTsare reviewed in
isolation.
Restingandexercisepulseoximetryarecommonlyassessedusinga6MWtest.
SerialPFTsobtainedatfollow-upvisitsareausefulwaytomonitorfordiseaseprogressionandmay
haveprognosticsignificance.
61
LABORATORIES
RoutinelaboratorytestingintheevaluationofILDpatientsincludesCBC,BMP,andLFTtesting.These
mayprovidecluestothediagnosis(eosinophilia).
ManydrugsusedinthetreatmentofILDrequireregularmonitoringofbloodcounts,renalfunction,or
liverfunction.
SerologictestingforCTDsisobtainedinallpatientswithclinicalstigmataofCTD.
Aldolase and creatininekinasemay be testedtoevaluateforevidenceofmyositis inpatientswith a
clinicalsuspicionforantisynthetasesyndrome.Incaseswheresuspicionishighformyositis,panelsof
muscle-specificantibodiesshouldalsobeobtained.
In any patient with a high suspicion for scleroderma or Sjögren disease, an ENA panel should be
obtained.ENApanelsvarybutcontainawidevarietyofantibodiestoscreenforCTD.
Inpractice,serologictestingisoftenobtainedinawiderangeofpatientstoexcludesubclinicalCTD.
https://t.me/med1917
Many centers obtain a minimum of an antinuclear antibody (ANA), rheumatoid factor, and a cyclic
citrullinatedpeptide antibodytests,evenpatientswith suspectedIPF, since rheumatoid arthritis ILD
oftenpresentswithaUIPpatternonHRCT.
In patients withcysticlungdiseaseonimaging,testing forvascular endothelialgrowthfactortypeD
(VEGF-D)may be helpfulinmaking the diagnosis ofLAM.Levels of VEGF-D> 800 pg/mL inthe
correctclinicalorradiographicsettingcanbediagnosticofLAM.
62
Genetic testing can also be helpful in cystic lung diseases and can screen for tuberous sclerosis–
associatedLAM(TSC1andTSC2genemutations)andBHD(FLCN,folliculingenemutations)where
appropriate.
LUNGSAMPLING
When an extensive evaluation does not result in a confident diagnosis, lung sampling can be
considered.
Bronchoalveolarlavage(BAL)isusedtosamplethecellularcontentofthelungs.Ithaslimitedutility
intheevaluationofILD.
BALisusefulinexcludingcoexistinginfectionand,insomecases,malignancy.Itisalsousefulincases
wherediffusealveolarhemorrhageoreosinophiliclungdiseaseissuspected.
63
LungbiopsyshouldonlybeundertakenatcenterswithexpertiseinevaluatingILDpatients.Generally,
biopsyshouldbereservedforcircumstanceswherethediagnosisisuncertainandclarificationwould
resultinasignificantlyalteredapproachtomanagement.
Whilelungbiopsyisdesiredinmanycases,patientswithILDareoftenconsiderablyphysiologically
impairedandmaynottoleratetheprocedure.
Although many patients tolerate lung biopsy well, certain subgroups of patients are predisposed to
complications,includingdecompensationoftheirILDfollowinglungbiopsy.64PatientswithIPFmay
developdiseaseexacerbationsfollowinglungbiopsy,resultingindiseaseprogressionandevendeath.
Twotypesoflungbiopsyareavailable:transbronchialforcepsbiopsy(TBBx)andVATSbiopsy.
TBBx is often performed along with BAL during bronchoscopy. Itis most useful incases inwhich
smallbiopsysamplessufficefordiagnosis.
TBBx has the highest yield in bronchiolocentric ILDs, such as sarcoidosis, berylliosis, and
lymphangiticcarcinomatosis.65Itisalsousefulincaseswhereeosinophilic pneumoniaor pulmonary
alveolarproteinosisissuspected.
TBBxisinsufficientfordifferentiatingmostidiopathicILDs,especiallybetweenUIPandNSIP,given
inadequatesamplesize.
However,a recentlydeveloped test,theEnvisia Genomic Classifier,uses genomicpatternsinTBBx
samplestodistinguishUIPfibroticlungdiseasefromnon-UIPfibroticlungdisease.Itismayhelpfulin
well-selectedpatientswithlungfibrosiswhowouldnottolerateVATSbiopsy.
Transbronchialcryobiopsyisaneweroptionthatallows forlargervolumetissuesampling without a
surgical lung biopsy. Further studies are required tointegrate this techniqueinto theILD diagnostic
algorithm.
66
IncaseswithUIPpatterns,VATSbiopsiesarepreferredastheyyieldtissuesampleslargeenoughfor
accuratediagnosis.HRCTshouldbeusedtotargetareasofactivediseaseandavoidlungregionswith
end-stagefibrosis,whichisnondiagnostic.
SpecificILDs
IdiopathicPulmonaryFibrosis
https://t.me/med1917
IPFisthemostcommonformofidiopathicinterstitialpneumonia.
The incidence of IPF in the US is estimated to be 7–16 cases per 100,000 population. In older
populations(e.g.,>65years),theincidencegoesupconsiderably.
67
Thepathophysiology is incompletelyunderstood,butalveolar epithelial cellinjury and dysregulated
tissuerepairarethoughttoplayasignificantrole.
AhistoryofcigarettesmokingisthestrongestriskfactorassociatedwithIPF.OtherriskfactorsforIPF
includeage>60years,malesex,andGERD.
60
Increasingly, familial clusters of IPF are being identified andare referred to as familial pulmonary
fibrosis(FPF).
Genetic variant associated with FPF includes those in pulmonary surfactant protein C (SFTPC),
surfactantproteinA2(SFTPA2),andmucin5B(MUC5B).
68
Around 15% cases of FPF have short telomere syndrome resulting from mutations in telomere
maintenance genes including telomerase RNA component (TERC), telomerase reverse transcriptase
(TERT),andothers.Shorttelomeresyndromecanpresentwithbonemarrowfailure,prematuregraying,
cirrhosis,naildystrophy,andmucosalleukoplakia.
69
PatientswithIPFareusuallyaged60yearsorolder.
They commonly present with slowly progressive dyspnea and nonproductive cough over years to
months.
Extrapulmonaryorsystemicsymptomsarerare.
Physicalexaminationmayrevealdryinspiratorycrackles.
Digitalclubbinghasbeenreportedin45%–75%ofcasesdependingontheseries.
70
AdiagnosisofIPFrequires:
exclusionofallothercausesoffibrosinglungdisease(CTD,HP,sarcoid)and
aradiographicpatternofdefiniteUIPonHRCT(Table10-6,Figure10-3)or
https://t.me/med1917
Figure 10-3 Usual interstitial pneumonitis.(Figure courtesy of Dr. Constantine Raptis, Mallinckrodt Institute of
Radiology.)
aUIPpatternonsurgicallungbiopsy.
Forpatientsrequiringlungbiopsytoconfirmthediagnosis ofIPF,VATSlungbiopsyispreferred,as
tissuesamplingTBBxisdiagnosticinlessthanone-thirdofcases.
71
A multidisciplinary approach employing discussion with pulmonary, radiology, and pathology staff
withILDexperienceincreasesdiagnosticaccuracy.
Radiographicpattern:
HRCTismandatoryforimagingILDsandparticularlyIPF.
A pattern of definite UIP on HRCT includes the presence of honeycombing, and subpleural and
basilarpredominantreticulation.Theaffectedportionsofthelungshoulddemonstrateageographic
orheterogeneousinvolvement.Thepresenceor absenceoftractionbronchiectasis does notimpact
thediagnosis.
The pattern of a definite UIP on HRCT in the appropriate clinical setting(where other potential
causesofILDhavebeenruledout)maybesufficienttodeterminethediagnosisofIPF.
InthesettingofaFPF,theHRCTpatternmaybeatypical,oftenlackingabasalpredominance.Even
histologically,strictlydefinedUIPisidentifiedinlessthanhalfofFPFcases.
72
Disease-modifyingtreatmentoptionsarelimitedinIPF(Table10-7).
ThereisnomedicalcureforIPF.
TABLE10-7
https://t.me/med1917
MEDICALTREATMENTOFSELECTEDINTERSTITIALLUNGDISEASES
ILD PotentialTherapeuticInterventions
a
Medication-inducedILD
Discontinueculpritmedication
Corticosteroids
Connectivetissuedisease–
associatedILD(UIP,NSIP,COP)
Corticosteroids
Immunosuppressive therapy (e.g.,
cyclophosphamide, azathioprine, mycophenolate,
rituximab)
IPF
Pirfenidone
Nintedanib
Considerationforparticipationinaclinicaltrial
DIP,RB-ILD
Smokingcessation
Corticosteroids(likelyoflimitedbenefit)
Sarcoidosis
Corticosteroids
Immunosuppressive therapy (e.g., methotrexate,
azathioprine,infliximab)
HP
Avoidoffendingantigens
Corticosteroids(likelyoflimitedbenefit)
Antifibrotictherapy
Immunosuppressivetherapy
a
Lungtransplantationisaconsiderationforselectpatientswithend-stageinterstitiallungdisease.
COP, cryptogenic organizing pneumonia; DIP, desquamative interstitial pneumonia; HP, hypersensitivity pneumonitis; ILD,
interstitial lung disease; IPF, idiopathic pulmonary fibrosis; NSIP, nonspecific interstitial pneumonia; RB-ILD, respiratory
bronchiolitis–associatedinterstitiallungdisease;UIP,usualinterstitialpneumonia.
Mostmedicationsstudiedhavenotbeenfoundtoimpactdiseaseprogressionandsomehaveproved
dangerous(increasedrisksofdeathandhospitalizationhavebeenassociatedwithcombineduseof
N-acetylcysteine,azathioprine,andprednisone).
73
Pirfenidone, anoral antifibrotic agent,andnintedanib, anoral tyrosinekinaseinhibitor,haveboth
beenshowntoslowtherateoflungfunctiondeclineinpatientswithIPF.74,75Theymayalsohavea
mortalitybenefit.
Themostfrequentsideeffectswithnintedanibarediarrhea(62%ofpatients),nausea,andvomiting.
The most frequent side effects of pirfenidone are skin rash (30%), photosensitivity, nausea, and
diarrhea.
Drug-inducedliverdiseasecanoccurwithbothagents,andmonitoringofliverfunctiontests(LFT)
is mandatory with both agents. Elevations in LFTs can be managed with dose adjustments or
discontinuation.
Pulmonaryrehabilitationhasbeenassociatedwithimprovementsin6MWdistanceandqualityoflife
inIPF.
73
Prognosis and clinical course are variable, but those diagnosed with mild, moderate, and severe
https://t.me/med1917
disease byspirometryhave beenreportedtohavemedian survivals of 55.6, 38.7, and27.4 months,
respectively.
74
PoorprognosticfactorsforIPF:
Declineinforcedvitalcapacityof>10%oversixmonths
DecreaseinDLCOof>15%oversixmonths
Decreasein6MWdistanceof>150mover12months
61
ExacerbationsofIPFarecharacterizedbyacuteworseningofdyspneaoroxygenation(within30days)
andnewground-glassopacifications/consolidationsonCTwith noevidenceofinfection,pulmonary
embolus,orheartfailure.
60
Exacerbationsaretypicallytreatedwithhigh-dosecorticosteroids,althoughtheirbenefithasnotbeen
systematically proven. Patients often do not return to their pre-exacerbation baseline after IPF
exacerbationsandmortalityratesarehigh.
76
Lung transplantation remains the ultimate therapy in patients with advanced IPF. Without lung
transplantation,outcomesinIPFremainpoor.Patientsshouldbereferredtoalungtransplantprogram
atthetimeofdiagnosisofIPF.
NonspecificInterstitialPneumonia
NSIPcandescribebotharadiographicpatternonHRCTandpathologicpatternonlungbiopsy.
Itisreferredtoasnonspecificbecausebiopsies,inparticular,lackthehistologicfeaturescharacteristic
ofotheridiopathicinterstitialpneumonias.
NSIPisoneofthesubtypesofidiopathicinterstitialpneumonia.However,idiopathicNSIPisrare.
NSIPismuchmorecommonlysecondarytoothercausesincluding77:
CTD: Including scleroderma, Sjögren disease, antisynthetase syndrome, and rheumatoid arthritis.
InterstitialpneumoniawithautoimmunefeaturesisadistinctsubcategoryofILDwherepatientswith
confirmed NSIP on HRCT or lung biopsy have clinical features of autoimmune disease not
conformingtoanyparticularCTD.
78
Drug toxicity: Drugs commonly associated with NSIP including amiodarone, nitrofurantoin,
methotrexate,statins,andvariouschemotherapeutic agents havebeenassociatedwithdevelopment
ofNSIP.
HIVinfection:Lesscommonintheageofhighlyactiveantiretroviraltherapy(HAART).
Others:TheseincludefibroticHP,FPF,graftversushostdisease,andIgG4disease.
PatientswithNSIPtendtobeyoungerthanthosewithIPFandaremorecommonlyfemale.
79
Duetothefrequencyofsecondarycauses,NSIPmaypresentwithfevers,chills,weightloss,orflu-like
symptoms.SymptomsofCTDarealsocommon.
HRCT demonstrates a combinationofground-glass and reticular opacities often ina peripheral and
basal predominantdistribution. Aperibronchovasculardistribution may alsobenoted.Thereisoften
sparing of the immediate subpleural space from involvement, which is relatively specific for NSIP
(Table10-6)(Figure10-4).
https://t.me/med1917
Figure 10-4 Nonspecific interstitial pneumonitis.(Figure courtesy of Dr. Constantine Raptis, Mallinckrodt Institute of
Radiology.)
OtherfeaturesofNSIPincludetractionbronchiectasis,centrilobularnodules,airtrappingonexhalation
imagingandrarelymicroscopichoneycombing.
While thediagnosis ofidiopathic NSIP mayrequire a surgical lungbiopsy, mostcases ofsecondary
NSIP do not when the diagnosis is certain from the history, physical examination, and laboratory
testing.Incaseswherelungbiopsyisrequired,VATSbiopsyisessential.
TreatmentofCTD-associatedNSIPusuallyinvolvesimmunosuppressionwithsteroids,incombination
with steroid-sparing agents such as mycophenolate, azathioprine, rituximab, or cyclophosphamide
(Table10-7).
ManagementofpatientswithCTD-relatedILDshouldbeundertakeninamultidisciplinaryfashion.
NSIP from drug toxicity is treated by discontinuation of theoffending agent. In some cases, steroid
therapymayberequired.
Other secondary causes of NSIP are treated by targeting the primary disease, for example, HIV
(HAART)andIgG4disease(steroids).
Incasesrefractorytotherapy,lungtransplantremainsanoptioninselectedinstances.
PatientswithNSIPhaveasignificantlybetterprognosisthanthosewithIPF.
79
https://t.me/med1917
HypersensitivityPneumonitis
HPisapulmonarysyndromeofvaryingclinicalpresentationandnaturalhistory.
It is the result of an immune-mediated pulmonary response directed against a plethora of potential
inhaledantigenstowhichanindividualisbothsensitizedandhyperresponsive.
Hundreds ofantigens have been described as causing HP andinclude bacteria, mycobacteria, fungi,
organicproteins,chemicals,andmetals.
80
Well-described clinical presentationsof HP includefarmer’s lung (exposuretomoldy hay) andbird
fanciers’disease(exposuretobirds).
Sinceaminority ofpeople exposed toinhaled antigensmanifest withdisease, disease expressionis
thought to be dependent on a complex interaction of antigen dose, intensity and duration of antigen
exposure,antigenimmunogenicity,andhostfactorssuchasgeneticsusceptibility.
Currently, the optimal characterization of HP is based on the presence or absence of fibrosis and
honeycombing oneither HRCTor pathologic samples. The radiologic phenotypes in particular have
prognosticsignificance81:
HPwithoutfibrosis(mediansurvival>14years)
HPwithfibrosis(mediansurvival7.95years)
HPwithfibrosisandhoneycombing(mediansurvival2.8years)
Acuteforms ofHP(correspondingtononfibroticdisease) maymanifestoverhourstoweeksandare
often temporally related to antigen exposure. They present with relatively rapid onset of dyspnea,
cough,andchesttightness.Inaddition,patientsmaymanifestwithsystemicsymptomsincludingfevers,
chills,myalgias,andmalaise.Resolutionisexpectedfollowingantigenremoval.
Chronicdisease(correspondingtofibrosisandhoneycombing)anddyspneaoftenprogressindolently
over time and are often associated with dry cough. Unlike IPF, chronic forms of HP can be
accompaniedbysystemicsymptomsincludinganorexia,weightloss,andfatigue.
Acarefulandthoroughexposurehistoryshouldbetakenandshouldincludeinquiryaboutexposureto
birdsorbirdfeathers(includingdownpillowsandcomforters),hottubs(associatedwithaerosolized
mycobacterial exposure), air humidifiers, moldy homes or workplaces, animal furs, epoxies, plant
matter,industrialdusts,andchemicals.
82
Specificantibodytestingforculpritantigenscanbesenttospecializedlabswhenindicated,although
positiveserologiesonlysupportexposuretotheantigensagainstwhichtheantibodiesaredirectedand
donotnecessarilyconfirmcausation.
Thecausativeexposure/antigenisidentifiedin<50%ofcases.
HRCT findings in nonfibrotic disease include nodular or diffuse ground-glass opacities, mosaic
appearanceoninspiratoryscans,andairtrappingonexpiratorysequences.
82
HRCT findings infibrotic andhoneycomb disease include upper lobe–predominantreticulation in a
bronchovascular distribution, mosaic attenuation on inspiratory scans, air trapping on expiratory
sequences,andofcoursehoneycombing(Table10-6).
82
Treatment should involve paying careful attention to identification of the offending antigen because
antigen avoidance,whenidentified, has beenassociatedwith significantlyimproved survival (Table
10-7).
6
Corticosteroid therapyhasbeenthe classic mainstay oftreatmentforfibrotic honeycombing disease,
althoughdatafromrandomizedclinicaltrialsarelacking.
Inselectedcases,atrialofimmunosuppressioncanbeattemptedwithclosefollow-up.
83
Mostrecently,nintedanibhasbeenproposedasanalternativetreatment.Thisisbasedontheresultsof
triallookingattheuseofnintedanibinprogressivefibrosingILDs.
84
https://t.me/med1917
Соседние файлы в папке Библиотека им академика М.И. Перельмана