interstitial lung disease for the pcp
DESCRIPTION
Interstitial Lung Disease for the PCP. Jeff Swigris, DO, MS Associate Professor of Medicine Interstitial Lung Disease Program National Jewish Health Denver, Colorado. [email protected]. Objectives. Define the interstitium Define ILD Finding the cause Clinical presentation Therapy - PowerPoint PPT PresentationTRANSCRIPT
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
[email protected]@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