Idiopathic Interstitial Pneumonias

Adam Guttentag MD

Diffuse interstitial lung diseases

Known causeKnown cause

Drug reactionDrug reaction

Collagen vascular diseaseCollagen vascular disease

pneumoconiosispneumoconiosis

Idiopathic interstitial pneumoniasIdiopathic interstitial pneumonias

GranulomatousGranulomatous

SarcoidSarcoid

Miscellaneous idiopathicMiscellaneous idiopathic

LAMLAM

LCHLCH

Idiopathic interstitial pneumonias

Distinct unrelated disordersDistinct unrelated disorders

Clinical, radiological and pathological dxClinical, radiological and pathological dx

Non-neoplastic, non-infectious diseaseNon-neoplastic, non-infectious disease

Subacute to chronic symptomsSubacute to chronic symptoms

Not always purely interstitialNot always purely interstitial

Usually characterized by varying degrees ofcellular proliferation and collagendepositionUsually characterized by varying degrees ofcellular proliferation and collagendeposition

IIP early classification – Liebow

UIPUIP

BIPBIP

DIPDIP

LIPLIP

GIPGIP

IIP new classification

UIP →IPFUIP →IPF

BIP →COP (BOOP)BIP →COP (BOOP)

DIP →DIP, RB-ILDDIP →DIP, RB-ILD

LIP →LIPLIP →LIP

GIP →hard metal pneumoconiosis)GIP →hard metal pneumoconiosis)

 →NSIP →NSIP

 →AIP →AIP

Idiopathic Interstitial Pneumonias Making the Diagnosis

HRCTHRCT

Identify IPF accurately and confidentlyIdentify IPF accurately and confidently

Guide lung biopsyGuide lung biopsy

Transbronchial bx, TTNBx, BAL of very limitedusefulnessTransbronchial bx, TTNBx, BAL of very limitedusefulness

Open lung biopsy in at least two lobes isrecommendedOpen lung biopsy in at least two lobes isrecommended

Histological patterns nonspecific: rule out knownprimary disease, such as drug reaction.Histological patterns nonspecific: rule out knownprimary disease, such as drug reaction.

Importance of accurate dx:

IPF

Fibrotic NSIP

Cellular NSIP,DIP, COP,RBILD

SURVIVAL

Idiopathic Pulmonary Fibrosis

Most common DILD (25-50%)Most common DILD (25-50%)

Chronic fibrosing disease with nonspecifichistological pattern of UIPChronic fibrosing disease with nonspecifichistological pattern of UIP

Diagnosis of exclusion: no knownunderlying disease causing fibrosisDiagnosis of exclusion: no knownunderlying disease causing fibrosis

AsbestosisAsbestosis

Collagen vascular diseaseCollagen vascular disease

Chronic hypersensitivity pneumonitisChronic hypersensitivity pneumonitis

Idiopathic Pulmonary Fibrosis Clinical

>50 years old>50 years old

>6 mo of DOE, dry cough, insidious onset>6 mo of DOE, dry cough, insidious onset

PFT’s: low DLCO, low FVC, high FEV1/FVCPFT’s: low DLCO, low FVC, high FEV1/FVC

Digital clubbing in 75%Digital clubbing in 75%

Poor prognosisPoor prognosis

Median survival from dx ~3 yearsMedian survival from dx ~3 years

No response to steroidsNo response to steroids

Candidates for lung txpCandidates for lung txp

Idiopathic Pulmonary FibrosisHistology

Temporal and spatialinhomogeneityTemporal and spatialinhomogeneity

Mixture of interstitialinflammatory infiltration,collagen deposition,fibroblast proliferation indifferent areasMixture of interstitialinflammatory infiltration,collagen deposition,fibroblast proliferation indifferent areas

Inflammation usually slightInflammation usually slight

Profusion of fibroblastic focicorrelates with prognosisProfusion of fibroblastic focicorrelates with prognosis

Idiopathic Pulmonary Fibrosis Radiology

CXR:CXR:

Low volumesLow volumes

Reticular opacitiesReticular opacities

Basilar predominanceBasilar predominance

HRCT: “UIP pattern”HRCT: “UIP pattern”

Basilar and peripheral disease, asymmetricalBasilar and peripheral disease, asymmetrical

Reticular densities earlyReticular densities early

GGO not prominentGGO not prominent

Architectural distortion, traction bronchiectasisArchitectural distortion, traction bronchiectasis

Honeycombing lateHoneycombing late

Basilar honeycombing, upper lung irregular lines mostspecificBasilar honeycombing, upper lung irregular lines mostspecific

Idiopathic Pulmonary Fibrosis Radiology

Radiological Dx of UIP pattern is 90%accurateRadiological Dx of UIP pattern is 90%accurate

Confident HRCT dx in 50-75% of casesConfident HRCT dx in 50-75% of cases

Need for OLBx in atypical casesNeed for OLBx in atypical cases

$X:\photos\Interstitial\HRCT\I P F\pa.jpg$

$X:\photos\Interstitial\HRCT\I P F\ct2.jpg$

$X:\photos\Interstitial\HRCT\IPF\IPF.jpg$

IPF

Nonspecific Interstitial PneumonitisNSIPNonspecific Interstitial PneumonitisNSIP

Newly defined disease (1994)Newly defined disease (1994)

Includes many patients formerly called IPFIncludes many patients formerly called IPF

More favorable prognosis than IPFMore favorable prognosis than IPF

Patients about 10 years younger than IPFPatients about 10 years younger than IPF

15-20% 5 year mortality15-20% 5 year mortality

Cough, dyspneaCough, dyspnea

Histologic pattern also seen with collagen vasculardisease, drug reactions, hypersensitivityHistologic pattern also seen with collagen vasculardisease, drug reactions, hypersensitivity

“Wastebasket” that includes a variety of etiologieswith a similar lung response?“Wastebasket” that includes a variety of etiologieswith a similar lung response?

Nonspecific Interstitial PneumonitisNonspecific Interstitial PneumonitisHistology

Temporally homogeneouscellular or collagenousinfiltrationTemporally homogeneouscellular or collagenousinfiltration

“Cellular” and “fibrotic”forms“Cellular” and “fibrotic”forms

Cellular form has betterprognosis and responds tosteroidsCellular form has betterprognosis and responds tosteroids

Nonspecific Interstitial PneumonitisNonspecific Interstitial Pneumonitis

CXRCXR

Symmetric GGO in basesSymmetric GGO in bases

HRCTHRCT

Symmetric GGO in bases, subpleuralSymmetric GGO in bases, subpleural

Reticular opacitiesReticular opacities

Traction bronchiectasisTraction bronchiectasis

Honeycombing unusualHoneycombing unusual

$C:\Documents and Settings\guttenta\Desktop\nsip4.jpeg$

$X:\New Folder\NSIP HRCT.jpg$

NSIP

Cryptogenic Organizing PneumoniaCOPCryptogenic Organizing PneumoniaCOP

Name preferred to “BOOP”

Plugs of granulation tissue in bronchioles andalveolar ducts extending into air spaces

Organizing pneumonia is the predominant feature

Temporal homogeneity

Pattern seen with drug reactions, IBD, organizingaspirations, organizing infections, collagenvascular diseases

COP is the idiopathic form of this histological dx

Cryptogenic Organizing PneumoniaCryptogenic Organizing Pneumonia

Mean 55 years oldMean 55 years old

<3 months of sx<3 months of sx

Cough, fever, myalgias, dyspneaCough, fever, myalgias, dyspnea

Elevated ESR, C-reactive proteinElevated ESR, C-reactive protein

Excellent response to steroids, may recur iftx stopped earlyExcellent response to steroids, may recur iftx stopped early

Cryptogenic Organizing PneumoniaRadiologyCryptogenic Organizing PneumoniaRadiology

CXRCXR

Patchy bilateral consolidationsPatchy bilateral consolidations

Lower lobe predominanceLower lobe predominance

Opacities may be mobileOpacities may be mobile

HRCTHRCT

Peripheral consolidations with airbronchogramsPeripheral consolidations with airbronchograms

asymmetricasymmetric

GGO commonGGO common

Nodules less common, centrilobularNodules less common, centrilobular

COP

Acute Interstitial PneumoniaAIP

= Hamman-Rich syndrome, Idiopathic ARDS= Hamman-Rich syndrome, Idiopathic ARDS

Unusual diseaseUnusual disease

“Diffuse alveolar damage” histological pattern“Diffuse alveolar damage” histological pattern

Histology identical to ARDS from other causesHistology identical to ARDS from other causes

Exudation  proliferation  fibrosisExudation  proliferation  fibrosis

Acute illness: 2-3 weeks after viral-type sxAcute illness: 2-3 weeks after viral-type sx

Fever, cough, SOB, hypoxiaFever, cough, SOB, hypoxia

Respiratory failureRespiratory failure

Mortality >50% in 3 monthsMortality >50% in 3 months

Acute Interstitial PneumoniaRadiology

CXRCXR

Bilateral homogeneous air space opacitiesBilateral homogeneous air space opacities

No pleural effusionNo pleural effusion

Spares costophrenic anglesSpares costophrenic angles

HRCTHRCT

Progressive GGO +/- dependent consolidationProgressive GGO +/- dependent consolidation

Mosaic patternMosaic pattern

Later, fibrotic findings with tractionbronchiectasis, architectural distortionLater, fibrotic findings with tractionbronchiectasis, architectural distortion

Acute Interstitial Pneumonia

$C:\Documents and Settings\guttenta\Desktop\aip.jpeg$

$X:\New Folder\AIP CXR.jpg$

$X:\New Folder\AIP CT.jpg$

Desquamative Interstitial PneumonitisDIP

Misnomer: neither desquamative nor interstitialMisnomer: neither desquamative nor interstitial

Macrophages in distal air spacesMacrophages in distal air spaces

Normal interstitiumNormal interstitium

Rare disease of smokers in 30’s and 40’s (M>F)Rare disease of smokers in 30’s and 40’s (M>F)

Cough and dyspnea for monthsCough and dyspnea for months

Clubbing in 50%Clubbing in 50%

Restrictive PFT’s, low DLCORestrictive PFT’s, low DLCO

Excellent response to smoking cessation, steroidsExcellent response to smoking cessation, steroids

Desquamative Interstitial PneumonitisRadiology

CXRCXR

 25% normal 25% normal

Bilateral heterogeneous opacities, mostlybasilarBilateral heterogeneous opacities, mostlybasilar

HRCTHRCT

75% lower lobe predominance75% lower lobe predominance

Peripheral predilectionPeripheral predilection

GGO in allGGO in all

Some with early fibrosis, honeycombingSome with early fibrosis, honeycombing

Associated emphysema in upper lobesAssociated emphysema in upper lobes

DIP

Respiratory Bronchiolitis-Interstitial Lung DiseaseRB-ILD

Part of spectrum with DIP?Part of spectrum with DIP?

Seen in smokers in 30’s and 40’sSeen in smokers in 30’s and 40’s

More common in Europe – unfilteredcigarettes?More common in Europe – unfilteredcigarettes?

Usually Asx, some with cough, DOEUsually Asx, some with cough, DOE

Respiratory Bronchiolitis-Interstitial Lung DiseaseRadiology

CXRCXR

Vague reticulonodularopacitiesVague reticulonodularopacities

Bronchial wall thickeningBronchial wall thickening

“Dirty lungs”“Dirty lungs”

HRCTHRCT

Patchy GGOPatchy GGO

Poorly definedcentrilobular nodulesPoorly definedcentrilobular nodules

Associated emphysemaAssociated emphysema

Lymphocytic Interstitial PneumonitisLIP

Interstitial pneumonia orlymphoproliferative disorder?Interstitial pneumonia orlymphoproliferative disorder?

Probably not neoplasticProbably not neoplastic

Defined as idiopathic diffuse hyperplasticlymphoid infiltration of lungDefined as idiopathic diffuse hyperplasticlymphoid infiltration of lung

Interstitial infiltrate of lymphocytes and plasmacellsInterstitial infiltrate of lymphocytes and plasmacells

Included among DILD’s because of clinical,radiographic and histological appearanceIncluded among DILD’s because of clinical,radiographic and histological appearance

Lymphocytic Interstitial Pneumonitis

Associated with many autoimmune diseasesAssociated with many autoimmune diseases

Sjögren’sSjögren’s

Primary biliary cirrhosisPrimary biliary cirrhosis

Multicentric Castleman’s diseaseMulticentric Castleman’s disease

Rheumatoid arthritisRheumatoid arthritis

(AIDS in children)(AIDS in children)

Lymphocytic Interstitial Pneumonitis

F>MF>M

Mean age ~50Mean age ~50

Cough, dyspnea, wt loss, arthralgias,lymph node enlargementCough, dyspnea, wt loss, arthralgias,lymph node enlargement

May progress to fibrosisMay progress to fibrosis

Excellent response to steroidsExcellent response to steroids

Lymphocytic Interstitial PneumonitisRadiology

CXRCXR

Bilateral lower lobe reticulonodular andalveolar opacitiesBilateral lower lobe reticulonodular andalveolar opacities

Occ. consolidationOcc. consolidation

HRCTHRCT

Diffuse GGODiffuse GGO

Centrilobular and subpleural nodulesCentrilobular and subpleural nodules

Thickened BV bundlesThickened BV bundles

Thin walled cysts in 2/3Thin walled cysts in 2/3

+/- bronchiectasis, honeycombing+/- bronchiectasis, honeycombing

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosiAsbestosi

Chronic hypersensitivityChronic hypersensitivity

NSIPNSIP

Temporal homogeneityTemporal homogeneity

Fibrosis and inflammationFibrosis and inflammation

Basal , peripheral GGOBasal , peripheral GGO

ReticularReticular

Collagen vascularCollagen vascular

Drug reactionDrug reaction

DIPDIP

Chronic hypersensitivityChronic hypersensitivity

DIPDIP

Macrophages in alveoliMacrophages in alveoli

GGOGGO

Peripheral, basalPeripheral, basal

NSIPNSIP

hypersensitivityhypersensitivity

RB-ILDRB-ILD

RespiratorybronchiolitisRespiratorybronchiolitis

PeribronchioloarmacrophagesPeribronchioloarmacrophages

Bronchiolar fibrosisBronchiolar fibrosis

Centrilobular nodulesCentrilobular nodules

GGOGGO

Subacute hypersensitivitySubacute hypersensitivity

AIPAIP

DiffusealveolardamageDiffusealveolardamage

Exudation with hyalinemembranesExudation with hyalinemembranes

Cellular infiltratefibrosisCellular infiltratefibrosis

GGO and consolidationGGO and consolidation

Air bronchogramsAir bronchograms

ARDSARDS

InfectionInfection

Hydrostatic edemaHydrostatic edema

COPCOP

(BOOP)(BOOP)

OrganizingpneumoniaOrganizingpneumonia

Granulation plugs in smallairways and alveoliGranulation plugs in smallairways and alveoli

ConsolidationConsolidation

Peripheral, multifocalPeripheral, multifocal

BAC, lymphoma,infection,vasculitisBAC, lymphoma,infection,vasculitis

eosinophilic pneumoniaeosinophilic pneumonia

LIPLIP

Lymphocytes and plasmacells infiltrate interstitiumLymphocytes and plasmacells infiltrate interstitium

Cysts, GGO, nodulesCysts, GGO, nodules

Sarcoid, DIP, histiocytosisSarcoid, DIP, histiocytosis

$G:\photos\Cartoons\vacuum lock brain\vacuum lock brain .jpg$

$G:\photos\Cartoons\vacuum lock brain\brain pop.jpg$

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosisAsbestosis

Chronic hypersensitivityChronic hypersensitivity

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosisAsbestosis

Chronic hypersensitivityChronic hypersensitivity

NSIPNSIP

Temporal homogeneityTemporal homogeneity

Fibrosis and inflammationFibrosis and inflammation

Basal , peripheral GGOBasal , peripheral GGO

ReticularReticular

Collagen vascularCollagen vascular

Drug reactionDrug reaction

DIPDIP

Chronic hypersensitivityChronic hypersensitivity

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosisAsbestosis

Chronic hypersensitivityChronic hypersensitivity

NSIPNSIP

Temporal homogeneityTemporal homogeneity

Fibrosis and inflammationFibrosis and inflammation

Basal , peripheral GGOBasal , peripheral GGO

ReticularReticular

Collagen vascularCollagen vascular

Drug reactionDrug reaction

DIPDIP

Chronic hypersensitivityChronic hypersensitivity

DIPDIP

Macrophages in alveoliMacrophages in alveoli

GGOGGO

Peripheral, basalPeripheral, basal

NSIPNSIP

hypersensitivityhypersensitivity

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosisAsbestosis

Chronic hypersensitivityChronic hypersensitivity

NSIPNSIP

Temporal homogeneityTemporal homogeneity

Fibrosis and inflammationFibrosis and inflammation

Basal , peripheral GGOBasal , peripheral GGO

ReticularReticular

Collagen vascularCollagen vascular

Drug reactionDrug reaction

DIPDIP

Chronic hypersensitivityChronic hypersensitivity

DIPDIP

Macrophages in alveoliMacrophages in alveoli

GGOGGO

Peripheral, basalPeripheral, basal

NSIPNSIP

hypersensitivityhypersensitivity

RB-ILDRB-ILD

RespiratorybronchiolitisRespiratorybronchiolitis

PeribronchioloarmacrophagesPeribronchioloarmacrophages

Bronchiolar fibrosisBronchiolar fibrosis

Centrilobular nodulesCentrilobular nodules

GGOGGO

Subacute hypersensitivitySubacute hypersensitivity

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosisAsbestosis

Chronic hypersensitivityChronic hypersensitivity

NSIPNSIP

Temporal homogeneityTemporal homogeneity

Fibrosis and inflammationFibrosis and inflammation

Basal , peripheral GGOBasal , peripheral GGO

ReticularReticular

Collagen vascularCollagen vascular

Drug reactionDrug reaction

DIPDIP

Chronic hypersensitivityChronic hypersensitivity

DIPDIP

Macrophages in alveoliMacrophages in alveoli

GGOGGO

Peripheral, basalPeripheral, basal

NSIPNSIP

hypersensitivityhypersensitivity

RB-ILDRB-ILD

RespiratorybronchiolitisRespiratorybronchiolitis

PeribronchioloarmacrophagesPeribronchioloarmacrophages

Bronchiolar fibrosisBronchiolar fibrosis

Centrilobular nodulesCentrilobular nodules

GGOGGO

Subacute hypersensitivitySubacute hypersensitivity

AIPAIP

DiffusealveolardamageDiffusealveolardamage

Exudation with hyalinemembranesExudation with hyalinemembranes

Cellular infiltratefibrosisCellular infiltratefibrosis

GGO and consolidationGGO and consolidation

Air bronchogramsAir bronchograms

ARDSARDS

InfectionInfection

Hydrostatic edemaHydrostatic edema

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosisAsbestosis

Chronic hypersensitivityChronic hypersensitivity

NSIPNSIP

Temporal homogeneityTemporal homogeneity

Fibrosis and inflammationFibrosis and inflammation

Basal , peripheral GGOBasal , peripheral GGO

ReticularReticular

Collagen vascularCollagen vascular

Drug reactionDrug reaction

DIPDIP

Chronic hypersensitivityChronic hypersensitivity

DIPDIP

Macrophages in alveoliMacrophages in alveoli

GGOGGO

Peripheral, basalPeripheral, basal

NSIPNSIP

hypersensitivityhypersensitivity

RB-ILDRB-ILD

RespiratorybronchiolitisRespiratorybronchiolitis

PeribronchioloarmacrophagesPeribronchioloarmacrophages

Bronchiolar fibrosisBronchiolar fibrosis

Centrilobular nodulesCentrilobular nodules

GGOGGO

Subacute hypersensitivitySubacute hypersensitivity

AIPAIP

DiffusealveolardamageDiffusealveolardamage

Exudation with hyalinemembranesExudation with hyalinemembranes

Cellular infiltratefibrosisCellular infiltratefibrosis

GGO and consolidationGGO and consolidation

Air bronchogramsAir bronchograms

ARDSARDS

InfectionInfection

Hydrostatic edemaHydrostatic edema

COPCOP

(BOOP)(BOOP)

OrganizingpneumoniaOrganizingpneumonia

Granulation plugs in smallairways and alveoliGranulation plugs in smallairways and alveoli

ConsolidationConsolidation

Peripheral, multifocalPeripheral, multifocal

BAC, lymphoma,infection,vasculitisBAC, lymphoma,infection,vasculitis

eosinophilic pneumoniaeosinophilic pneumonia

IIP -- Quick reference guide

ClinicalsyndromeClinicalsyndrome

HistologicpatternHistologicpattern

Histologic featuresHistologic features

Imaging appearanceImaging appearance

Radiologic DDXRadiologic DDX

IPFIPF

UIPUIP

Temporal inhomogeneity,honeycombingTemporal inhomogeneity,honeycombing

Peripheral, basalPeripheral, basal

ReticularReticular

HoneycombingHoneycombing

Collagen vascularCollagen vascular

AsbestosisAsbestosis

Chronic hypersensitivityChronic hypersensitivity

NSIPNSIP

Temporal homogeneityTemporal homogeneity

Fibrosis and inflammationFibrosis and inflammation

Basal , peripheral GGOBasal , peripheral GGO

ReticularReticular

Collagen vascularCollagen vascular

Drug reactionDrug reaction

DIPDIP

Chronic hypersensitivityChronic hypersensitivity

DIPDIP

Macrophages in alveoliMacrophages in alveoli

GGOGGO

Peripheral, basalPeripheral, basal

NSIPNSIP

hypersensitivityhypersensitivity

RB-ILDRB-ILD

RespiratorybronchiolitisRespiratorybronchiolitis

PeribronchioloarmacrophagesPeribronchioloarmacrophages

Bronchiolar fibrosisBronchiolar fibrosis

Centrilobular nodulesCentrilobular nodules

GGOGGO

Subacute hypersensitivitySubacute hypersensitivity

AIPAIP

DiffusealveolardamageDiffusealveolardamage

Exudation with hyalinemembranesExudation with hyalinemembranes

Cellular infiltratefibrosisCellular infiltratefibrosis

GGO and consolidationGGO and consolidation

Air bronchogramsAir bronchograms

ARDSARDS

InfectionInfection

Hydrostatic edemaHydrostatic edema

COPCOP

(BOOP)(BOOP)

OrganizingpneumoniaOrganizingpneumonia

Granulation plugs in smallairways and alveoliGranulation plugs in smallairways and alveoli

ConsolidationConsolidation

Peripheral, multifocalPeripheral, multifocal

BAC, lymphoma,infection,vasculitisBAC, lymphoma,infection,vasculitis

eosinophilic pneumoniaeosinophilic pneumonia

LIPLIP

Lymphocytes and plasmacells infiltrate interstitiumLymphocytes and plasmacells infiltrate interstitium

Cysts, GGO, nodulesCysts, GGO, nodules

Sarcoid, DIP, histiocytosisSarcoid, DIP, histiocytosis

IIP Pearls

Honeycombingthink IPFHoneycombingthink IPF

Consolidationsthink COP or AIPConsolidationsthink COP or AIP

Cyststhink LIPCyststhink LIP

GGOthink DIP or NSIPGGOthink DIP or NSIP

GGO with reticulationthink NSIPGGO with reticulationthink NSIP

Centrilobular nodulesthink RB-ILDCentrilobular nodulesthink RB-ILD

RB-ILD and DIP only in smokersRB-ILD and DIP only in smokers

And remember…

This will change.

Reclassifying NSIP

DIP-RB-ILD relationship?

Causative factors may be identified.