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

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swigrisj_at_njhealth.org
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Objectives

• Define the interstitium
• Define ILD
• Finding the cause
• Clinical presentation
• Therapy
• Define internists role

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Where is the interstitium?
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170,000-800,000 alveoli in here
1-1.5cm
1-1.5cm
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Classification based on etiology
ILD
Genetic FPF
Exposure-related mold, bacteria, birds
medications XRT dusts
cigarette smoke
Idiopathic Sarcoidosis
IIP
CTD-related RA Systemic
sclerosis PM/DM Sjögrens syndrome
MCTD UCTD SLE
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DAD
BOOP
DIP
BO
UIP
OB
HP
NSIP
LIP
COP
Hamman-Rich
AIP
RB-ILD
IPF
UIP
CFA
OP
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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)

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Classification based on histology
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ILD
Genetic FPF
Exposure-related mold, bacteria, birds
medications XRT dusts
cigarette smoke
Idiopathic Sarcoidosis
LAM IIP
Autoimmune-related RA
Systemic sclerosis PM/DM
Sjögrens syndrome MCTD
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Scar bad prognosis
Inflammation
Fibrosis
Nicholson et al. Am J Respir Crit Care Med
20001622213-2217
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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

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Making the diagnosis You have to
be a detective

• History
• Exam
• Pulmonary physiology
• Radiography
• /- surgical lung biopsy

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History chief complaint

• Typically, ILD presents with
• Dyspneasubacute, insidious onset
• I thought I was just
• Getting older
• 5 heavier
• Out of shape
• /- dry cough
• Fatigue
• No wheeze, no chest pain

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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

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History exposuresBe a good detective

• Smoking PEARL
• IPF
• DIP, RB-ILD, PLCH
• Goodpastures

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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
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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)

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History exposuresBe a good detective

• Birds (proteins)
• Bloom on feathers
• Mucin in excrement
• Feather pillow/down comforter
• Fumes, dusts, gases
• Asbestos
• Beryllium

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History connective tissue diseases

• RA
• Symmetric arthritis/small joints
• Morning stiffness
• Subcutaneous nodules
• Smoker

PEARL
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History connective tissue diseases

• SSc
• Raynauds
• After 40 y.o. in FEMALE
• After 30 y.o. in MALE
• Esophageal dysmotility
• Skin tightening

PEARL
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History connective tissue diseases

• Sjögrens Syndrome
• Dry eyes/mouth
• Dental caries

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History connective tissue diseases

• PM/DM
• Proximal muscle weakness
• Rashes
• Rough skin on the hands

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Physical Exam
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Physical examinationYoure still a detective

• Skin
• Rash
• Purupura
• Telangiectasia
• Nodules
• Calcinosis

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Physical examination

• Nails
• Clubbing
• COPD no clubbing

PEARL
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Nailfold capillaroscopy
Abnormal
Normal
Fischer et al. Chest. In press
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Physical examination

• Chest
• Velcro crackles are NEVER normal

PEARL
Must listen here
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Laboratory
PEARLS

• ANAthe pattern matters
• Nucleolar ANA any titer TO RHEUM
• SSA is a myositis associated ab (ANA -)
• ACE level non-specific
• Dont order it
• HP panels unhelpful
• Precipitating IgG to organic antigens
• Dont order them

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Laboratory
PEARLS

• Isolated high MCV
• Methotrexate
• Azathioprine
• ??? Telomerase abnormality
• Elevated MCV
• History of bone marrow irregularities
• Premature graying
• Cryptogenic cirrhosis
• Pulmonary fibrosis

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Pulmonary physiology

• Pulmonary function testing
• Gas exchange

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Pulmonary function testing

• Lung volumes
• Spirometry
• DLCO
• ABG

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Patients with ILD have Restrictive Physiology

• Low static lung volumes
• Low forced volumes
• Low FVC
• Low FEV1
• Normal FEV1/FVC

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Volumes may be normal if

but the DLCO will be very low
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Impaired Gas Exchange
PEARL

• SpO2 at rest is unhelpful
• Exercise oximetry
• Never normal to desaturate
• 6-minute walk test

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Radiology diagnosing ILD

• ILD protocol HRCT
• No IV contrast
• Supine and prone
• Inspiratory and expiratory images
• Reconstruction algorithm 1-1.5mm thick

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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

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Interlobular septal
thickening
Traction bronchiectasis
Reticular opacities
Peripheral/subpleural
Lower zone
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Honeycombing
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Ground glass opacities
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Lung biopsy

• Transbronchial biopsy
• Sarcoidosis
• Lymphangitic carcinomatosis
• Subacute HP
• Surgical
• Thorascopic
• Usually not if CTD-related

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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
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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

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STABILITY SUCCESS
I dont want my patients ILD leaving clinic
thinking they dont have a serious condition I
dont want my patients with ILD leaving clinic
thinking they should go home, sit on their couch
and die
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Gauging Response

• Q 3mos visits to pulm
• Subjective
• Symptoms
• FVC
• DLCO
• 6MWT
• Not HRCT unless scenario mandates

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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

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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

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Internist after ILD dx

• Refer to pulmonary rehabilitation
• Vaccines
• Sunscreen for all on immunosuppressive Tx
• Monthly labs for all on immunosuppressive Tx

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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
  monitoringthe internist is vital here