Transcript Pulmonary Fibrosis - American Osteopathic Association

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

Define internist’s role
Where is the interstitium?
170,000-800,000 alveoli in here
~1-1.5cm
~1-1.5cm
Classification based on etiology
ILD
Exposure-related
mold, bacteria, birds
medications
XRT
dusts
cigarette smoke
Genetic
FPF
Idiopathic
Sarcoidosis
CTD-related
RA
Systemic sclerosis
PM/DM
Sjögren’s syndrome
MCTD
UCTD
SLE
IIP
BOOP
DIP
OB
AIP
IPF
UIP
LIP
Hamman-Rich
RB-ILD
UIP
OP
BO
HP
NSIP
COP
DAD
CFA
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)
Classification based on histology
SC
INFLA
MM AT I O
N
AR
ILD
Genetic
Exposure-related
FPF
C AR
mold, bacteria, birds
INFLAM
medications
Autoimmune-related
XRT
dusts
RA
cigarette smoke
Systemic sclerosis
SC
PM/DM
AR
Sjögren’s syndrome
FLAMM AT
MCTD
S
M ATI O N
IN
IO N
SC
INFLA
MM ATI O
N
AR
Idiopathic
Sarcoidosis
LAM
IIP
SC
INFLA
MM ATI O
N
AR
Scar = bad prognosis
INFLA
Inflammation
N
SC
A
R
MM AT I O
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?

++++ Idiopathic pulmonary fibrosis (IPF)


++++ Connective tissue disease-related


Aging population
RA
+ Chronic hypersensitivity pneumonitis

Organic exposure (M/M/B/B
Making the diagnosis
You have to be a detective

History

Exam

Pulmonary physiology

Radiography

+/- surgical lung biopsy
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

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

Smoking
IPF
 DIP, RB-ILD, PLCH
 Goodpasture’s

PEARL
History: exposures
Be a good detective

Current or previous medications

www.pneumotox.com
 Chemotherapy
 Amiodarone
 Nitrofurantoin
PEARL

External beam radiation

Current or previous recreational drug use

Occupational, environmental, avocational
History: exposures
Be 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 detective

Birds (proteins)
Bloom on feathers
 Mucin in excrement
 Feather pillow/down comforter


Fumes, dusts, gases
Asbestos
 Beryllium

History: connective tissue
diseases

RA

Symmetric arthritis/small joints

Morning stiffness

Subcutaneous nodules

Smoker
PEARL
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
diseases

Sjögren’s Syndrome
Dry eyes/mouth
 Dental caries

History: connective tissue
diseases

PM/DM
Proximal muscle weakness
 Rashes
 Rough skin on the hands

Physical Exam
Physical examination
You’re still a detective

Skin
Rash
 Purupura
 Telangiectasia
 Nodules
 Calcinosis

Physical examination

Nails

Clubbing

COPD no clubbing
PEARL
Nailfold capillaroscopy
Abnormal
Normal
Fischer et al. Chest. In press
Physical examination

Chest

Velcro crackles are NEVER normal
PEARL
Must listen here
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

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 function testing

Gas exchange
Pulmonary function testing




Lung volumes
Spirometry
DLCO
ABG
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…
+
…but the DLCO will be very low
Impaired Gas Exchange


SpO2 at rest is unhelpful
Exercise oximetry


Never normal to desaturate
6-minute walk test
PEARL
Radiology: diagnosing ILD

“ILD protocol” HRCT
No IV contrast
 Supine and prone
 Inspiratory and expiratory images
 Reconstruction algorithm — 1-1.5mm thick

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
Interlobular
septal
thickening
Traction bronchiectasis
Reticular
opacities
Peripheral/subpleural
Lower zone
Honeycombing
Ground glass opacities
Lung biopsy

Transbronchial biopsy
Sarcoidosis
 Lymphangitic carcinomatosis
 Subacute HP


Surgical
Thorascopic
 Usually not if CTD-related

Putting it all Together

History

Exam

Labs


Physiology


6MWT
Radiology


Full PFTs
Gas exchange


ANA, RF, anti-CCP
HRCT
Pathology
Integrate to get
“summary diagnosis”
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
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

Q 3mos visits to pulm
 Subjective

Symptoms
 FVC
 DLCO
 6MWT
 Not
HRCT unless scenario mandates
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

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 dx

Refer to pulmonary rehabilitation

Vaccines

Sunscreen for all on immunosuppressive Tx

Monthly labs for all on immunosuppressive Tx
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