内科学教学课件：16 间质性肺炎（Interstitial Lung Diseases）.pptVIP

InterstitialLungDiseases

(ILDs)

CaseFemale,52yearsoldChiefComplaintShortnessofbreathfor2monthsClinicalPresentationCoughwithminimalsputumMuscleandjointpains

ChestCT

ChestCT

ChestCT

ExaminationsBloodRoutineWBC2.3*10^9/L,N1.47*10^9/LLungFunctionFVC:70%,FEV1:78.6%,FEV1/FVC80%,TLCO51.5%Slightrestrictiveventilationdysfunction,moderatelydecreaseddiffusioncapacityAutoantibodiesAnti-JO-1(+),ANA1:100CreatinekinasesCK532U/L,CK-MBsubtype28U/L,CK-MMsubtype504.0U/L

Diagnosis?

ILDILDassociatedwithconnectivetissuedisorders(CTD)Multipledematomyositis

IntroductionILDsrepresentalargenumberofconditionsthatinvolvetheparenchymaofthelung-thealveoli,thealveolarepithelium,thecapillaryendothelium,andthespacesbetweenthesestructures,aswellastheperivascularandlymphatictissues.

AheterogeneousgroupofdisordersMorethan200entitiesDifficulttoclassify

Classification(2002ATS)已知原因的ILD特发性间质性肺炎（idiopathicinterstitialpneumonia，IIP）pneumonia,DIP)肉芽肿性ILD其他罕见ILD

Classification(2002ATS)KnownetiologyOccupationalandenvironmentalexposuresDrugCollagenvasculardisease–associatedILD

Classification(2002ATS)Idiopathicinterstitialpneumonia(IIPs)Idiopathicpulmonaryfibrosis(IPF)Nonspecificinterstitialpneumonia(NSIP)Cryptogenicorganizingpneumonia(COP)Acuteinterstitialpneumonia(AIP)Respiratorybronchiolitisinterstitiallungdisease(RB-ILD)Desquamativeinterstitialpneumonia(DIP)Lymphoidinterstitialpneumonia(LIP)

Clinic-Radiographic

-PathologicalClassification

Idiopathicinterstitialpneumonia(IIPs)

UIP(usualinterstitialpneumonia)——IPFNSIP(nonspecificinterstitialpneumonia)——NSIPOP(organizingpneumonia)——COPDAD(diffusealveolidamage)——AIPRB(respiratorybronchiolitis)——RBILDDIP(desquamativeinterstitialpneumonia——DIPLIP(lymphoidinterstitialpneumonia——LIP

Classification(2002ATS)GranulomatousILDSarcoidosisExtrinsicallergicalveolitisWegenergranuloma

Classificatio