UMMC Internal Medicine
Board Review Course
Pulmonary Review
Demondes Haynes, M.D., FCCP
Associate Professor
Fellowship Program Director
Division of Pulmonary, Critical Care,
and Sleep Medicine
July 16, 2014
Disclosures
Speakers Bureau or Advisory Board
Participant

Forest Pharmaceuticals, Actelion
Pharmaceuticals, and Gilead Sciences
Asthma
Cardinal features



airway obstruction
inflammation
Hyperresponsiveness
Airway remodeling

Subepithelial fibrosis, increased smooth
muscle mass, angiogenesis, hyperplasia of
mucous glands and goblet cells
Classification of Asthma Severity
Symptoms
Nighttime
Symptoms
Step 5 or 6 Severe
Persistent
Continual symptoms
Limited physical activity
Frequent exacerbations
Frequent
Step 3 or 4 Moderate
Persistent
Daily symptoms
Daily use of inhaled SABA
Exacerbations affect activity
Exacerbations >2x/week
> 1x/week
Step 2 Mild Persistent
Symptoms>2x/week but not daily
Exacerbations may affect activity
>2 times a month
Step 1 Intermittent
Symptoms <2x/week
Asymptomatic & normal FEV1 between
exacerbations
Exacerbations brief; intensity may vary
< 2x/month
Asthma Pearls
Symptoms more than 2 days/week or more than 2
nights/month have persistent asthma
Inhaled corticosteroids with PRN bronchodilator is
the cornerstone of therapy for persistent asthma
If not persistent, can be treated with PRN
bronchodilator only
Occupational Asthma
Symptoms at work and improvement during
times away from work
Nearly 50% have symptoms 3-4 years after
cessation of exposure
RADS (reactive airways dysfunction
syndrome)


high level of respiratory irritant exposure
symptoms (cough, chest tightness, wheezing,
dyspnea) after exposure and may persist for
years
Allergic Bronchopulmonary
Aspergillosis (ABPA)
Manifests as severe persistent asthma
Clinical Manifestations







central bronchiectasis
pulmonary infiltrates (upper lobe
predominance)
brown sputum/mucous plugs
elevated IgE levels (>1000 ng/mL)
positive skin test to Aspergillus
peripheral eosinophilia
Steroids treatment
Exercise-induced Asthma
Symptoms begin 10-15 minutes after
exercise
Prevent with inhaled β-agonist 15 minutes
before exercise (effective in more than
80% of patients)
Alternatives not as effective as β-agonists
Regular exercise may lessen the degree
of exercise-induced asthma
Question 1
36 y/o woman with h/o anxiety, severe
asthma (multiple intubations) is seen in ER
with 1-day h/o severe SOB
PE: ↑ RR, ↑ HR, global wheezing & stridor
CXR: WNL
She was intubated with resultant near-total
resolution of abnormal breath sounds.
Most likely diagnosis is
Question 1
a.
b.
c.
d.
Acute severe asthma
ABPA
Anaphylaxis
Vocal cord dysfunction
Question 1
a.
b.
c.
d.
Acute severe asthma
ABPA
Anaphylaxis
Vocal cord dysfunction
Question 2
Smoking cessation
a. Eliminates lung cancer risk
b. Does not reduce CAD risk
c. Reduces but not eliminate lung volume
loss (FEV1)
d. Eliminates head & neck cancer risk
Question 2
Smoking cessation
a. Eliminates lung cancer risk
b. Does not reduce CAD risk
c. Reduces but not eliminate lung
volume loss (FEV1)
d. Eliminates head & neck cancer risk
COPD
Cigarette smoking is most important risk
factor & cessation is single most effective
intervention to stop decline in lung function
Accelerated decline in lung function in
smokers
O2 > 15 hrs/day increases survival,
hemodynamics, exercise capacity, lung
mechanics, and mental status
COPD Exacerbation
Change in dyspnea, change in cough, change in sputum
volume/character
Requires only ONE of above
Usually caused by virus, inhalant/irritant
Treatment
1. Albuterol/ipratropium
2. Glucocorticoids – 2 weeks
3. Antibiotics – none defined as better than others…except those at
risk for Pseudomonas – FEV1<50%, ABX 4x in last year, recent
hospitalization
4. NIPPV – decreases work of breathing, improves all outcomes
5. Endotracheal intubation
6. O2 to keep Sp02 90%
Alpha 1 Antitrypsin Deficiency
Screen early onset COPD (age < 50) and
strong family history of lung and/or liver
disease
Lower lobe emphysema predominance
Predominantly in whites of Northern
European heritage
Therapy at Each Stage of COPD
I: Mild
II: Moderate
III: Severe
IV: Very Severe
 FEV1/FVC < 70%
 FEV1/FVC < 70%
 FEV1 > 80%
predicted
 FEV1/FVC < 70%
 50% < FEV1 < 80%
predicted
 FEV1/FVC < 70%
 30% < FEV1 < 50%
predicted
 FEV1 < 30%
predicted
or FEV1 < 50%
predicted plus
chronic respiratory
failure
Active reduction of risk factor(s); influenza vaccination
Add short-acting bronchodilator (when needed)
Add regular treatment with one or more long-acting
bronchodilators (when needed); Add rehabilitation
Add inhaled corticosteroids if repeated
exacerbations
Add long term
oxygen if chronic
respiratory failure.
Consider surgical
treatments
Question 3
The role of aggressive pulmonary
rehabilitation in severe COPD is to
a.
b.
c.
d.
Improve survival
Improve exercise tolerance
Decrease oxygen requirement
Retard FEV1 decline
Question 3
The role of aggressive pulmonary
rehabilitation in severe COPD is to
a.
b.
c.
d.
Improve survival
Improve exercise tolerance
Decrease oxygen requirement
Retard FEV1 decline
Question 4
75 y/o with h/o COPD, DM, HTN was brought
to ER with cough, wheezes and weakness
CXR: Emphysematous apices,
WBC- 12,000; ABG:7.31/72/61 (on 3LPM)
Apart from starting IV steroids and antibiotics,
you also?
Question 4
a.
b.
c.
d.
Intubate him to protect his airway
Start NIPPV with BIPAP at 12/5 cm
Decrease O2 to reduce PCO2 retention
Intubate him because of hypercapnic
respiratory failure
Question 4
a.
b.
c.
d.
Intubate him to protect his airway
Start NIPPV with BIPAP at 12/5 cm
Decrease O2 to reduce PCO2 retention
Intubate him because of hypercapnic
respiratory failure
Bronchiectasis
Bronchial wall thickening and dilation
Causes – recurrent infection, obstruction,
mucus dysfunction
1.
2.
3.
4.
5.
6.
7.
Cystic fibrosis – poor sputum clearance, recurrent
infections and inflammation
Post-Pneumonia
Atypical mycobacterium – MAC, etc.
Ciliary dyskinesia – poor sputum clearance
(Assoc. w APKD, called Kartagener’s syndrome if
situs inversus is present)
ABPA
Ig deficiencies
Collagen vascular dz – RA, systemic sclerosis
Treatment involves bronchodilators, improved sputum clearance, abx.
Cystic Fibrosis
Autosomal recessive dz, abnormal cystic fibrosis transmembrane
regulator protein – regulates Cl- secretion
-Critical for proper fluid movement across resp, sweat duct, GI, and
reproductive epithelia
– low cl- secretion = low water secretion = thick secretions in all
organs
– sinus, lungs, pancreas, biliary, intestines, sweat
Note: thicker mucus, less
airway surface liquid,
matted down cilia
Cystic Fibrosis
Suspect in young to middle
age



Recurrent sinopulmonary
infections
Pancreatic insufficiency or
recurrent pancreatitis
Infertility (particularly men)
Diagnosis



Sweat chloride – High
Nasal potential difference
Gene mutation used when
chloride is normal or
borderline
CPT, high frequency
oscillator vest, exercise,
flutter device
Mucolytics – Dornase, NAC,
Hypertonic saline (7%)
Bronchodilators
Antibiotics (Inhaled and
systemic) based on micro
results
Inhaled or systemic steroids
Lung transplant
CF Complications
Pulmonary
Bronchiectasis
ABPA – Unresolving
exacerbation, sputum changes
to brown, hard plugs.
Massive hemoptysis
Pneumothorax
Atypical mycobacterium,
Pseudomonas, MRSA in
sputum
Cor pulmonale
Reactive airway dz
GI
Cholecystitis – remember
your CF pts have had
cholecystectomy
Pancreatic insufficiency –
on pancreatic enzymes
DIOS (distal intestinal
obstruction syndrome)
Interstitial Lung Disease
Clinical



dyspnea on exertion
nonproductive cough
abnormal CXR
HRCT most sensitive modality in
evaluation
Surgical biopsy required in most patients
IPF
Most common of idiopathic interstitial pneumonias
Age 50 or greater
UIP on pathology


spatial and temporal heterogeneity (fibrosis and honeycomb
change interspersed with normal lung)
“fibroblast foci”
Signs/symptoms




velcro-crackles
insidious onset (3 months or longer)
clubbing
lower lobe and peripheral predominance
Lung transplant improves survival and quality of life
PFT’s reveal restriction and gas exchange impairment
Nonspecific Interstitial Pneumonia
Younger age than IPF
Bilateral lower lobe opacities on CXR
HRCT – ground glass opacities; honeycombing
uncommon
PFT’s reveal restriction and gas exchange
impairment
Exclude CVD, exposures, etc.
Prognosis better than IPF
Treatment

steroids with or without cytotoxic agent
Cryptogenic Organizing Pneumonia
(COP)
Formerly known as BOOP
2/3 pts are nonsmokers
Presentation suggestive of LRTI
Crackles can be present but no clubbing
CXR reveals patchy consolidation (unilateral or
bilateral)
PFT’s reveal restriction and gas exchange
impairment
Treatment – steroids tapered over 6 months
Good prognosis
DIP/RBILD
Smokers
DIP – restrictive PFT’s and decreased
DLCO with hypoxemia
RBILD – mixed obstructive/restrictive
defect with decreased DLCO with
hypoxemia
Smoking cessation is best treatment; may
consider steroids
Lymphangioleiomyomatosis (LAM)
Proliferation of atypical smooth muscle cells
Primarily women of childbearing age
3rd -4th decade of life
Pneumothorax
PFT’s – obstruction, normal or increased lung
volumes, ↓DLCO
Diffuse thin-walled cysts
Chylous pleural effusions
Lung transplant

slowly progressive disease
Pulmonary Langerhans Cell Histiocytosis
Eosinophilic granuloma, histiocytosis x
Young (< 40) smokers
Obstructive (or mixed restrictiveobstructive) PFT’s with ↓DLCO
Combination of cysts and nodules in mid
and upper lung zones
CD1a and S-100 protein
“Birbeck granules”
Sarcoidosis
Non-caseating granulomas and exclusion
of specific cause
Bronchovascular and subpleural nodules
>90% have lung involvement
CD4 lymphocyte predominance on BAL
Endobronchial and transbronchial biopsy
diagnostic in up to 90% of cases
50-90% spontaneous remission in stage I
disease
Hypersensitivity Pneumonitis
CD8+ lymphocyte predominance on BAL
HRCT more sensitive than CXR
Antigen avoidance with steroids for
treatment
Pathology – cellular bronchiolitis,
interstitial infiltrate, poorly formed nonnecrotizing granulomas
Chronic HP may lead to pulmonary fibrosis
with UIP pattern
Miscellaneous ILD from
exposures
Hypersensitivity pneumonitis – may be
obstructive PFT’s – mold, birds, hay, hot tubs
(occurs shortly after exposures)
Byssinosis – raw cotton, hemp, flax
Berryliosis (just like sarcoid) – beryllium –
fluorescent lighting factory
Cobalt – (Giants cell pneumonitis) drill bits
Drug Induced Lung Dz
MTX


Hypersensitivity interstitial pneumonitis
Most common cause of non-cytotoxic reaction
Gold

Reversible by stopping drug
Bleomycin, Amiodarone, Nitrofurantoin (fibrosis)


Dose related pulmonary dz
Bleo most common cause of cytotoxic pulmonary
toxicity
Crack
Hypersensitivity, alveolar hemorrhage, bronchiolitis
obliterans
ATRA syndrome – fevers, alveolar infiltrates,
hypotension

Asbestos
Exposure
Calcified pleural plaques
Benign asbestos pleural
effusion (BAPE)

Exudative, eosinophilic
Increased risk of lung
cancer
Increased risk of
mesothelioma (less than
other cancers)
Asbestosis
ILD – requires long-term
exposure to asbestos
dust/fibers.



Basilar predominance
Restrictive PFT’s
May have other findings
related to asbestos
exposure
Treatment - supportive
Coal Workers Pneumoconiosis
Primarily in upper lobes
Caplan Syndrome

When associated with seropositive RA
Silicosis
Exposures

Mining, glass making,
ceramics, sand blasting,
foundry work
Primarily in upper lobes
Increases susceptibility
to TB – check PPD in
new silicosis
Progressive massive
fibrosis – rare
Pleural Effusions
Light’s criteria
 Protein ratio > 0.5
 LDH ratio > 0.6
 LDH > 2/3 ULN
Modified Light’s
 Protein >2.9
 Cholesterol >45
 LDH >60% Upper
Limit Normal
 Serum-Pleural
albumin <1.2gm
Serum-Pleural Albumin gradient <1.2 on diuresis is transudative
(pseudoexudate)
Pleural Effusion
Transudates
CHF, cirrhosis, nephrotic syndrome
Exudates
 infection, inflammation, malignancy
Bloody pleural effusion with associated with
pulmonary embolism not a contraindication to
anticoagulant therapy
Malignant Pleural Effusions
Exudative
Lymphocyte predominant
Cytology


initial negative, then repeat tap with cytology
pleural biopsy (VATS) increases yield to >66%
Recurrent malignant effusions


pleurodesis
long-term indwelling catheter
Parapneumonic Effusion
Exudative, neutrophil predominant, complicates
bacterial pneumonia
TPA-Dnase for nonimproving
Indications for chest tube drainage
 loculations on CT
 PF pH <7.0 (or 7.2)
 positive PF gram stain
 pus in pleural space
Surgical intervention
persistent sepsis or loculations despite drainage
TPA-Dnase
Tuberculous Pleuritis
Lymphocyte predominant (may be
neutrophilic early on)
Mesothelial cells <5%
PF adenosine deaminase (ADA)
PPD negative in up to 50% pts
Chylothorax
Milky-white effusion caused by thoracic duct
disruption
Causes



trauma
mediastinal tumors
LAM
PF triglycerides > 110 mg/dL
Treatment


pleuro-pertioneal shunt
prolonged CT drainage can lead to malnutrition
Hepatic Hydrothorax
5% of cirrhotics with portal hypertension
Congenital defects in diaphragm
Treatment


TIPS, liver transplant
NO CHEST TUBE – permanent fistulous tract
Pleural Pearls
Lymphocytic – TB, malignancy
Very low glucose and pH – Rheumatoid,
empyema
Eosinophils – blood, air, CABG, parasite,
asbestos
PE – exudative or transudative
Urinothorax – transudate & low pH, creatinine
TB – lymphs, ADA>40, IFN-γ
Exudate by LDH, not protein – parapneumonic,
malignancy
Venous Thromboembolism
Clinical suspicion drives algorithm
Don’t order V/Q scan with abnormal CXR
EKG

S in 1, Q in III, T inversion in III (reflects right ventricular strain)
D-Dimer


use in pts with low pretest clinical suspicion
good negative predictive value
Treatment


unfractionated heparin, LMWH, or fondaparinux
anticoagulation for at least 3 months (3-6 mts) with reversible
factor
Question 5
a.
b.
c.
d.
Pulmonary hypertension due to chronic
thromboembolic disease is best treated
with
Coumadin only
Bosentan only
Thromboendarterectomy + Coumadin
Calcium-channel blockers only
Question 5
a.
b.
c.
d.
Pulmonary hypertension due to chronic
thromboembolic disease is best treated
with
Coumadin only
Bosentan only
Thromboendarterectomy + Coumadin
Calcium-channel blockers only
Chronic Thromboembolic Pulmonary HTN
(CTEPH)
Develops in small percentage of patients after
acute PE
Definitive therapy
 anticoagulation
 thromboendarterectomy
Pulmonary thromboendarterectomy can improve
cardiac output, reduce mortality, and enhance
quality of life
Pulmonary Arterial HTN (PAH)
Classification


Idiopathic
Collagen vascular dz, shunt, portal htn, hiv,
drugs/toxin, sickle cell disease
Diagnosis and Evaluation



Echo
Right heart cath with vasodilator testing
6 minute walk test
Treatment of PAH
Anticoagulation
Oxygen +/- diuretics
Calcium Channel Blockers
Prostacylcins
Endothelial Receptor Antagonists
Phosphodiesterase inhibitors
Lung Transplant and atrial septostomy
Lung Cancer
Leading cause of death in men and
women
85% linked to smoking
Risk returns to normal after 15 years
cessation
Lung function does not return to normal
SPN guidelines
1. Pt high risk or low risk
High – smoker, first degree relative, age 65, known
exposure (asbestos, etc.)
Diagnosis guidelines
If nodule >8mm
1. PET-CT
1.
This combines both to
determine metabolic
activity and stage
2. If PET-CT positive
1.
False neg PET
-BAC,
hyperglycemia,
carcinoid
False Pos PET
-histo, sarcoid
Obtain biopsy of
nodule or lymph node
that represents highest
stage.
Stage determines resectability.
I-II –resectable (local or mets to hilar nodes)
III-IV-unresectable (subcarinal LN or distant or effusion)
Paraneoplastic Syndromes
Squamous- Hypercalcemia
Small Cell- SIADH, ectopic ACTH, EatonLambert syndrome
Adenocarcinoma- Hypertrophic pulmonary
osteoarthopathy
Sleep Apnea
OSA


obstructive apnea – absence of airflow for at least 10
secs with resp effort
obstructive hypopnea - ↓ of airflow by at least 30% for
at least 10 secs with desat of 4% or more
Risk factors
 neck size >17 cm in diameter, retrognathia,
elevated BMI, males, age > 50
Clinical Features
 Excessive daytime somnolence, fatigue,
morning headaches, snoring, etc.
Diagnosis - Polysomnogram (PSG)
Treatment of OSA
CPAP – treatment of choice

pneumatic splint for the airway
Surgery


craniofacial or anatomical airway abnormalities
tracheotomy in most severe with poor CPAP
compliance
Oral appliances

mild to mod OSA with intolerance to CPAP or
no response to upper airway surgery
Pulmonary Alveolar Proteinosis (PAP)
Loss of GM-CSF activity → alveolar
macrophage dysfunction → impaired
surfactant clearance
Bilateral alveolar infiltrates
“Crazy-paving” on HRCT
BAL stains positive with PAS
Whole lung lavage is treatment
Associated conditions: nocardia, PCP,
HIV, IgA deficiency
TB
Treat active TB with four drug therapy including:
INH, rifampin, PZA, and Ethambutol for 6 mos
total duration of tx
After 2 mos of 4 drugs can change to INH and
rifampin only for 4 more months
Give Vit B6(pyridoxine) when treating with INH
Latent TB
Treat with INH for 9 months for latent TB
PPD is considered positive according to risk
factors
PPD 5mm – HIV, organ transplant, recent close
contact
PPD 10 mm – Health care workers, homeless,
prisoners
PPD 15mm – No risk factors
Community Acquired Pneumonia
Severity – Admit or Not (There are many scores, this is easiest)
Confusion; Uremia; RR >30; BP <90; Age>65 (CURB-65)
1 point for each.
≤2 – outpatient (2 consider admit/obs)
≥3 admit
Bugs:
S. pneumoniae
gram negative
Atypicals
Outpatient
1. Macrolide or respiratory
quinolone (levofloxacin,
moxifloxacin)
Inpatient
1. Respiratory quinolone
2. Macrolide + β Lactam
Healthcare-Associated Pneumonia
Definition:
1. Hospitalized for 48hrs in last 3 months
2. Live in NH for healthcare facility
3. Outpt IV abx in last 30 days
4. Dialysis
Bugs
1. MRSA
2. gram-negative enterics
3. Pseudomonas
Empiric Treatment
4th gen ceph or carbapenem or pip/tazo + quinolone or AG + Anti-MRSA
De-escalate Treatment (sputum culture)
MRSA – 8 days single coverage
Gram negatives – 8 days single coverage
Pseudomonas – 2 weeks double coverage
Though some data argue only 5 days of double coverage,
then complete course with single coverage.
Misc. Pneumonia Pearls
Test question key facts

Bed bound, alcoholic
Klebsiella

Cattle/sheep
Q fever, pulmonary anthrax (gram + rod)

Birds
Psittacosis

Hunters
Tularemia

Bats, Chickens, Miss/Ohio river valley
Histoplasmosis

Southwest, E-multiforme
Coccidiomycosis

Central, southeast, mid-atlantic
Blastomycosis – a hunter with a sick dog

HIV/AIDS
PCP
Question 9
Risk factors for poor outcome in CAP
include the all of the following except
a.
b.
c.
d.
e.
Positive blood culture
Diastolic hypotension
RR> 24
Altered mental status at presentation
Density of localized pneumonic process
on CXR
Question 9
Risk factors for poor outcome in CAP
include the all of the following except
a.
b.
c.
d.
e.
Positive blood culture
Diastolic hypotension
RR> 24
Altered mental status at presentation
Density of localized pneumonic
process on CXR
Question 10
70 y/o man is being treated for VAP with
Vancomycin, Ciprofloxacin & Zosyn. After 3
days, BAL culture (sent on admission) now
is growing pen-sensitive Klebsiella spp.
Also patient has significant clinical
improvement.
What is the appropriate next step?
Question 10
a. Continue all antibiotics as patient is
improving
b. Stop all antibiotics as patient has
improved
c. De-escalate: continue Zosyn only
d. Repeat BAL to confirm cure
Question 10
a. Continue all antibiotics as patient is
improving
b. Stop all antibiotics as patient has
improved
c. De-escalate: continue Zosyn only
d. Repeat BAL to confirm cure
Pulmonary Miscellaneous Topics
Wegener’s granulomatosis: upper respiratory
tract and sinuses, granulomatous vasculitis,
glomerulonephitis. C-ANCA. Bx sinuses, lung or
kidney. Treat with cyclophosphamide +/steroids
Goodpasture’s syndrome: young males,
hemoptysis and hematuria. Anti GBM. Treat
with immunosuppressants and plasmapheresis
Lung Volumes
Normals
Tidal volume 500mL
Minute Vent 5-7L/min
Flow Volume Loops
Obstruction
Normal
Restriction
Asthma
COPD
Sarcoid
LAM
IPF
CTD-related
fibrosis
NM disease
Thoracic
abnormality
Sarcoid
Silicosis
LAM
Stepwise PFT interpretation
Spirometry (FEV1/FVC, FEV1, FVC)
1.FEV1/FVC
1.
2.
>70 is normal
<70 is obstruction
FEV1 (severity of obstruction)
>80% pred – normal
65-80% pred – mild
50-65% pred – moderate
<50% pred - severe
PFT’s
2. Lung Volumes (VC, TLC, RV)
- TLC <80%– restriction
Extrathoracic (Ribcage, spinal, muscle abnormalities)
(Think: scoliosis, kyphosis, ALS, myasthenia, MS. These also may need
cough assist, NIPPV, due to low MEP/MIP etc)
Intrathoracic (ex parenchymal dz)
(Think: sarcoid, IPF, ILD, silicosis, coal-workers, bird exposure,
etc)

COPD/Asthma – High RV, TLC
3. DLCO – If not associated with restrictive or obstructive
lung disease, consider pulmonary HTN, chronic
thromboembolic disease, CHF.
Variable Intrathoracic obstruction
Examples:
 Tracheomalacia
 Small Tracheal tumors
 Mediastinal
adenopathy
Variable Intrathoracic Obstruction
Variable Extrathoracic obstruction
Examples:
 Vocal cord dysfxn
 Tracheomalacia
Variable Extrathoracic Obstruction
Fixed upper airway obstruction
Examples:
 Large airway tumor
 Tracheal stenosis
 Goiter compressing
airway
Chronic Cough
Post-nasal Drip Syndrome
Asthma

Cough variant – Met challenge, rhinitis
GERD

Vagally mediated esophageal-tracheobronchial
reflex
Chronic Bronchitis
Bronchiectasis
ACE-I
Post-infectious
Observe which therapy relieves symptom
Pulmonary Pre-Op Evaluation
1. Quit smoking
2. Optimize medical
regimen
3. Do not operate during
exacerbation
4. Open abdominal and
thoracic have highest
pulmonary risk – close to
diaphragm.
***PFT’s not needed for preop!!
Only need for pre-lung
resection evaluation.
Best wishes on the board!!!! Get a good
night of sleep before the test. Read
the questions carefully!