Interstitial lung disease for the pcp
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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. swigrisj@njhealth.org. 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


Interstitial lung disease for the pcp

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)



  • Interstitial lung disease for the pcp

    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


    Interstitial lung disease for the pcp

    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


    Interstitial lung disease for the pcp

    Interlobular septal thickening

    Traction bronchiectasis

    Reticular opacities

    Peripheral/subpleural

    Lower zone


    Interstitial lung disease for the pcp

    Honeycombing thickening


    Interstitial lung disease for the pcp

    Ground glass thickening opacities


    Lung biopsy
    Lung biopsy thickening

    • Transbronchial biopsy

      • Sarcoidosis

      • Lymphangitic carcinomatosis

      • Subacute HP

    • Surgical

      • Thorascopic

      • Usually not if CTD-related


    Putting it all together
    Putting it all Together thickening

    • 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 thickening

    • 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


  • Interstitial lung disease for the pcp

    STABILITY = SUCCESS thickening

    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 thickening

    • Q 3mos visits to pulm

      • Subjective

        • Symptoms

      • FVC

      • DLCO

      • 6MWT

      • Not HRCT unless scenario mandates


    Internist before ild dx
    Internist: before ILD dx thickening

    • 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 thickening

    • 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 thickening

    • Refer to pulmonary rehabilitation

    • Vaccines

    • Sunscreen for all on immunosuppressive Tx

    • Monthly labs for all on immunosuppressive Tx


    Five main points
    Five Main Points thickening

    • 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