Interstitial Lung Interstitial Lung Disease for the PCPDisease for the PCP

Jeff Swigris, DO, MSJeff Swigris, DO, MSAssociate Professor of MedicineAssociate Professor of MedicineInterstitial Lung Disease ProgramInterstitial Lung Disease Program

National Jewish HealthNational Jewish HealthDenver, ColoradoDenver, Colorado

swigrisj@njhealth.orgswigrisj@njhealth.org

ObjectivesObjectives

Define the interstitiumDefine the interstitium

Define ILDDefine ILD

Finding the causeFinding the cause

Clinical presentationClinical presentation

TherapyTherapy

Define internist’s roleDefine internist’s role

Where is the interstitium?Where is the interstitium?

170,000-800,000 alveoli in here170,000-800,000 alveoli in here

~1-1.5cm

~1-1.5cm

Classification based on etiologyClassification based on etiology

ILD

Exposure-related mold, bacteria, birds

medications XRT

dusts cigarette smoke

Idiopathic Sarcoidosis

IIP

Genetic FPF

CTD-related RA

Systemic sclerosis PM/DM

Sjögren’s syndrome MCTD

UCTD SLE

DAD

DIP

OP

RB-ILD

CFA

IPF

BOOP

COP LIPNSIP

UIP

AIPHamman-Rich

BO

HP

UIPOB

Idiopathic interstitial pneumonias (IIP)Idiopathic interstitial pneumonias (IIP)

Idiopathic pulmonary fibrosis (IPF) Idiopathic pulmonary fibrosis (IPF)

Nonspecific interstitial pneumonia (NSIP)Nonspecific interstitial pneumonia (NSIP)

Cryptogenic organizing pneumonia (COP)Cryptogenic organizing pneumonia (COP)(Idiopathic BOOP)(Idiopathic BOOP)

Acute interstitial pneumonia (AIP)Acute interstitial pneumonia (AIP)

Desquamative interstitial pneumonia (DIP)Desquamative interstitial pneumonia (DIP)

Respiratory bronchiolitis-ILD (RB-ILD)Respiratory bronchiolitis-ILD (RB-ILD)

Lymphoid interstitial pneumonia (LIP)Lymphoid interstitial pneumonia (LIP)

Classification based on histology

S C ARNFLAMMAT I O NI

ILD

Exposure-related mold, bacteria, birds

medications XRT

dusts cigarette smoke

Idiopathic Sarcoidosis

LAM IIP

Genetic FPF

Autoimmune-related RA Systemic sclerosis

PM/DM Sjögren’s syndrome

MCTD

S C ARNFLAMMAT NI OI

S C ARNFLAMMAT NI OI

S C ARNFLAMMAT NI OI

S C ARNFLAMMAT NI OI

Nicholson et al. Am J Respir Crit Care Med 2000;162:2213-2217

Scar = bad prognosisScar = bad prognosis

Fibrosis

Inflammation

SC

AR

NF

LA

MM

AT

I ON

I

What type of fibrosis is the PCP What type of fibrosis is the PCP most likely to see?most likely to see?

++++++++ Idiopathic pulmonary fibrosis (IPF) Idiopathic pulmonary fibrosis (IPF) Aging populationAging population

++++++++ Connective tissue disease-related Connective tissue disease-related RARA

++ Chronic hypersensitivity pneumonitis Chronic hypersensitivity pneumonitis Organic exposure (M/M/B/BOrganic exposure (M/M/B/B

Making the diagnosis Making the diagnosis You have to be a detectiveYou have to be a detective

HistoryHistory

ExamExam

Pulmonary physiologyPulmonary physiology

RadiographyRadiography

+/- surgical lung biopsy+/- surgical lung biopsy

History: chief complaintHistory: chief complaint

Typically, ILD presents with:Typically, ILD presents with: Dyspnea—subacute, insidious onsetDyspnea—subacute, insidious onset

““I thought I was just…” I thought I was just…” Getting olderGetting older 5# heavier5# heavier Out of shapeOut of shape

+/- dry cough+/- dry cough FatigueFatigue No wheeze, no chest painNo wheeze, no chest pain

HistoryHistoryBe a good detective

Symptoms/existence of concurrent diseaseSymptoms/existence of concurrent disease Patients may…Patients may…

1. Have known CTD1. Have known CTD2. Dyspnea from occult CTD-related ILD2. Dyspnea from occult CTD-related ILD

Family historyFamily history Pulmonary fibrosisPulmonary fibrosis Rheumatologic illnessRheumatologic illness

History: exposuresHistory: exposuresBe a good detective

Smoking Smoking PEARLPEARL IPFIPF DIP, RB-ILD, PLCHDIP, RB-ILD, PLCH Goodpasture’sGoodpasture’s

History: exposuresHistory: exposuresBe a good detective

Current or previous medicationsCurrent or previous medications www.pneumotox.com

ChemotherapyChemotherapy AmiodaroneAmiodarone NitrofurantoinNitrofurantoin

External beam radiationExternal beam radiation

Current or previous recreational drug useCurrent or previous recreational drug use

Occupational, environmental, avocationalOccupational, environmental, avocational

PEARLPEARL

History: exposuresHistory: exposuresBe a good detective

Microbial agentsMicrobial agentsM/M/B/BM/M/B/B

Hot tubs (indoor/enclosed)Hot tubs (indoor/enclosed)Basement showerBasement showerFree-standing humidifiersFree-standing humidifiersWater damage to homeWater damage to homeCooling systems (swamp cooler)Cooling systems (swamp cooler)

History: exposuresHistory: exposuresBe a good detective

Birds (proteins)Birds (proteins) Bloom on feathersBloom on feathers Mucin in excrementMucin in excrement Feather pillow/down comforterFeather pillow/down comforter

Fumes, dusts, gasesFumes, dusts, gases AsbestosAsbestos BerylliumBeryllium

History: connective tissue History: connective tissue diseasesdiseases

RARA Symmetric arthritis/small jointsSymmetric arthritis/small joints

Morning stiffnessMorning stiffness

Subcutaneous nodulesSubcutaneous nodules

SmokerSmoker PEARLPEARL

History: connective tissue History: connective tissue diseasesdiseases

SScSSc RaynaudsRaynauds

After 40 y.o. in FEMALEAfter 40 y.o. in FEMALEAfter 30 y.o. in MALEAfter 30 y.o. in MALE

Esophageal dysmotilityEsophageal dysmotility

Skin tighteningSkin tightening

PEARLPEARL

History: connective tissue History: connective tissue diseasesdiseases

Sjögren’s Syndrome Dry eyes/mouthDry eyes/mouth Dental cariesDental caries

History: connective tissue History: connective tissue diseasesdiseases

PM/DMPM/DM Proximal muscle weaknessProximal muscle weakness RashesRashes Rough skin on the handsRough skin on the hands

Physical ExamPhysical Exam

Physical examinationPhysical examinationYou’re still a detective

SkinSkin RashRash PurupuraPurupura TelangiectasiaTelangiectasia NodulesNodules CalcinosisCalcinosis

Physical examinationPhysical examination

NailsNails ClubbingClubbing

COPD no clubbingCOPD no clubbing PEARLPEARL

Nailfold capillaroscopyNailfold capillaroscopy

Normal

Abnormal

Fischer et al. Chest. In press

Physical examinationPhysical examination ChestChest

Velcro crackles are NEVER normalVelcro crackles are NEVER normal

Must listen here

PEARLPEARL

Laboratory Laboratory ANA—the pattern mattersANA—the pattern matters

Nucleolar ANA any titer – TO RHEUMNucleolar ANA any titer – TO RHEUM

SSA is a myositis associated ab (ANA -) SSA is a myositis associated ab (ANA -)

ACE level non-specificACE level non-specific Don’t order itDon’t order it

HP panels unhelpfulHP panels unhelpful Precipitating IgG to organic antigensPrecipitating IgG to organic antigens Don’t order themDon’t order them

PEARLSPEARLS

Laboratory Laboratory

Isolated high MCVIsolated high MCV MethotrexateMethotrexate AzathioprineAzathioprine

??? Telomerase abnormality??? Telomerase abnormalityElevated MCVElevated MCVHistory of bone marrow irregularitiesHistory of bone marrow irregularitiesPremature grayingPremature grayingCryptogenic cirrhosisCryptogenic cirrhosisPulmonary fibrosisPulmonary fibrosis

PEARLSPEARLS

Pulmonary physiologyPulmonary physiology

Pulmonary function testingPulmonary function testing

Gas exchangeGas exchange

Pulmonary function testingPulmonary function testing

Lung volumesLung volumes Spirometry Spirometry DLCODLCO ABGABG

Patients with ILD have Patients with ILD have Restrictive PhysiologyRestrictive Physiology

Low static lung volumesLow static lung volumes Low forced volumesLow forced volumes

Low FVCLow FVC Low FEV1Low FEV1

Normal FEV1/FVCNormal FEV1/FVC

Volumes may be normal if…Volumes may be normal if…

+

…but the DLCO will be very low

Impaired Gas ExchangeImpaired Gas Exchange

SpO2 at rest is unhelpfulSpO2 at rest is unhelpful Exercise oximetryExercise oximetry

Never normal to desaturateNever normal to desaturate 6-minute walk test6-minute walk test

PEARLPEARL

Radiology: diagnosing ILDRadiology: diagnosing ILD

““ILD protocol” HRCT ILD protocol” HRCT No IV contrastNo IV contrast Supine and prone Supine and prone Inspiratory and expiratory imagesInspiratory and expiratory images Reconstruction algorithm — 1-1.5mm thickReconstruction algorithm — 1-1.5mm thick

HRCT TerminologyHRCT Terminology

OpacitiesOpacities Lines (reticular)Lines (reticular) Dots or Circles (nodules)Dots or Circles (nodules) PatchesPatches

Attenuation (shade of gray)Attenuation (shade of gray) ConsolidationConsolidation – obscures underlying vessels – obscures underlying vessels Ground glassGround glass – does not obscure underlying vessels – does not obscure underlying vessels

Lower zoneLower zone

Peripheral/subpleuralPeripheral/subpleural

Reticular Reticular opacitiesopacities

Traction bronchiectasisTraction bronchiectasis

Interlobular Interlobular septal septal

thickeningthickening

HoneycombingHoneycombing

Ground glassGround glass opacities opacities

Lung biopsyLung biopsy

Transbronchial biopsyTransbronchial biopsy SarcoidosisSarcoidosis Lymphangitic carcinomatosisLymphangitic carcinomatosis Subacute HPSubacute HP

SurgicalSurgical ThorascopicThorascopic Usually not if CTD-relatedUsually not if CTD-related

Putting it all TogetherPutting it all Together HistoryHistory

ExamExam

LabsLabs ANA, RF, anti-CCPANA, RF, anti-CCP

PhysiologyPhysiology Full PFTsFull PFTs

Gas exchangeGas exchange 6MWT6MWT

RadiologyRadiology HRCTHRCT

PathologyPathology

Integrate to get “summary diagnosis”

Therapy for ILDTherapy for ILD

Not all patients require therapyNot all patients require therapyGeneral: treat clinically significant, progressive dzGeneral: treat clinically significant, progressive dz

All therapeutic regimens require monitoringAll therapeutic regimens require monitoring

Glucocorticoids may be the mainstayGlucocorticoids may be the mainstay

Steroid-sparing / immune-suppressing / Steroid-sparing / immune-suppressing / immunomodulatory / cytotoxic agentsimmunomodulatory / cytotoxic agents

NuanceNuance

STABILITY = SUCCESS

I don’t want my patients ILD leaving clinic thinking they don’t have a serious condition

I don’t want my patients with ILD leaving clinic thinking they should go home, sit on

their couch and die

Gauging ResponseGauging Response

Q 3mos visits to pulmQ 3mos visits to pulmSubjectiveSubjective

SymptomsSymptoms

FVCFVCDLCODLCO6MWT6MWTNot HRCT unless scenario mandatesNot HRCT unless scenario mandates

Internist: before ILD dxInternist: before ILD dx

Thorough history and examinationThorough history and examination

Order HRCTOrder HRCT

Order serologiesOrder serologies ANA with pattern and ENA panelANA with pattern and ENA panel RF/anti-CCPRF/anti-CCP

Order PFTs/6MWT/HRCTOrder PFTs/6MWT/HRCT

Refer: ILD on HRCTRefer: ILD on HRCT

Internist: after ILD dxInternist: after ILD dx Monitor for side effects of therapyMonitor for side effects of therapy

GlucocorticoidsGlucocorticoids WeightWeight SugarSugar BPBP EyesEyes BonesBones

Be on the lookout for infectionBe on the lookout for infection

Monitor need for oxygenMonitor need for oxygen

Communicate with patientCommunicate with patientMood: therapy needed?Mood: therapy needed?End-of-life discussionsEnd-of-life discussions

Internist: after ILD dxInternist: after ILD dx

Refer to pulmonary rehabilitationRefer to pulmonary rehabilitation

VaccinesVaccines

Sunscreen for all on immunosuppressive TxSunscreen for all on immunosuppressive Tx

Monthly labs for all on immunosuppressive TxMonthly labs for all on immunosuppressive Tx

Five Main PointsFive Main Points You will see ILD — be a detective You will see ILD — be a detective

Velcro crackles never normal — get HRCTVelcro crackles never normal — get HRCT

Surgical lung biopsy often needed to make a Surgical lung biopsy often needed to make a confident diagnosisconfident diagnosis

All patients and most therapies require All patients and most therapies require monitoring—the internist is vital heremonitoring—the internist is vital here