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ABIM Board Review:
Pulmonary Medicine
Overview

Topics
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Respiratory Infections
Airway Disease
Restrictive Lung Diseases
Pulmonary Vascular Disease
Pleural Disease
Sleep
Potpourri
Hints

Stress certain topics
– ASTHMA, TB, SARCOID, ILD’s,PFT’s
‘Things marked with these things are pearls
to remember’
 Disclaimer: A 3 year fellowship is difficult
to condense into a 1 hour lecture…

Respiratory Infections

Community Acquired PNA (CAP)
 HealthCare Assocoiated PNA
– Hospital Acquired PNA (HAP)
– Ventilator Associated PNA (VAP)
– Nursing Home Associated PNA

TB
Types of Pneumonia

Community Acquired
– Bacterial
– Atypical

Hospital Acquired >72 hours after
admission
 Ventilator Associated
 Aspiration Syndromes
Physical Signs of Pneumonia

Bronchial Breath sounds
 Dullness to percussion
 Egophony
Community Acquired
Pneumonia Bugs

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

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

Strep pneumoniae
mycoplasma
viral
Chlamydia
H. influenzae
Staph aureus
Legionella
Anerobic?
Hospital Acquired PNA Bugs

Gram negative enterics
 Staph aureus (MRSA)
 H. influenzae
 Strep pneumonia
Indicators of Severe
Pneumonia

Multilobar
 Advanced age
 Elevated BUN
 Elevated Respiratory rate
 High or Low WBC
 High or Low Temp.
 Hypotension
Antibiotics

Outpatient younger than 60
 ATS
– Macrolide or Tetracycline

IDSA
– Macrolide or Fluoroquinolone or Doxycycline
– Alternative: Oral second-generation
cephalosporin or Augmentin

E-mycin does not cover H. flu (COPD)
Antibiotics Cont.

Outpatient older than 60 or with co-morbid
conditions
 ATS
– Second generation cephalosporin, Bactrim,
Augmentin with or without a macrolide
 IDSA
– Macrolide or Fluoroquinolone or Doxycycline
– Alternative: Oral second-generation
cephalosporin or Augmentin
Antibiotics Cont.

Inpatient Ward
– ATS
 Second or third generation cephalosporin or beta
lactam/beta lactamase inhibitor with a macrolide
– IDSA
 Beta lactam with a macrolide or fluoroquinolone
alone
Antibiotics Cont.

Inpatient Severe
– ATS
 Macrolide with antipseudomonal agent plus an
aminoglycoside
– IDSA
 Cefotaxime, ceftriaxone, or Beta-lactam/ Betalactamase inhibitor with macrolide or
fluoroquinolone
Follow-Up CXR

Fifty percent of pneumonias clear within 2
weeks and 75 percent clear with in 6 weeks
 <5% of 20 year olds, 10-20% of 40 year
olds, 30% of 60 year olds and 50% of 80
year olds will continue to have infiltrates at
12 weeks
 Multilobar pneumonias are slower to
resolve
Pneumovax

Persons older than the age of 50
 Persons with asplenia, high risk
environments, immunosuppression and
chronic illness.
 Give vaccine if status is unknown
 Repeat vaccination should be considered for
patients at high risk and those vaccinated
before 65 if not given with in 5 years.
TB
For Another Time…

Extra-Pulmonary Tuberculosis
– Lungs> Kidneys> Bone> Brain

Mycobacterium Other Than Tuberculosis
(MOTT) AKA Non-Tuberculous
Mycobacteria (NTM)
AKA
Phthisis- “wasting”; chronic pulmonary
tuberculosis
 “Consumption”
 “White Plague”

Nomenclature

Phthisiology- the study of tuberculosis of the
lungs
 Converter- patient who has experienced an
increase of > 10mm of induration in PPD test size
within a two-year period, regardless of age.
 Reactor- a non-converter patient with a positive
skin test.
 LTBI- latent tuberculosis infection. “Treatment of
Latent Tuberculosis Infection” has replaced
“preventative therapy” and “chemoprophylaxis”.
Epidemiology
An estimated 1/3 of the world’s population is
believed to be infected with
M. Tuberculosis (roughly 2 billion people).
 Because actual reporting is generally unreliable,
several surrogate values have been used,
including:

–
–
–
–
Average annual risk of MTB infection (ARTI)
Estimated incidence of smear positive MTB
Case Notifications
Estimated Case-Fatality rates.
Re-Emerging Scourge?

Although MTB experienced a resurgence in
the mid-late 80’s, the incidence has actually
continued to decline since 1993 and, as of
2005 was at an all-time low…
Location, Location, Location

In 1998 active TB reported in every state.
 Seven states (CA, FL, GA, IL, NJ, NY and
TX) accounted for 60% of all cases.
 40% of all cases in America’s 64 largest
cities.
A Disease of Import

Incidence of MTB among Foreign-born
persons varies by country of origin:
– Latin America (57% from Mexico)
– Philippines
– Vietnam
– South Korea
– China
HIV: TB’s Not-So Silent
Partner
HIV’s effect on cell-mediated immunity
uniquely positions it as a dominant factor in
susceptibility to MTB in SE Asia and SubSaharan Africa.
 Rates of co-infection have increased as high
as 45-fold between 1990 and 1994
(Thailand)

Where the Money is…

Active MTB cases: 1990-1999
– North America: 320,000
– Sub-Saharan Africa: 15,000,000
– Asia and the Sub-Continent:
55,000,000
High-Risk Groups: Infection







HIV+
Foreign-born in endemic regions (4-6X)
Children of foreign-born from endemic regions
Homeless
Veterans
IV Drug users
Congregate living- Nursing homes, prisons, etc.
High-Risk Groups: Infection
con’t

Close contacts of individuals known, or
suspected, to have active MTB.
 Health care workers (i.e. you)
High Risk Groups: Disease






HIV+ (100X)
Diabetes Mellitus (3X)
Post-Gastrectomy or Intestinal Bypass
Silicosis (30X)
Certain Cancers- Leukemia, Lymphomas,
HEENT(16X)
Pharmacologically Immunosuppressed
– Post-chemotherapy, DMARD, Steroids


Post solid-organ transplant (20-74X)
Recent infection (within 2 years)
High-Risk Groups: Disease
con’t

CXR consistent with prior disease without
adequate treatment.
 ESRD (10-25X)
 Chronic malabsorption syndromes
 Low Body Weight (<10% below ideal) (2X)
Clinical Pathogenesis

Although one organism per 12,000 cu ft has been
shown to produce infection, only up to 1/3 of
individuals in close contact with patient with
active MTB develop infection.
 Most infected aerosolized droplets are cleared by
upper airway mechanisms.
 Those less than 5 microns reach the alveolus, and
are phagocytosed by macrophages (MTB
infection).

Bacilli multiply at this primary site of infection
and within 2 weeks are transported to lymphatics
to establish secondary site.
 Within 4 weeks delayed-type hypersensitivity
develops leading to granuloma formation and
subsequent decrease in bacterial burden.
 However, sterilization rarely occurs, even though
host displays acquired immunity, rapidly clearing
subsequent exposures.

THEORETICALLY, as the host begins to
control MTB through the primary response,
the normally aerophilic bacilli downshifts
into a non-replicating stage as surrounding
oxygen tension drops.
 This non-replicating, or latent, stage allows
organisms to avoid the anti-microbial
effects of MTB regimens.

The goal of treating latent tuberculosis
infection (LTBI) is prolonged courses of
therapy so effective drug levels are
persistent for months as latent organisms
reactivate, rendering themselves
susceptible.
By the Numbers…

Once infected with MTB 3-5% of
immunocompetent hosts develop active
disease within one year. A further 3-5% will
develop active disease within their lifetime.
 Said another way: Once primarily infected,
lifetime risk of active MTB is 10%; half of
those within the following year.
More Numbers

For immunosuppressed, risk of developing
active MTB is 7-10% annually.
Diagnosis: Symptoms

Pulmonary
– Prolonged productive cough
– Hemoptysis
– Chest pain

Systemic
– Fevers/Chills
– Drenching Night Sweats
– Anorexia/Weight loss
– Easy Fatigability
Diagnosis: H&P

After considering diagnosis of MTB (and
putting a mask on you or patient), focus
history on risk factors for MTB infection or
disease (see previous) as well as past
exposure and treatment history.
 Don’t forget to perform adequate review of
systems as only 73% of pulmonary MTB
cases are exclusively pulmonary.
Diagnosis: The Laboratory

The gold standard for diagnosis, the sputum
smear, is neither sensitive nor specific.
– >10,000 organisms/ml are required for
detection, leaving the smear positive in only
50% of active MTB cases
– Any acid-fast bacilli present will cause the test
to be positive.
The Sputum

Early AM specimens on three consecutive
days are ideal. Induced sputum with inhaled
saline may be required in patients unable to
provide adequate lower airway samples.
 Alternatives
– Bronchoscopy (choose wisely)
– Early AM Gastric aspiration
Sensitivities

All initially positive MTB cultures must be
tested for sensitivities to guide antimicrobiologic treatment and identify MultiDrug Resistant-TB (MDRTB).
 Sensitivities should be repeated if patient
experiences clinical treatment failure or
cultures remain positive despite two months
of treatment.
Purified Protein Derivative

First recognized as potential tool for
diagnosis by Sir Arthur Conan Doyle.
 Later perfected by Mantoux.
 Though fairly accurate for diagnosing
infection (not disease), limited by false
positive rate around 10% and false negative
rates around 20-30% (higher in
immunocompromised).
PPD con’t

A PPD may take up to 10 weeks to turn
positive after initial infection.
 The PPD is the only way to diagnose MTB
infection prior to MTB disease.
– Role of Interferon-Gamma…
The PPD Itself

0.1 ml of PPD tuberculin containing 5 TU is
injected intradermally on the inner surface
of the forearm producing a 6-10 mm wheal.
 A reading 48-72 hours from PPD placement
should be obtained:
– Positive readings may be obtained up to one
week after placement.
– Failure to obtain reading within 72 hours
indicates need for repeat testing
More PPD

The area of induration (not erythema) is
measured in millimeters perpendicular to
the long-axis of the forearm.
 “Conversion”- defined as an increase of
>10mm within a 2-year period.
A Positive PPD

>5 mm
– HIV+
– Recent contacts of active MTB case
– CXR consistent with healed MTB
– Patients with organ transplants or
immunosuppression > 15mg/day of prednisone
> 1 month

>10 mm
– Recent arrivals (<5 years) from endemic regions.
– IVDU
– Residents/Employees of:



Prisons/jails
Nursing home/shelters
Hospitals, including mycobacterial labs
– High-Risk of Progressing to MTB disease
– Children <4 yo

> 15mm
– No known risk factors for MTB.
PPD: False Positives

MOTT
 BCG vaccination
PPD: False Negatives

Recent MTB infection
 Very Young (< 6mos)
 Live-Virus vaccination
 Overwhelming MTB disease
 HIV+ or other viral infection
 Immunosuppressive Therapy
BCG

Bottom line: prior vaccination with BCG
should be ignored and the patient treated
appropriately if the PPD is positive.
Boost Effect?

Delayed-Type Hypersensitivity may fade
over time, resulting in subsequent negative
testing in those previously infected. This
exposure to tuberculin may “re-awaken”
sensitivity and lead to potential
misinterpretation of future positive testing
as a new infection.
Two-Step Testing
This “Boost” phenomenon, and subsequent
misinterpretation, may be sidestepped by
performing two-step testing.
 This entails a second test 1-3 weeks after
the first with positive tests indicating past
infection and treated appropriately

Who Not to Test

Pregnant women without specific high-risk.
 Previously positive PPD patients should not
receive repeat testing (including yearly
CXR’s); instead, these patients should be
followed symptomatically.
Other Testing

Some populations may require screening for
active MTB disease which is more
appropriately performed by CXR.
– Prisoners
– New accessions to congregate living
– POW’s/Detainees
Targeted Testing

Screening should be limited to previously
described high-risk groups.
 Prior to testing a follow-up plan for further
testing and treatment must be considered:
– “The decision to test is the decision to treat.”
The Chest Radiograph

Though traditionally relied upon to assist in
making diagnosis, HIV and other forms of
immunosuppression have impacted its utility.
– Old-school:


Primary: Middle or lower lung field infiltrates with ipsilateral
lymphadenopathy.
Reactivation: Upper-lobe infiltrates and cavities in 98% of
non-AIDS cases.
– Predilection for apical and posterior segments of upper lobe and
superior segments of lower lobes
CXR con’t
– New: Up to 35% of AIDS patients with active
MTB may have clear CXR; frequent findings
include lymphadenopathy or effusion alone.
Old Granulomatous Disease
(OGD)

Common radiographic term used to describe
stigmata of prior infection, frequently MTB.
– Implies dense, smaller nodules without or
without visible calcification or fibrotic scarring
typically seen in the upper lobes.
– Bronchiectasis, volume loss or pleural scarring
may accompany OGD.
Diagnosis: Lab

Smear/Cultures
– AFB+/MTB culture growth from sputum,
pleural fluid or pleural biopsy

Pathology
– Demonstration of caseating granulomas in
pleural tissue.

Pleural Fluid Analysis
Diagnosis: Lab
Pleural Fluid

Exudative with higher total protein levels (esp.>
5.0g/dl) adding to specificity.
 Lymphocyte pre-dominance (though early
effusions may be neutrophil pre-dominant).
 > 10% eosinophils virtually excludes MTB as
diagnosis (unless prior thoracentesis or PTX).
 Likewise, the presence of > 5% mesothelial cells
makes MTB less likely (does not apply to HIV+
individuals).
Pleural Fluid: ADA

Adenosine Deaminase levels may correspond
directly with likelihood of MTB infection.
– < 40 U/L- unlikely MTB
– 40-70 U/L- questionable
– > 70 U/L- likely MTB

Other clinical conditions (RA and empyema) may
have elevated ADA levels, but should be easy to
clinically differentiate.
Treatment

Several different nuances to treatment
modalities:
– LTBI
– Empiric (4 drug)
– Therapeutic
– MDRTB
Treatment of LTBI

Essential for any program aimed at reducing
future spread of MTB.
 MTB disease must be considered and ruled
out prior to treating LTBI.
Regimens

Isoniazid (INH) alone
 Rifampin/Pyrazinamide (PZA)
 Rifampin alone
 Known exposure to MDRTB
INH alone

Daily INH for 12 months reduces the risk of MTB
disease by 90%.
 6 months of therapy reduces risk by 70%.
 Two acceptable regimens:
– 300mg once daily
– 15mg/kg twice weekly (DOT) (900mg max)

Though a 6 month regimen is acceptable, 9
months of therapy is considered optimal in both
HIV+ and HIV- individuals.
Post-exposure to MDRTB

Regimens should consist of two drugs to
which the organism has demonstrated
susceptibility.
– PZA
– Ethambutol
– Quinalone
Monitoring

Though baseline laboratory testing is not indicated
at the start of treatment for LTBI, baseline liver
function tests should be obtained in those whose
initial evaluation suggests a liver disorder,
pregnant women, and HIV+ patients.
 Though advancing age increases risk of
hepatotoxicity, routine screening of LFT’s is not
recommended in the elderly.
Monitoring con’t

Monthly evaluations should address:
– Adherence to prescribed regimen.
– Signs and Symptoms of active MTB disease.
– Signs and Symptoms of hepatitis
– Other potential side effects from individual
regimens.
Empiric (4-drug) Therapy

Clinical Dogma
– Adherence must be ensured.
– Multiple drugs to which the organism is
susceptible must be used for prolonged periods.
– Never add a single drug to a failing regimen.
PIRES

4-drug therapy should be administered for
the initial 2 months of therapy; potential
agents include:
–
–
–
–
–
Pyrazinamide (PZA)
Isoniazid (INH)
Rifampin (RIF)
Ethambutol (EMB)
Streptomycin (SM)
More PIRES

Each agent plays a special role in the initial
2-month course of therapy.
– INH and RIF allow for short-course regimens
with high cure rates.
– PZA has potent sterilizing ability allowing for
shortening from 9 to 6 mos
– EMB (or SM) is added to prevent emergence
of further drug resistance if primary INH
resistance is possible.
Continuation of Treatment

As culture and sensitivity data return, regimens
may be tailored, recalling that at least two drugs
must be used at all times.
 Typical minimal length of therapy is six months:
– “4 drugs for 2 months, followed by 2 drugs for 4
months”
– If RIF is not used, 18 months of therapy is required.

INH therapy should be discontinued if:
– LFT’s > 3X nl and Pt is symptomatic
– LFT’s > 5X nl and Pt is asymptomatic
End of Therapy

A chest x-ray should be obtained to
establish baseline for future examinations.
 Sputum sample should be obtained at end of
therapy to document cure.
MTB Treatment: Pregnancy

The preferred initial treatment is INH, RIF
and EMB.
– SM has proven harmful fetal effects.
– PZA’s effect are unknown

Since PZA is excluded treatment must
continue for 9 months.
Multi-Drug Resistant TB
(MDRTB)

Definition: MTB resistant to both INH and
rifampin.
 Always treated with daily DOT therapy.
 XDRTB not likely to be tested…
MDR TB Cases, 1993 - 1998
MDRTB: High-Risk Groups

Prior treatment with MTB drugs.
 Contacts with known carriers of MDRTB
 Foreign-born persons from MDRTB
endemic regions
 Remains smear or culture positive despite 2
months of treatment
 Received inadequate therapy for > 2 weeks
 Cavitary Disease
Public Health

Assume infectiousness in persons known, or
suspected to have pulmonary or laryngeal
MTB if they are:
– Coughing or are “smear-positive” and
– Not receiving therapy, just started therapy or
have a poor response to therapy.

Mask on you or them and ISOLATE.
Public health con’t

Patients with drug-susceptible MTB disease
are no longer considered infectious if they
meet all of the following:
– On adequate therapy.
– Have experienced a clinical response to
therapy.
– Three consecutive negative sputum smears
from three different days.
Home Isolation

Isolation is not required to occur in a hospital. In
fact, an estimated 60% of all costs spent in the US
treating MTB are due to hospitalization.
 Must ensure that others in the home are not at high
risk for developing MTB disease and that simple
precautions (physical isolation, surgical masks,
etc) are followed until non-infectivity can be
assured.
Directly-Observed Therapy
(DOT)

Health Care Worker watches patient
swallow each and every dose of MTB meds
 Considered for patients with:
– Concerns for non-adherence.
– Intermittent dosing regimen.
– Household member on DOT for active MTB
disease.
Airway Disease

Asthma
 COPD
 Bronchiectasis
– CF
– ABPA
Definition

Asthma is an inflammatory disorder
manifested by a clinical syndrome of
episodic dyspnea, wheeze, and cough with
reversible airflow obstruction and bronchial
hyper-responsiveness.
Initial Assessment and
Diagnosis of Asthma

Determine that:
– Patient has history or presence of episodic
symptoms of airflow obstruction
– Airflow obstruction is at least partially
reversible
– Alternative diagnoses are excluded
Initial Assessment and
Diagnosis of Asthma (continued)
Is airflow obstruction at least partially
reversible?

Use spirometry to establish airflow obstruction:
– FEV1 < 80% predicted;
– FEV1/FVC <65% or below the lower limit of normal

Use spirometry to establish reversibility:
– FEV1 increases >12% and at least 200 mL after using a short-
acting inhaled beta2-agonist
Bronchoprovocation Testing

Methacholine Challenge
 Exercise Induced Bronchospasm

Increased sensitivity
 Decreased specificity
 Very high negative predictive value
Methacholine Challenge

Increasing doses of methacholine given by
inhalation
 Repeated spirometry performed
 Decrement of FEV1 by 20% is diagnostic of
bronchial hyper-reactivity at dose < 4 mg/ml.
– 4-16 mg/ml is considered by most to be borderline
– Clinical interpretation in requires correlation with
symptoms.
Classification of Asthma Severity:
Clinical Features Before Treatment
Monitoring Symptoms

Symptom history should be based on
a short (2 to 4 weeks) recall period

Symptom history should include:
– Daytime asthma symptoms
– Nocturnal wakening as a result of
asthma symptoms
– Exercise-induced symptoms
– Exacerbations
Monitoring Lung Function:
Spirometry

Spirometry is recommended:
– At initial assessment
– After treatment has stabilized symptoms
– At least every 1 to 2 years
Monitoring Lung Function:
Peak Flow Monitoring (continued)
Patients should:

Measure peak flow on waking before taking
a bronchodilator

Use personal best

Be aware that a peak flow <80% of personal best indicates
a need for additional medication

Use the same peak flow meter over time
Monitoring Pharmacotherapy

Monitor:
– Patient adherence to regimen
– Inhaler technique
– Frequency of inhaled short-acting
beta2-agonist use
– Frequency of oral corticosteroid “burst” therapy
– Side effects of medications
Control of Factors
Contributing to Asthma Severity

Assess exposure and sensitivity to:



Inhalant allergens
Occupational exposures
Irritants:


Indoor air (including tobacco smoke)
Air pollution
Control Other Factors That
Can Influence Asthma Severity

Rhinitis
–

Sinusitis
–

Promote drainage; antibiotics for complicating
acute bacterial infection
Gastroesophageal reflux
–

Intranasal corticosteroids are most effective
Medications; no food before bedtime; elevate
head of bed
Influenza vaccine annually
Overview of
Asthma Medications

Daily: Long-Term Control
–
–
–
–
–
Corticosteroids (inhaled and systemic)
Cromolyn/nedocromil
Long-acting beta2-agonists
Methylxanthines
Leukotriene modifiers
Overview of
Asthma Medications (continued)

As-needed: Quick Relief
–
–
–
Short-acting beta2-agonists
Anticholinergics
Systemic corticosteroids
Inhaled Corticosteroids
Most effective long-term-control therapy for
persistent asthma
 Small risk for adverse events at
recommended dosage
 Reduce potential for adverse events by:

–
–
–
–
Using spacer and rinsing mouth
Using lowest dose possible
Using in combination with long-acting
beta2-agonists
Monitoring growth in children
Inhaled Corticosteroids
(continued)

Benefit of daily use:
–
–
–
–
–
Fewer symptoms
Fewer severe exacerbations
Reduced use of quick-relief medicine
Improved lung function
Reduced airway inflammation
Long-Acting Beta2-Agonists

Not a substitute for anti-inflammatory therapy

Not appropriate for monotherapy

Beneficial when added to inhaled corticosteroids

Not for acute symptoms or exacerbations
Short-Acting Beta2-Agonists
Most effective medication for relief of
acute bronchospasm
 More than one canister per month suggests
inadequate asthma control
 Regularly scheduled use is not generally
recommended

Leukotriene Modifiers

Mechanisms
–
–

5-LO inhibitors
Cysteinyl leukotriene receptor antagonists
Indications
–
Long-term-control therapy in mild
persistent asthma



–
–
–
Improve lung function
Prevent need for short-acting beta2-agonists
Prevent exacerbations
Further experience and research needed
Do not replace inhaled corticosteroids
Not for monotherapy
Stepwise Approach to
Therapy: Gaining Control
1. Start high and
step down.
STEP 4
Severe Persistent
STEP 3
Moderate Persistent
1
STEP 2
Mild Persistent
STEP 1
Mild Intermittent
2
2. Start at initial
level of severity;
gradually step
up.
Indicators of Poor
Asthma Control

Step up therapy if patient:
– Awakens at night with symptoms
– Has an urgent care visit
– Has increased need for short-acting
inhaled beta2-agonists
– Uses more than one canister of short-acting
beta2-agonist in 1 month
Indicators of Poor
Asthma Control (continued)

Before increasing medications, check:
– Inhaler technique
– Adherence to prescribed regimen
– Environmental changes
– Also consider alternative diagnoses
Managing Exercise-Induced
Bronchospasm (EIB)



Anticipate EIB in all patients
Teachers and coaches need to be notified
Diagnosis
–
History of cough, shortness of breath, chest pain or
tightness, wheezing, or endurance problems
during exercise
–
Conduct exercise challenge OR have patient
undertake task that provoked the symptoms
–
15% decrease in PEF or FEV1 is compatible with EIB
Managing Exercise-Induced
Bronchospasm (EIB) (continued)

Management Strategies
–
–
–
–
–
Short-acting inhaled beta2-agonists used shortly
before exercise last 2 to 3 hours
Salmeterol may prevent EIB for 10 to 12 hours
Cromolyn and nedcromil are also acceptable
A lengthy warmup period before exercise may
preclude medications for patients who can
tolerate it
Long-term-control therapy, if appropriate
Management of
Asthma Exacerbations

Inhaled beta2-agonist to provide prompt
relief of airflow obstruction

Systemic corticosteroids to suppress and
reverse airway inflammation
–
For moderate-to-severe exacerbations, or
–
For patients who fail to respond promptly and
completely to an inhaled beta2-agonist
Risk Factors for
Death From Asthma
Past history of sudden severe exacerbations
 Prior intubation or admission to ICU
for asthma
 Two or more hospitalizations for asthma
in the past year
 Three or more ED visits for asthma
in the past year

Risk Factors for
Death From Asthma (continued)

Hospitalization or an ED visit for asthma
in the past month

Use of >2 canisters per month of inhaled
short-acting beta2-agonist

Current use of systemic corticosteroids
or recent withdrawal from systemic
corticosteroids
Risk Factors for
Death From Asthma (continued)
Difficulty perceiving airflow obstruction
or its severity
 Co-morbidity, as from cardiovascular
diseases or chronic obstructive pulmonary
disease
 Serious psychiatric disease or psychosocial
problems

Risk Factors for
Death From Asthma (continued)

Low socioeconomic status and
urban residence

Illicit drug use

Sensitivity to Alternaria
Aspirin Sensitive Asthma

Sampter’s Triad
– Asthma
– ASA sensitivity
– Nasal polyps

Difficult to control
 Probable role for leukotriene modifiers
 Role for ASA desensitization
Difficult to Control Asthma

Consider alternative or complicating
diagnoses
– Rhinitis, Sinusitis, GERD
– Allergic Bronchopulmonary Aspergillosis
– Churg-Strauss Vasculitis
– Vocal Cord Dysfunction
– Non-pulmonary Disorders
COPD- Epidemiology
Rising Mortality rates now place COPD 4th in US
 Tobacco use responsible for 85-90% of cases

– Alpha-1 AT def < 1%

~20% of smokers develop COPD
– ~20% of COPDer’s develop CO2 retention

FEV1 < 0.75L Mortality
– 1 year = 30%
– 10 year = 95%
COPD

Emphysema
– Centrilobular
 Tobacco Related
– Panacinar
 Alpha-1 AT def

Chronic Bronchitis
– ‘Two tablespoons of sputum daily for three
months of any 2 year period’
Emphysema- Dx

Suspected in patients with sig tobacco
exposure and sx’s
– Dx: SPIROMETRY!

DLCO reduction with emphysema vice CB
 Imaging reveals bullous lung disease
– Only severe disease on CXR
Emphysema- Dx

Alpha1- AT def- Suspect in patients with
sx’s and age < 50.
– May have no tobacco exposure, but smoking
will accelerate sx presentation
– May have family hx of lung or liver
involvement
– ‘Basilar Predominance to Bullous lung disease’
Emphysema- Tx
‘Tobacco Cessation and Oxygen’
 Symptom relief

–
–
–
–
Bronchodilators
Anti-inflammatories
Methylxanthines
Oral steroids as tx of last resort

Pulmonary Rehabilitation
 Surgery
– Bullectomy- Bullae > 1/3 hemithorax
– LVRS- Upper lobe predom with low exercise cap
– Transplant-
Emphysema- Tx
Acute Exacerbations

Pt’s with 2/3 ‘Winnipeg Criteria’ deserve
Abx
– Increased SOB
– Increased Sputum volume
– Sputum purulence

Trump card = Hospitalization
 Any abx will do…
‘Indications for O2’

Resting Sp02 < 88%
 Resting Pa02 < 55mmHg
 Resting Pa02 56 to 59 mmHg if
– Cor pulmonale (including peripheral edema)
– Polycythemia

Remember to re-eval for O2 requirements
~4-6 weeks after acute exacerbation.
Emphysema- Tx
Acute Exacerbations

Steroids
– Oral = IV (equivalent to 40-60mg prednisone)
– Rapidly tapered over 2 weeks

Aggressive BD tx’s
‘Bronchiectasis’ Defined

Bronchial dilatation frequently associated
with:
– Bronchial wall thickening
– Fluid retention within the bronchi
– Chronic inflammation/infection

Can be localized or diffuse
Bronchiectasis
Restrictive Lung Diseases

Pleural Disease
 Alveolar Diseases
 Interstitial Diseases
 Neuromuscular Diseases
 Thoracic Cage Diseases
Parenchymal Disease

Interstitial Lung Disease
–
–
–
–

Idiopathic Interstitial PNA’s
Sarcoidosis
Collagen Vascular Disorders
Eosinophilic Lung Diseases
Alveolar Lung Disease
– Pulmonary Edema Syndromes
– Pulmonary Alveolar Proteinosis
Nomenclature

Pneumonia vs Pneumonitis
 Alphabet Soups
– COP, BOOP, BO, BOS, OB, CB

Name Game
– That was then, this is now…

Disease’s Clinical name vs Histologic name
New Names

Hypersensitivity Pneumonitis
– Extrinsic Allergic Alveolitis (EAA)

Eosinophilic Granuloma or Histiocytosis X
– Langerhan’s Cell Histiocytosis (LCH)

Idiopathic Bronchiolitis Obliterans
Organizing Pneumonia
– Cryptogenic Organizing Pneumonia (COP)
An Alphabet Soup of New
Names

Idiopathic Interstitial Pneumonias (IIP’s)
– Idiopathic Pulmonary Fibrosis (IPF)
– Non-Specific Interstitial Pneumonia (NSIP)
– Desquamative Interstitial Pneumonia (DIP)
– Respiratory Bronchiolitis Interstitial Lung
Disease (RBILD)
– Cryptogenic Organizing Pneumonia (COP)
– Acute Interstitial Pneumonia (AIP)
Clinical Name vs
Histologic Name

Same entity may be referred to by different
names by different specialists or in different
circumstances.
– Idiopathic Pulmonary Fibrosis -Clinical
 Usual Interstitial Pneumonia (UIP)- Histologic
Blood,Pus, Water…

Blood
 Mineral
 Water
 Cells
 Proteins
Blood

Diffuse Alveolar Hemorrhage Syndromes
 Hemosiderosis
Minerals

Calcium
– Pulmonary Alveolar Microlithiasis
– Metastatic Calcinosis

Pneumoconioses
–
–
–
–
Asbestos
Silicosis
Talc
Coal Worker’s Pneumoconiosis
Water

Cardiogenic Pulmonary Edema
 ARDS
 Radiation Toxicity
Cells

Lymphocytes
– LIP, most Collagen Vascular Dz assoc. ILD

Eosinophils
– Eosinophilic Pneumonias, ABPA, CSS

Multi-Nucleated Giant Cells (Granuloma)
– Sarcoid/Berylliosis, EAA

Histiocytes
– Langerhan’s Cell Histiocytosis

Malignant
– Lymphangitic CA, BAC
Protein

Amyloidosis
 Gaucher’s Disease
 Pulmonary Alveolar Proteinosis
When to Think “Interstitial
Lung Disease?”

Dyspnea evaluation
– +/- Hypoxemia

Chronic Cough
 Refractory “CHF”
 Abnormal radiograph
– Exceptions: Stage 0/1 Sarcoid, EAA, DIP/RBILD

Abnormal PFT’s
– Isolated DLCO defect
Epidemiology

Previously felt to occur in 5 per 100,000
 Recently estimated to occur in 31.5 per
100,000 males in US (by death certificate
diagnosis of ILD).
– 26.1/100,000 females

Most common ILD is “Idiopathic
Pulmonary Fibrosis” accounting for up to
45% of ILD diagnoses.
Epidemiology Con’t

Accounts for approx. 100,000 hospital
admissions annually in US.
 Represents approx. 15% of office visits to
pulmonologists.
 Expanding populations of patients at risk
for ILD ( AIDS, post-chemotherapy, etc.) is
likely to lead to higher incidence of these
diseases.
Idiopathic Pulmonary Fibrosis

Prevalence: 13–20/100,000 in US (approximately
35,000-55,000 cases)
 Onset: Usually between 50 and 70 yr
 Clinical presentation
–
–
–
–
–
Progressive dyspnea on exertion
Paroxysmal cough, usually nonproductive
Abnormal breath sounds on chest auscultation
Abnormal chest x-ray or HRCT
Restrictive pulmonary physiology with reduced lung
volumes and DLCO and widened AaPO2
Coultas DB et al. Am J Respir Crit Care Med. 1994;150:967.
ATS/ERS. Am J Respir Crit Care Med. 2000;161:646.
DIAGNOSIS OF IPF

Major criteria
–
–
–
–

Exclusion of other known causes of ILD
Abnormal pulmonary function studies
Bibasilar reticular abnormalities on HRCT scan
No histologic or cytologic features on transbronchial
lung biopsy or BAL analysis supporting another
diagnosis
Minor criteria
– Age >50 yr
– Insidious onset of otherwise unexplained exertional
dyspnea
– Duration of illness 3 mo
– Bibasilar, dry (“Velcro”) inspiratory crackles
Sarcoidosis
“Sarcoidosis is a multi-system disorder of
unknown cause…” characterized by
“…histological evidence of noncaseating
epithelioid cell granulomas. Granulomas of
known causes and local sarcoid reactions must be
excluded.” ATS Statement on Sarcoidosis. AJRCCM 1999.
 First described in 1877 by Hutchinson
 Boeck coined the term “sarkoid” in 1899
 Organs that may be involved:

– Lung, skin, eyes, liver, spleen, lymph nodes, salivary
glands, heart, nervous system, muscles, bones, kidneys,
joints, stomach
Epidemiology

Most commonly affects < 40 years old
 Slightly higher disease rates in women
 Swedes, Danes, and US AA’s highest prevalence
rates
– Lifetime risk US white: .85%, US black: 2.4%

Significant variability in disease presentation and
severity among different groups
– More severe disease in AA’s, caucasians with asx
disease
– EN in Europeans
– Cardiac and ocular more common in Japan
Skin


Approx 20% of patients
Most common subacute finding => maculopapular
eruption
– Nares, lips, eyelids, neck, previous trauma (‘scars and tatoos’)

Erythema Nodosum
–
–
–
–
–

Hallmark of acute sarcoidosis
Red, raised, tender nodules on anterior legs
Adjacent joints may be painful, swollen
Lasts 6-8wks and rarely recurs
‘Lofgren’s syndrome – fever, arthralgia, EN, bilat hilar LAN’
Lupus pernio
– Marker of chronic sarcoidosis
– Indurated plaques located on cheeks, lips, nose, ears
– Prolonged course with rare spontaneous remission
Erythema nodosum
Lupus
pernio
Pulmonary

>90% of patients
 1/3-1/2 describe dyspnea, dry cough, vague
chest tightness
 <20% with “crackles”, clubbing is rare
 Rare findings – effusion, chylothorax, PTX,
cavitary lesions, calcified LAN
 5 stages based on CXR findings
– Stage 0 – no intrathoracic findings
Stage 1
Bilateral hilar adenopathy – 50% of patients
 60-80% spontaneous remission

Stage 2

Bilateral hilar adenopathy with parenchymal
infiltrate – 25% of patients
 50-60% spontaneous remission
Stage 3

Parenchymal infiltrate without hilar LAN
 <30% spontaneous remission
Stage 4

Advanced fibrosis
– Honey-combing, hilar retraction, bullae, cysts,
emphysema
Diagnostic evaluation

Diagnosis:
– Compatible clinical picture
– Compatible histology
– Exclusion of other causes

Goals of work-up:
1 – histologic confirmation
2 – assess extent and severity of organ involvement
3 – assess disease stability and whether will progress
4 – determine if therapy will benefit patient
Diagnostic evaluation

Biopsy
– Lymph node
– Skin lesions
– Bronchoscopy
 40- 90+% yield
– Mediastinoscopy
– VATS
– Open lung bx
Natural history

Highly variable and influenced by race and genetic factors
– 4-7% present with serious extrapulm involvement

Spontaneous remission in 2/3
– >85% within 2yrs
– If remission or stabilization, 2-8% relapse

Chronic or progressive in 10-30%
– Failure to remit in 24 mos


Mortality 1-5%
Poor prognostic indicators
-
Lupus pernio
AA race
age onset >40
neurosarcoid
progressive pulm dz
nasal mucosal involvement
- chronic high Ca
- nephrocalcinosis
- chronic uveitis
- cardiac involvement
- cystic bone lesions
Treatment

Controversial and unclear
– Large number of spont remissions or benign
clinical course

Stg 1 – 60-80%, 2 – 50-60%, 3 – 30%
– No good method to assess activity
– Variability in presentation and course not
amenable to developing guidelines
– Cause is unknown, no specific tx
Treatment

Steroids first line therapy
 Topical therapy
– Uveitis, skin, cough

Systemic therapy
– Definite indications- heart, neuro, ocular not
responding, hyperCa
Treatment of pulmonary
sarcoid

Observation
– Asx or mild sx stage 1, 2, 3
– Eval q6mth stage 1, q3mth stage 2, 3
– Tx if worsening sx’s, deteriorating lung fxn, progressive xray
findings

Treatment
– .5 – 1mg/kg for 4-6 wks
– If response, taper by 5-10mg every 4-8wks to maintenance of 5-
10mg/d
– If no response after 3mths then taper off steroids
– Total duration of therapy 12mths then taper off
– Relapse rate high – 60-90%
Surveillance

If no treatment, most intense in first 2 yrs
then annual
 If treated, most intense in first 3 yrs after
discontinuation of therapy then “at least
annually”
Pulmonary Edema Syndromes

Cardiogenic
– High-Output Failure
– Low-Output Failure
 Systolic Dysfunction
 Diastolic Dysfunction

Noncardiogenic
– Pulmonary
– Non-pulmonary
Pulmonary Etiologies

PNA
 Aspiration
 Acute Interstitial Pneumonia (HammanRich)
 Acute Eosinophilic Pneumonia
 Diffuse Alveolar Hemorrhage Syndrome
Non-Pulmonary Etiologies

‘Surgical’
– Burns
– Trauma
– Surgical SIRS




Re-perfusion PE
Negative Pressure PE
Narcotic-Related PE
General Anesthesia PE

‘Medical’
– MICU
 TRALI
 Pancreatitis
 Sepsis
 Neurogenic
– Emboli
 Fat
 Amniotic
 Air
– Other
 HAPE
 SIPE
 Drugs
Fat Emboli

Patients:
– ‘Post-Trauma, esp. long bone fracture’
– Sickle Cell patients, exp post-partum

Signs
–
–
–
–
Central Nervous System
Renal Failure
Acute Lung Injury
Petechiae
Eosinophilic Lung Diseases

Pulmonary Infiltrates w/Eosinophilia (PIE)
– Primary



Acute vs. Chronic Eosinophilic PNA
Simple Pulmonary Eosinophilia (Loeffler’s)
Idiopathic Hypereosinophilic Syndrome
– Secondary





ILD’s
‘Asthma syndromes’
Malignancies
Infections
Drugs
Acute vs. Chronic Eosinophilic
PNA

Acute

Chronic
– Presents acutely like
– Presents subacutely
PNA/ARDS
– CXR mimics
PNA/ARDS
– Peripheral Eosinophilia
rare; High count on
BAL
– Prompt, lasting
response to steroids
with Mild Hypoxemia
– CXR- ‘Photographic
negative of CHF’
– Peripheral Eosinophilia
common; High count
on BAL
– Prompt response to
steroids, but recurrence
common
Simple Pulmonary
Eosinophilia (Loeffler’s)

Migratory Pulmonary Infiltrates
 Eosinophilia
 Cause:
– Ascaris Lumbricoides
ILD’s

Sarcoidosis
 Langerhans Cell Histiocytosis
 Idiopathic Pulmonary Fibrosis
 Collagen Vascular Diseases
 Bronchioloitis Obliterans Organizing
Pneumonia (BOOP/COP)
‘Asthma syndromes’

Allergic Angiitis & Granulomatosis
 Allergic Bronchopulmonary Mycosis
 Allergic Reaction
Allergic Angiitis &
Granulomatosis

AKA Churg-Strauss Syndrome
– Diagnostic Criteria (4/6)






Migratory Pulmonary Infiltrates (‘fleeting’)
Eosinophilia (>10%)
Peripheral Neuropathy
Sinus disease
Asthma
Biopsy findings
– Extravascular eosinophils
– Granuloamtous Angiitis
– Extravascular Necrotizing Granulomas
Allergic Bronchopulmonary
Mycosis

Diagnostic Criteria
– Asthma
– + skin test to fungus
– + IgG precipitins
– + IgE precipitins
– Elevated IgE
– Central BTX
– Eosinophilia with CXR
ASO’s
‘Central or Proximal
Bronchiectasis’
 ‘Coughs up brown,
plugs’
 ‘Finger-in-gloves-’ Xray

Malignancies

NHL
 NSCLCA
 Leukemias
Infections

Parasites
 PCP
 Mycobacteria
 Fungal
– Especially Cocci
Other ILD’s

Wegener’s
– ‘ELK’
– ‘c-ANCA’

Goodpasture’s
– Bleeding Kidneys and lungs
– ‘Anti-GBM ab’

Langerhans Cell Histiocytosis
– ‘Young, male smokers with recurrent PTX’

LAM
– ‘Young, female non-smokers with recurrent PTX’
‘Drugs and Lung Disease’

ARA-c = ARDS
 Bleomycin = ARDS/Fibrosis worsened with
oxygen exposure
 Amiodarone = Pulm fibrosis, typically
dose-dependant
 Hydralazine/Procainamide = SLE-like
 Crack-lung = hemorrhage, ARDS, vaculitis
Pulmonary Vascular Disease

Venous Thromboembolic Disease (VTE)
– DVT
– PE
– CTEPH

Pulmonary Hypertension (PH)
DVT

Etiologies
 Diagnosis
 Treatment
DVT: Etiologies

Trauma
 Recent Surgery
 Medical Immobility
 Medications- e.g. OCP’s
 Medical conditions- Behcet’s, IBD, SCD,
CA, Nephrotic syn
 Hypercoaguable States
Hypercoaguable States

APC resistance (Factor V Leiden)
 APLS
 Protein C def
 Protein S def
 AT III def
 Homocystenemia
 Prothrombin Gene mutation
Diagnosis

Duplex compression U/S
– Role of serial testing in low-risk setting

Venography
 CT
Treatment

Anticoagulation
– UFH vs LMWH
 Look for HIT/HAT
– Warfarin

Vena Caval Filter
 Stockings
PE
Total Incidence
630,000
11%
Death within
1 hr
67,000
89%
Survival
> 1 hr
563,000
71%
Diagnosis not
made
400,000
70%
Survival
280,000
30%
Death
120,000
29%
Diagnosis made,
therapy instituted
163,000
92%
Survival
150,000
8%
Death
13,000
Pathophysiology of Acute PE

Related to reduction in cross-sectional area of
pulmonary vasculature
 increase in PVR=> impedance of RV ejection of
blood => decreased filling of LV
Predicting the Severity of Pulmonary Embolism
PE Severity
Mild
Mod or Submassive
Massive
PA
obstruction
<50
50-75
>75
PA mean
pressure
<20
25-40
40-45
RA mean
pressure
<10
<10
>10
CI
>2.5
>2.5
<2.5
Acute PE (cont.)

Thrombolysis or embolectomy
 massive PE with hemodynamic instability or
refractory hypoxemia
Heparin/Lovenox
 Oral anticoagulation
 IVC filter

 patients with contraindication to anticoagulation
 recurrent embolism despite anticoagulation
 unable to tolerate further emboli
Pulmonary Hypertension
Symptoms and Signs

Dyspnea on exertion in a young female

Fatigue

Shortness of Breath and Chronic Hypoxia

About 10% of primary and >50% of
secondary P-HTN patients have Raynaud's
phenomenon
Symptoms and Signs

Chest pain
– Secondary to RV ischemia in the face of RV
hypertrophy and increased sys and dias
pressures

Syncope
– Usually exertional or post exertional implies a
severely restricted CO with deminished
cerebral blood flow
Symptoms and Signs

Symptoms of right sided heart failure
– Peripheral edema and hepatic congestion
(abdominal pain)
– Distended neck veins and fluid retention
– Ineffective filling of Left ventricle, with
resultant hypotension
The Demise in Pulmonary
Hypertension

Progressive RV failure leads to dyspnea,
hypoxemia, and progressive decrease in CO
 This leads to death from RV failure or fatal
dysrhythmias
 Arterial hypoxemia and acidosis predispose
to fatal dysrhythmias
The Demise in Primary
Pulmonary Hypertension

Common causes of death include:
– Brady and tachy dysrhythmias
– PE
– Massive pulmonary hemorrhage
– Sudden RV ischemia / infarction
Diagnosis

Clinical Definition
 Presence of pulmonary HTN: mean PA
pressure >25mm Hg at rest (or 30mm in
exercise)
– Normal pulmonary capillary wedge pressure
(PCWP)
– Absence of secondary etiology (for primary PHTN)
Diagnosis

Evaluate for secondary etiology
– Echocardiography
– Ventilation-Perfusion (V/Q) Scanning
– Pulmonary Angiography
– Autoantibody serologies
– Pulmonary Function Testing
– Note that many patients with primary P-HTN
have low titer autoantibodies
Diagnosis

CXR: Evidence of pulmonary hypertension
– Prominent main pulmonary artery
– Enlarged hilar and pulmonary vessels
– Enlarged R heart structures
– 6-10% of patients have a normal CXR
Diagnosis

EKG
– May show right axis deviation
– RVH
– R Heart strain pattern
– Peaked P waves in lead II
– Findings do not correlate with the severity of
disease
Diagnosis

Echocardiography
– Elevated pulmonary pressures
– May reveal RA and RV enlargement
– May reveal a normal to decreased LV chamber
size
– Loss of normal septal curvature and decreased
LV filling may reflect disease severity
Treatment

Overview of Vasodilator Therapy

In general, response to acute vasodilator
administration predicts chronic response

Chronic prostacyclin may be beneficial in absence
of acute response

Diltiazem combined with oxygen may have
synergistic chronic effects

Invasive monitoring a must during acute infusions in
patients
Treatment

Other Therapies
– Oxygen - usually improves function, acts as vasodilator
– Digoxin - may be useful in setting of atrial fibrillation,
improved inotropy
– Note that diltiazem may also reduce ventricular
response in atrial fibrillation
– Lung (± heart) transplantation may be only other
treatment at present
Treatment

Anticoagulation
– Patients with PPH are at increased risk for
intrapulmonary thrombosis and
thromboembolism due to:





Sluggish pulmonary blood flow
Dilated right heart chambers
Venous stasis
Sedentary lifestyle
Increased risk of atrial fibrillation
Internship
Pleural Disease

Pleural Effusions
– Transudates
– Exudates

Pneumothorax (PTX)
‘Light’s Criteria’

Any one of the following qualifies the
effusion as exudative:
– Protein P/S > 0.5
– LDH P/S > 0.6
– Pleural LDH > 200 or > 2/3 upper limits of
serum normal
Transudates

‘-Oses’
– Nephrosis- Nephrotic syn
– Cirrhosis
– Cardiosis- Pulmonary Venous HTN (CHF)

Other
– Hypo-Thyroid, PE, ATX, pericardial disease,
trapped lung, urinothorax, SVC syn
Exudates

Parapneumonic Effusions
 Malignancy
 Chylothorax
Lymphocyte Predominant
Exudates








Chylothorax
Rheumatoid
Yellow Nail
Sarcoid
TB
Acute Rejection
Lymphoma
Post CABG
Eosinophilic Exudates









Air
Benign Asbestos Pleural Effusion (BAPE)
Cancer, esp lymphomas
Drugs
Embolism
Fungus
Granulomatous Disease
Hemothorax
Infection (Parasite, TB?)
Parapneumonic Effusions

Predictors for Poor Outcomes (i.e. should
be drained)
– Low Glucose
– Low pH
– + gram stain
– + culture
– Complicated appearance (i.e. loculated) on CT
or US
Malignancy

Usually proven with cytology
 Poor prognostic indicator
– IIIb disease
– Average life expectancy ~ 6 mos

Low pH implies poor response to
pleurodesis
Chylothorax
‘Milky Appearance’
 Triglycerides > 110 or chylomicrons on
lipid analysis
 DDx- post-trauma > tumor > idiopathic
 High cholesterol implicates ‘pseudochylous
effusion’
 Do NOT drain repeatedly- leads to
immunocompromise and malnutrition

Pneumothorax

Spontaneous
 Secondary
– Traumatic
– COPD
– CF
– TB
– Diffuse Parenchymal Lung Disease
PTX: Treatment

ASX PTX < 15% of hemithorax = observe
 >15% = simple aspiration
– Failure of aspiration will require tube
thoracostomy.
– BPF’s may require large bore tube.

A 2nd spontaneous PTX deserves
pleurodesis
Sleep

Sleep-Disordered Breathing
– OSA
– Central Apneas

Narcolepsy
 Insomnia
OSA

Incidence- ~3% of all Americans, mostly
undiagnosed.
 Signs/Sx’s- Excessive daytime somnolence,
morning headaches, HTN, caffeine
dependence, snoring, witnessed apneas
 AHI > 5 arousals/hour
OSA: Treatment

CPAP
 Oral appliances
 Surgery
Central Apneas

‘Cheynes-Stokes is associated with CHF.’
Narcolepsy

EDS
 Cataplexy- sudden loss of tone in weightbearing muscles.
 Hypnagogic Hallucinations- visual/auditory
hallucinations occuring as patient falls
asleep.
 Sleep Paralysis- total paralysis while falling
asleep or waking up.
Narcolepsy: Dx

Multiple Sleep Latency Test- demonstrates
sleep onset in < 5 minutes and two episodes
of Rapid Onset REM sleep.
Narcolepsy: Tx

Avoid excessive sleep deprivation.
 Stimulants
– Amphetamines
– Modafinil
Potpourri

Lung Transplant
 Lung Cancer
 Perioperative Lung Eval
 PFT’s
 Hemoptysis
Lung Transplant

Lung Transplant may be considered for
end-stage Lung Disease.
– Post-Transplant Survival
 1 month- 88%
 1 year- 72%
 3 year- 56%
 5 year- 43%
Lung Cancer
Epidemiology





Estimated in 2004, 174,000 Americans will be
diagnosed
In 2003, approximately 157,200 deaths due to
lung cancer
Leading cause of cancer death in both men and
women in U.S.
Causes more deaths than colon, breast, and
prostate combined
5 year survival for all patients newly diagnosed is
15% (colon – 61%, breast – 86%, prostate – 96%)
Epidemiology
Rare disease beginning 20th century; sharp rise in
1930’s; by mid-century leading cancer death in
men
 Rise in women followed in 1960’s to present when
is now leading cancer death
 Similar rates among African-American and white
women, but 50% more frequent among AA males
 More common in developed countries

Epidemiology

Histologic subtype has shifted with
adenocarcinoma replacing squamous cell as most
common
 Median survival untreated metastatic non-small
cell 4-5 months (8 mths with state-of-the-art
treatment)
 5 year survival for potentially resectable disease:
–
–
–
–
–
IA – 67%
IB – 57%
IIA – 55%
IIB – 39%
IIIA – 23%
Cigarette Smoking

Leading cause of lung cancer and accounts for
approximately 90% of cases in U.S.
– Leading cause of preventable death (1 out of 5 deaths)
– ½ of regular smokers die prematurely of tobacco-related disease

First scientific report associating cigarette smoking with
increased risk of premature death in 1938
 Doll and Hill in 1950 demonstrated clear epidemiologic
evidence linking smoking and lung cancer
 Compared to never smokers, 20 fold increase in risk
Cigarette Smoking

Risk increases with duration and number of
cigarettes per day
– Models show duration of smoking even stronger risk
than number per day
– Those starting younger, most likely to develop cancer
and to do so at younger age

Risk decreased in those who quit compare to those
who continue
– As period of abstinence increases, risk decreases
– Never returns to level of risk of never smokers
Occupational/Environmental

Lung cancer attributed to occupational exposure
approximately 9-15%
 Asbestos
– Approximately 7 fold increased risk
– Acts synergistically with cigarettes to increase to 16 fold
– Dose dependent and fiber dependent

Radon
– Formed from breakdown of uranium
– Found in soil, groundwater, rock – can accumulate in homes


Ionizing radiation
Approx 1-2% attributed to atmospheric pollution
Histology

Adenocarcinoma
– Neoplastic gland formation or intracytoplasmic mucin
– Peripheral in 75% of cases

Squamous cell
– Proximal tracheobronchial tree 60-80% of cases
– Can demonstrate central necrosis with cavitation

Small cell
– Neuroendocrine features
– Commonly proximal airways with involvement of hilum and mediastinum

Large cell
– Diagnosis of exclusion
– Commonly peripheral
Signs/Symptoms Due to
Intrathoracic Spread

Due to lymphatic spread or direct extension
 Nerve involvement
– Recurrent laryngeal, phrenic, Pancoast tumor, Horner’s
syndrome

Chest wall and pleura
 Vascular invasion
– SVC syndrome

Visceral invasion
– Esophagus, heart and pericardium
Paraneoplastic Syndromes

Occur in approximately 10% of patients
 Unrelated to size of tumor
 Can sometimes precede the diagnosis of the
tumor
 Can mark the recurrence of malignancy
Paraneoplastic Syndromes
Endocrine Neurologic
Skeletal
Renal
Metabolic
SIADH
Hypercalcemia
Gynecomastia
Hypercalcitonemia
Elevated LH, FSH
Hypoglycemia
Hyperthyroidism
Carcinoid
Subacute Sensory Neurop
Mononeuritis multiplex
Intestinal Pseudo-obstruct
Lambert-Eaton Syndrome
Encephalomyelitis
Necrotising myelopathy
Cancer-asstd retinopathy
HOA
Clubbing
Glomerulonephritis
Nephrotic
Syndrome
Lactic acidosis
Hypouricemia
Systemic
Coll/Vasc
Skin
Heme
Coagulation
Anorexia
Cachexia
Fever
Dermatomyositis
Polymyositis
Vasculitis
SLE
E. Gyratum repens
E. Multiforme
Tylosis
Erythroderma
Exfoliative dermatitis
Acanthosis nigricans
Sweet Syndrome
Pruritis, urticaria
Hypertrichosis
languinosa
Anemia
Leukocytosis
Eosinophilia
Leukemoid rxn
Thrombocytosis
Thrombocytopenic
purpura
Thrombophlebitis
DIC
Evaluating suitability for
surgery




FEV1 most commonly used parameter
If FEV1 > 2L for pneumonectomy or >1.5L for lobectomy,
no further eval (low risk)
If suitable FEV1, but suspect ILD or patient with excessive
DOE, can use DLCO (more testing if <60%)
If patient does not meet above, then testing to evaluate
post-op FEV1 and DLCO
– Post-op predicted FEV1 or DLCO <40% indicates high risk

Can use exercise testing to further assist
– VO2 max >20ml/kg/min – low risk
– VO2 max <15ml/kg/min – increased risk of perioperative
complications
– <10ml/kg/min – very high risk
Perioperative Lung Eval
Effects of Surgery

Lung volumes
 Diaphragm function
 Gas exchange
 Control of breathing
 Lung defense mechanisms
Lung Volumes

Dependent on site of surgery
 Restrictive
 Reduction in vital capacity (VC) and
functional residual capacity (FRC) up to 70
and 50 percent respectively
FRC and CC

FRC = lung volume at end of normal
expiration
 CC = lung volume at which small airways
in bases begin to close during expiration
because of reduction in airway radial
traction
 Normally FRC > CC and airways remain
open throughout tidal breath
Diaphragm

Temporary dysfunction following thoracic
or upper abdominal surgery
Gas Exchange

Arterial hypoxemia
– First phase: initially post-op secondary to
residual anesthesia, shunting, V/Q mismatch
– Second phase: CC > FRC
Control of Breathing

Anesthetic agents inhibit respiratory drive
and reduce ventilatory response to
hypercapnia, hypoxia, and acidemia
 Narcotics decrease sighs and may
precipitate OSA
Pulmonary Complications





Incidence 5-90 percent
528 patients underwent elective abdominal
surgery
Pulmonary complications >> cardiac
complications
Pulmonary complications associated with longer
hospitalization
Healthy non-obese, non-smoker < 1 percent
J Gen Intern Med, 1995.
Pulmonary Complications

Atelectasis
 Infection (tracheobronchitis and
pneumonia)
 Prolonged mechanical ventilation or
respiratory failure
 Exacerbation of underlying pulmonary
disease
 Thromboembolic disease
Pre-operative Risk Factors







Chronic lung disease
Smoking
General health
Age
Obesity
Antecedent respiratory infection
Obstructive sleep apnea
Asthma

If under good control, minimal complications
 Tracheal intubation can initiate bronchospasm
 Consider regional anesthesia in severe asthma
 Consider stress dose steroids
 Treat acute bronchospasm
 Delay surgery if necessary
Smoking

200 consecutive CABG patients stratified by
smoking history
 Significant reduction in complications if patient
stops at least 8 weeks prior to surgery1
 Stop 12-18 hours pre-op to allow for sufficient
carboxyHB clearance
 Evidence for decreased HR, BP, and
cathecholamine levels within 60 minutes after
smoking
Mayo Clin Proc 1989.
Obesity

Common false assumption
 10 series of gastric bypass surgery found no
increased incidence of pneumonia or
atelectasis
 Meta-analysis of 6 studies (4526 patients)
demonstrated equal rate of complications in
obese and non-obese
Ann Intern Med 1986.
Post-operative Risk Factors

Inadequate post-operative analgesia
 Immobilization
Inadequate post-operative
analgesia

Pain inhibits coughing and deep breathing
 Pain discourages mobility
 Hesitancy to report pain
 Anxiety of prescribing narcotics
Immobilization

FRC decreases 500-1000cc when moving
from upright to supine position
 Increased risk of thromboembolic disease
 Ambulation is associated with clearance of
secretions
Pulmonary Function Tests

Not indicated for routine pre-operative
screening
 Indications:
– Persistent cough or unexplained dyspnea
– Hx of chronic lung disease
– Hx of smoking
– Planned lung resection
Incentive spirometer

Non-invasive
 Inexpensive
 Decreased atelectasis
 Decreased hospital stay
PFT’s
Indications






Detect presence or absence of lung dysfunction
suggested by history, physical or presence of other
abnormal tests.
Quantify severity of known lung disease.
Assess change in function over time or effect of
therapy.
Assess effects of environmental or occupational
exposure.
Pre-surgical evaluation.
Assess impairment or disability
Normal Flow-Volume Loop
Obstructive Flow-Volume
Loop
Obstructive FVL
Restrictive Flow-Volume Loop
Restrictive FVL
‘Fixed Airway Obstruction’
‘Dynamic Extrathoracic Airway
Obstruction’
‘Dynamic Intrathoracic Airway
Obstruction’
Determining “Normal”
Flows

The use of “80% of predicted” as a cutoff
between normal and abnormal is arbitrary.
– Extremes of age and height are frequently
mislabeled
– Lower limits of normal for flows
(e.g. ‘FEF25-75’) is closer to 50%.
Determining “Normal”
FEV1/FVC Ratio

This ratio is inversely related to age and
height; therefore, use of a fixed ratio (i.e.
80%) will result in increased labeling of
impairment in older patients.
 Athletes and workers in demanding
occupations frequently have
disproportionately high FVC compared to
FEV1.
Definition of An Obstructive
Defect

A disproportionate reduction of maximal
airflow from the lung with respect to the
maximal volume that can be displaced from
the lung.
– Low FEV1/ FVC.
‘Significant BD Response’

Many different definitions looking at
different flows, volumes or ratios.
 ATS:
– Increase in FVC or FEV1 of 12% from baseline
AND
- an absolute increase of 200ml.
Definition of a Restrictive
Defect

One may infer the presence of a restrictive
defect when VC is reduced and FEV1/FVC
is preserved, but…
– A restrictive defect is physiologically defined
by a reduction in TLC.

If a contradiction between TLC and VC
arises, defining restriction should be based
on TLC.
When to Obtain Lung
Volumes

To confirm and help stage the severity of
disease suspected by spirometry.
 To provide evidence of lung dysfunction
not clearly evident from spirometric tests.
 To trend the course of a disease or response
to therapy.
Lung Volumes in Restrictive
Lung Disease

Since Restrictive Lung Diseases are defined
by a decrease in VC and TLC,
measurements of lung volumes are most
helpful in detecting, confirming and staging
restrictive lung defects.
 Lung Volumes may suggest the physiologic
type of disease from the pattern of lung
volume alterations.
Restrictive Lung Disease:
Differential Diagnosis

Pleural- Effusion
 Alveolar- PAP, PNA
 Interstitial- ILD’s, IPF
 Neuromuscular Thoracic Wall- Kyphoscoliosis, AS
Low DLCO:
Differential Diagnosis

Obstructive Lung Disease- COPD, CF
 Interstitial Lung Disease Pulmonary Vascular Disease- Pulm HTN,
Venous Thromboembolic Disease

Cardiovascular Disease- Pulm Edema
 Anemia
Elevated DLCO:
Differential Diagnosis

Polycythemia
 Alveolar Hemorrhage
 Asthma
 Increased Pulmonary Blood Flow
– Left  Right Intracardiac shunt
– Exercise
– Mild CHF
Hemoptysis

DDx:
– Bronchitis
– Lung Cancer
– Idiopathic
– Bronchiectasis
– TB
Hemoptysis
‘Massive Hemoptysis’ = > 100-150ml
 Requires Emergent imaging and
bronchoscopy because:

– Mortality due to asphyxiation > exsanguination
Hemoptysis

Tx:
– Hemodynamic management
– Reverse coagulopathies
– Triage the DDx
– Consider FOB, IR, Surgical intervention
– ‘Bleeding side down if hemoptysis persists’