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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

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interstitial lung disease for the pcp

Interstitial Lung Disease for the PCP

Jeff Swigris, DO, MS

Associate Professor of Medicine

Interstitial Lung Disease Program

National Jewish Health

Denver, Colorado

objectives
Objectives
  • Define the interstitium
  • Define ILD
  • Finding the cause
  • Clinical presentation
  • Therapy
  • Define internist’s role
classification based on etiology
Classification based on etiology

ILD

GeneticFPF

Exposure-relatedmold, bacteria, birds medications XRT dusts cigarette smoke

Idiopathic Sarcoidosis IIP

CTD-relatedRA Systemic sclerosis PM/DM Sjögren’s syndrome MCTD UCTD SLE

slide8

BOOP

DAD

DIP

BO

OB

UIP

HP

NSIP

LIP

COP

Hamman-Rich

AIP

RB-ILD

IPF

UIP

CFA

OP

idiopathic interstitial pneumonias iip
Idiopathic interstitial pneumonias (IIP)
  • Idiopathic pulmonary fibrosis (IPF)
  • Nonspecific interstitial pneumonia (NSIP)
  • Cryptogenic organizing pneumonia (COP)
      • (Idiopathic BOOP)
  • Acute interstitial pneumonia (AIP)
  • Desquamative interstitial pneumonia (DIP)
  • Respiratory bronchiolitis-ILD (RB-ILD)
  • Lymphoid interstitial pneumonia (LIP)
slide11

ILD

GeneticFPF

Exposure-relatedmold, bacteria, birds medications XRT dusts cigarette smoke

Idiopathic Sarcoidosis LAM IIP

Autoimmune-relatedRA Systemic sclerosis PM/DM Sjögren’s syndrome MCTD

slide12

Scar = bad prognosis

Inflammation

Fibrosis

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

what type of fibrosis is the pcp most likely to see
What type of fibrosis is the PCP most likely to see?
  • ++++ Idiopathic pulmonary fibrosis (IPF)
    • Aging population
  • ++++ Connective tissue disease-related
    • RA
  • + Chronic hypersensitivity pneumonitis
        • Organic exposure (M/M/B/B
making the diagnosis you have to be a detective
Making the diagnosis You have to be a detective
  • History
  • Exam
  • Pulmonary physiology
  • Radiography
  • +/- surgical lung biopsy
history chief complaint
History: chief complaint
  • Typically, ILD presents with:
    • Dyspnea—subacute, insidious onset
      • “I thought I was just…”
        • Getting older
        • 5# heavier
        • Out of shape
    • +/- dry cough
    • Fatigue
    • No wheeze, no chest pain
history be a good detective
HistoryBe a good detective
  • Symptoms/existence of concurrent disease
    • Patients may…
      • 1. Have known CTD
      • 2. Dyspnea from occult CTD-related ILD
  • Family history
    • Pulmonary fibrosis
    • Rheumatologic illness
history exposures be a good detective
History: exposuresBe a good detective
  • Smoking PEARL
    • IPF
    • DIP, RB-ILD, PLCH
    • Goodpasture’s
history exposures be a good detective1
History: exposuresBe a good detective
  • Current or previous medications
      • www.pneumotox.com
        • Chemotherapy
        • Amiodarone
        • Nitrofurantoin
  • External beam radiation
  • Current or previous recreational drug use
  • Occupational, environmental, avocational

PEARL

history exposures be a good detective2
History: exposuresBe a good detective
  • Microbial agents
    • M/M/B/B
      • Hot tubs (indoor/enclosed)
      • Basement shower
      • Free-standing humidifiers
      • Water damage to home
      • Cooling systems (swamp cooler)
history exposures be a good detective3
History: exposuresBe a good detective
  • Birds (proteins)
    • Bloom on feathers
    • Mucin in excrement
    • Feather pillow/down comforter
  • Fumes, dusts, gases
    • Asbestos
    • Beryllium
history connective tissue diseases
History: connective tissue diseases
  • RA
    • Symmetric arthritis/small joints
      • Morning stiffness
    • Subcutaneous nodules
    • Smoker

PEARL

history connective tissue diseases1
History: connective tissue diseases
  • SSc
    • Raynauds
      • After 40 y.o. in FEMALE
      • After 30 y.o. in MALE
    • Esophageal dysmotility
    • Skin tightening

PEARL

history connective tissue diseases2
History: connective tissue diseases
  • Sjögren’s Syndrome
    • Dry eyes/mouth
    • Dental caries
history connective tissue diseases3
History: connective tissue diseases
  • PM/DM
    • Proximal muscle weakness
    • Rashes
    • Rough skin on the hands
physical examination you re still a detective
Physical examinationYou’re still a detective
  • Skin
    • Rash
    • Purupura
    • Telangiectasia
    • Nodules
    • Calcinosis
physical examination
Physical examination
  • Nails
    • Clubbing
    • COPD no clubbing

PEARL

nailfold capillaroscopy
Nailfold capillaroscopy

Abnormal

Normal

Fischer et al. Chest. In press

physical examination1
Physical examination
  • Chest
    • Velcro crackles are NEVER normal

PEARL

Must listen here

laboratory
Laboratory

PEARLS

  • ANA—the pattern matters
    • Nucleolar ANA any titer – TO RHEUM
  • SSA is a myositis associated ab (ANA -)
  • ACE level non-specific
    • Don’t order it
  • HP panels unhelpful
    • Precipitating IgG to organic antigens
    • Don’t order them
laboratory1
Laboratory

PEARLS

  • Isolated high MCV
    • Methotrexate
    • Azathioprine
    • ??? Telomerase abnormality
      • Elevated MCV
      • History of bone marrow irregularities
      • Premature graying
      • Cryptogenic cirrhosis
      • Pulmonary fibrosis
pulmonary physiology
Pulmonary physiology
  • Pulmonary function testing
  • Gas exchange
pulmonary function testing
Pulmonary function testing
  • Lung volumes
  • Spirometry
  • DLCO
  • ABG
patients with ild have restrictive physiology
Patients with ILD have Restrictive Physiology
  • Low static lung volumes
  • Low forced volumes
    • Low FVC
    • Low FEV1
  • Normal FEV1/FVC
volumes may be normal if
Volumes may be normal if…

+

…but the DLCO will be very low

impaired gas exchange
Impaired Gas Exchange
  • SpO2 at rest is unhelpful
  • Exercise oximetry
    • Never normal to desaturate
  • 6-minute walk test

PEARL

radiology diagnosing ild
Radiology: diagnosing ILD
  • “ILD protocol” HRCT
    • No IV contrast
    • Supine and prone
    • Inspiratory and expiratory images
    • Reconstruction algorithm — 1-1.5mm thick
hrct terminology
HRCT Terminology
  • Opacities
    • Lines (reticular)
    • Dots or Circles (nodules)
    • Patches
  • Attenuation (shade of gray)
    • Consolidation – obscures underlying vessels
    • Ground glass – does not obscure underlying vessels
slide41

Interlobular septal thickening

Traction bronchiectasis

Reticular opacities

Peripheral/subpleural

Lower zone

lung biopsy
Lung biopsy
  • Transbronchial biopsy
    • Sarcoidosis
    • Lymphangitic carcinomatosis
    • Subacute HP
  • Surgical
    • Thorascopic
    • Usually not if CTD-related
putting it all together
Putting it all Together
  • History
  • Exam
  • Labs
      • ANA, RF, anti-CCP
  • Physiology
      • Full PFTs
  • Gas exchange
      • 6MWT
  • Radiology
      • HRCT
  • Pathology

Integrate to get “summary diagnosis”

therapy for ild
Therapy for ILD
  • Not all patients require therapy
      • General: treat clinically significant, progressive dz
  • All therapeutic regimens require monitoring
  • Glucocorticoids may be the mainstay
  • Steroid-sparing / immune-suppressing / immunomodulatory / cytotoxic agents
      • Nuance
slide47

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 response
Gauging Response
  • Q 3mos visits to pulm
    • Subjective
      • Symptoms
    • FVC
    • DLCO
    • 6MWT
    • Not HRCT unless scenario mandates
internist before ild dx
Internist: before ILD dx
  • Thorough history and examination
  • Order HRCT
  • Order serologies
    • ANA with pattern and ENA panel
    • RF/anti-CCP
  • Order PFTs/6MWT/HRCT
  • Refer: ILD on HRCT
internist after ild dx
Internist: after ILD dx
  • Monitor for side effects of therapy
      • Glucocorticoids
        • Weight
        • Sugar
        • BP
        • Eyes
        • Bones
  • Be on the lookout for infection
  • Monitor need for oxygen
  • Communicate with patient
      • Mood: therapy needed?
      • End-of-life discussions
internist after ild dx1
Internist: after ILD dx
  • Refer to pulmonary rehabilitation
  • Vaccines
  • Sunscreen for all on immunosuppressive Tx
  • Monthly labs for all on immunosuppressive Tx
five main points
Five Main Points
  • You will see ILD — be a detective
  • Velcro crackles never normal — get HRCT
  • Surgical lung biopsy often needed to make a confident diagnosis
  • All patients and most therapies require monitoring—the internist is vital here
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