Pulmonary Board Review

Pulmonary function tests:

TLC- total lung capacity

VC- vital capacity

RV- residual volume

IC- inspiratory capacity

FRC -functional residual volume

IRV- inspiratory reserve volume

TV—total lung capacity

ERV—expiratory reserve volume

FVC- forced vital capacity

FEV1- forced expiratory volume at one second

FEV1/FVC

FEV 25-50%

Intra-thoracic obstruction

Extra-thoracic obstruction

Diffusion capacity

Increased or decreased diffusion capacity

Alveolar hemorrhage

Early pulmonary edema

polycythemia

left to right cardiac shunt

pregnancy

Diffusion capacity/alveolar volume

6 second expiration

12% (200ml) improvement with bronchodilators

Methacholine challenge. FEV1 decreases by 20%

Good negative predictive value

Positive in asthma, COPD, viral illness, smoking, allergic rhinitis

Asthma

Cough, shortness of breath, chest tightness, intermittent symptoms

Cough variant asthma

Bimodal distribution

Airway obstruction, inflammation and hyper-responsiveness

Airway eosinophilia

Infiltration of inflammatory cells, denudation of epithelium, subepithelial fibrosis, mucous gland hyperplasia, increase smooth muscle mass, goblet cell hyperplasia

Airway remodeling

Wheezing on exam.

No wheezing on exam during status asthmaticus

If CO2 rises or ph lowers, time to intubate

Normal DLCO

Clinical definitions:

Mild intermittent asthma

<2 times per week, <2 per month nocturnal symptoms

Mild persistent asthma

>2 times per week<1 time per day, >2 per month nocturnal symptoms

Moderate persistent

Daily symptoms, daily use of bronchodilators, > 1 time per week nocturnal symptoms

Severe persistent

Continual symptoms, limited activity

Peak flow measurements

Worse at night, peak flows at the lowest in the am

Adjunct therapies

Allergic rhinitis treatment

Allergy meds

Nasal steroids

Anti-reflux meds

Occupational asthma

Recurrent symptoms while in working environment

Allergic sensitization or non allergic mechanisms

Hundreds of agents have been identified

Flour-byssinosis

RADS

No history of asthma, exposed to high levels of respiratory irritants

Allergic bronchopulmonary aspergillosis

wheezing

Humoral and cellular immune reaction to aspergillus fumigates

Bronchiectasis

Pulmonary infiltrates

Pulmonary fibrosis

Positive skin test for aspergillus

Elevated IGE levels

Serum precipitants

Steroids/antifungals

Churg-strauss vasculitis

Small vessel necrotizing vasculitis

“uncovered” in patients with asthma on leukotriene inhibitors and tapering off steroids

Exercise induced asthma/exercise induce urtacaria

Cold dry air

Exercise tolerance test reveals decrease in FEV1 with exercise

Aspirin sensitive asthma

Severe asthma with nasal polyps exacerbated by aspirin or NSAIDS

Treatment

Short acting β agonists

Inhaled corticosteroids

Long acting β agonists

Increased inhaled corticosteroids

Cromolyn

Leukotriene modifiers

Theophylline

Anti-IgE antibody

Omalizumab (Xolair)

Vocal cord dysfunction

Flutter waves on PFT’s

Obstructive lung disease

COPD/emphysema/chronic bronchitis/obliterate bronchiolitis/asthmatic bronchitis

GOLD guidelines

Airflow limitation which is not fully reversible

Smoking association

Air pollution/exposure to dust and chemicals/cooking/heating

Prevalence 6.9% for mild COPD

Prevalence 6.6% for moderate COPD

Screen for α-1 antitrypsin (AAT): younger, family occurrence, liver disease

ZZ alleles severely deficient

smoking cessation decrease the rate of decline of the FEV1

inflammatory response

chronic hyper-secretion of mucus, bronchial gland hypertrophy and goblet cells, ciliary dysfunction

loss of alveolar attachments and loss of elasticity, small airway collapse, loss of tethering

FEV1 does not correlate with symptoms

BODE

Body mass index/air flow obstruction/dyspnea/exercise capacity index (6 minute walk distance)

Correlates with survival

More than 75% of COPD patients have some reversibility with bronchodilators in FEV1

52% of non responders with change reversibility on the next set of PFT’s

Dynamic hyperinflation occurs with exercise, increases dyspnea

Flattened diaphragm. Increased retrosternal airspace. Increase A-P diameter

ABG to assess for hypercapnea and hypoxemia

Normal 6 minute walk distance is 500-600m. assess for hypoxemia

Screen for alpha-1 antitrypsin deficiency

Smoking cessation is the most effective intervention to reduce risk of developing COPD and stop progression

Only oxygen therapy and smoking cessation improve survival

Short-acting β-agonist, long acting β-agonists

SMART trial (salmeterol in asthma)

Short-acting anti-cholinergics, ipratropium

Long acting anti-cholinergics, triotropium

Theophylline (methylxanthines)

Inhaled corticosteroids does not affect the yearly decline in lung function

Inhaled corticosteroids decreases the rate of exacerbations

Inhaled CS and long acting beat agonist combined decline in quality of life and health status and rate of acute exacerbations is reduced, improvement of lung function and alleviation of Dyspnea

Oral CS not recommend for long term maintenance

Influenza and pneumococcal vaccine recommended

Weekly infusions of α-1 antitrypsin purified protein

Antibiotics for exacerbations

n-acetylcysteine (mucomyst) does not decrease the number of exacerbations

cough has a significant airway protective effect

leukotriene modifiers not recommended in COPD unless patient also has allergic rhinitis or asthma, but 1 study showed used acutely decrease the number of returns to ER within 30 days

oxygen for >15 hrs per day increases survival

P02 <55mmHg and sat <88% with or without hypercapnea

Or PaO2 >55mmHg but <60mmHg or sat >89% with evidence of pulmonary hypertension, peripheral edema suggesting right heart failure or polycythemia with Hct >55%

Nocturnal oxygen supplement

CPAP for nocturnal hypercapnea

Pulmonary rehabilitation—exercise training, nutrition counseling and education

Bullectomy

Lung volume reduction surgery (NETT)

Low FEV1 and low DLCO worse mortality

Predominant upper lobe bullae and low baseline line exercise function, improved function and survival

Lung transplant. Refer when FEV1<35%. PaO2 55-60 and PCO2 >50

COPD exacerbations

Increase in dyspnea, sputum production and sputum volumes

Many causes for exacerbations

Antibiotics therapy decrease hospitalization time

Bacteria: strep pneumo, H flu, Moraxella, Klebsiella, Pseudomonas, atypical (Chlamydia, legionella)

Systemic steroids

Bronchodilator therapy

Non-invasive positive pressure ventilation

Deceases rate of intubation by 28%, length of stay and in-hospital mortality (10%)

pH 7.25-7.35, CO2>45 and RR >25. Expect to see improvement by 10 in CO2 by 2-3 hrs

Intubation and Mechanical ventilation

Severe dyspnea, hemodynamic instability, impending respiratory arrest, life-threatening hypoxemia, sever acidosis (ph<7.25) and hypercapnea >60)

Discharge criteria:

Use of beta agonist no more than q 4hrs

Ability to walk across the room

Ability to eat and sleep without frequent awakenings by dyspnea

Stable clinical status and ABG for 24 hrs

Understanding by the patient and care givers the correct use of inhalers

Follow-up and home care arrangements

Sleep medicine

Obstructive sleep apnea

Absence of airflow for 10 seconds despite persistence of respiratory effort with desaturation of 4%

Oxygen saturation occurs after the obstruction

Terminates with snore and arousal

Hypopnea is decreased airflow of 30% lasting 10 seconds with 4% desaturation

Degree classified by AHI (apnea-hyponea index)

Mild 5-15

Moderate 16-30

Severe >30

Estimated prevalence is 24% of middle aged men and 9% of middle aged women

Epworth scale is an estimate of daytime sleepiness (>10 indicates sleepiness)

Incidence increases with neck circumference and increase in BMI

Increased risk with craniofacial abnormalities (enlarged tonsils too)

Incidence increases with age, women after menopause, hypothyroidism

Associated with increase risk of stroke, hypertension, MI, CHF and metabolic syndrome

Increases driving and work-related accidents due to sleepiness

Loss of upper airway tone

Complain of daytime sleepiness, fatigue, snoring, am headaches, dry mouth, witnessed apneas, gasping for breath

Evaluate with nocturnal polysomnography

Treatment with positive airway pressure, CPAP, BiPAP, weight loss

Adequate sleep hygiene, avoid sleeping on back

No driving or operating machinery until treated

CPAP improves quality of life, cognitive function, and daytime sleepiness

Improves mortality, blood pressure and cardiac function

Improve compliance with heated humidifier, appropriate mask selection

AutoPAP

Surgical procedures: tonsillectomy, uvulopalatopharyngoplasty (UPP), genioglossal or mandibular advancement, tracheostomy

Oral devices: tongue retaining devices, or mandibular advancement

modafinil

Central Sleep apnea

Carbon dioxide threshold, high ventilator response

“unmasked” by treatment of OSA with CPAP

Associated with congestive heart failure (EF<40%) and strokes

Insomnia, poor sleep quality, daytime sleepiness and fatigue

Treat underlying illness

Cheyne-stokes respiration, periodic breathing

BiPAP, oxygen

Obestity hypoventilation (pickwickian syndrome)

Increased CO2, decreased O2 due to hypoventilation. Chemoreceptors in brain are reset and less responsive to elevated CO2

Medroxyprogesterone, acetazolamide

Associated with OSA but not always (10-20%)

Oxygen desaturation during REM sleep (hypoventilation) in COPD patients

Neuromuscular diseases: muscular dystrophy, myotonic dystrophy, amyotrophic lateral sclerosis, post polio syndrome, myasthenia gravis

Restrictive lung disease, interstitial lung disease, kyphoscoliosis, obesity

Nocturnal respiratory impairment can precede daytime abnormalities

REM-related hypoventilation

Upright to supine reduction by >25% in vital capacity indicates diaphragmatic weakness

Non-invasive positive pressure ventilation

Narcolepsy

Excessive daytime sleepiness

Abdnormalities in REM sleep

Diagnosis with normal nocturnal PSG followed by abnormal MSLT (multiple-sleep latency test)

Short mean sleep latency and 2 or more SOREM’s (sleep onset REM’s)

Decreased hypocretin in CSF

HLA DR-4 associate

Cataplexy with excitement, laughing, hyponogogic hallucinations, sleep paralysis

Scheduled daytime naps recommened

CNS stimulants, modafinil

Rest leg syndrome diagnosised with URGE criteria

1. an urge to move the limbs with or without sensations 2. improvement with activity 3. worsening at rest 4. worsening in the evening or night

Peridoic limb movement disorder

Rhythmic movements of arms or legs during sleep

REM-behavior disorder

Associated with Parkinson’s disease

High altitude pulmonary edema

Sleeping at high altitude is associated with greater hypoxemia and crescendo-decrescendo breathing pattern followed by hypopnea or apnea

Increased ventilator response to hypoxia

Patients complain of frequent arousals and poor sleep quality.

Prevent by gradual ascent, acetazolamide, low dose hypnotics

Acute altitude sickness (mountain sickness) (18-40%)

Occurs with in 4-36 hours of ascent to 2000m (6500ft)

Headache, loss of appetite, nausea, vomiting, weakness, malaise, difficulty sleeping

Neurologic abnormalities can occur (HACE)

Resolves spontaneously in 2-5 days, worse the first night

Slow ascent, rest, acetazolamide, dexamethasone, if symptoms don’t reverse, have to descend

High-Altitude Pulmonary edema (HAPE) (.01%)

Occur 2-4 days after ascent.

Dyspnea, dry cough, worsening hypoxemia, frothy blood tinged sputum, fever, mental status changes, death

x-ray changes consistent with pulmonary edema

rate of ascent, male gender, physical activity, individual susceptibility

blunted ventilator response to hypoxia, accentuated pulmonary vasoconstrictor response to hypoxia, reduced vital capacity, leads to increase pulmonary pressures

inflammatory response, high pulmonary pressures and leakage of high protein fluid into alveoli

nifedipine is effective at treating along with descent and oxygen

air travel

commercial jets pressurized to 8000ft, leads to alveolar PO2 of 15% of inspired oxygen at sea level

with COPD patients, goal is PaO2 >50 mmHg

formula to predict the PaO2

Pulmonary Hypertension

Secondary, associated and primary

Pulmonary venous hypertension

Left atrial or ventricular disease (systolic or diastolic dysfunction)

Left-sided valvular disease

Pulmonary hypertension due to chronic hypoxemia

COPD/emphysema

Interstitial lung disease/Idiopathic pulmonary fibrosis

Sleep-disordered breathing

Alveolar hypoventilation disorders

Chronic exposure to high altitude

Pulmonary hypertension due to thrombotic or embolic disease

Chronic thrombo-embolic disease

Tumor, parasite, foreign material emboli

Sickle cell disease

Sarcoidosis, histiocytosis X, LAM (lymphangioleiomyomatosis)

Extensive compression of pulmonary arteries—tumor, adenopathy, fibrosisng mediastinisis

Pulmonary venous or capillary disease

Pulmonary veno-occlusive disease

Pulmonary capillary hemangiomatosis

Associated diseases:

Collagen vascular diseases (scleroderma, lupus)

Congenital systemic to pulmonary shunts

Portal hypertension

HIV infection

Drugs (Phen-Fen, rapseed oil)

Most common cause of pulmonary hypertension is left heart disease. Must be ruled out by right heart cath

Additional screening tests for diagnosis of pulmonary HTN

PFT’s

V/Q followed by pulmonary angiogram

High resolution CT chest

Serologic screening tests for HIV, sickle cell, collagen vascular disease

Polysomnography (sleep study)

Echocardiogram with bubble study (contrast, agitated saline)

Pathophysioloy of Pulmonary arterial hypertension

Narrowing of lumen of the small pulmonary arteries resulting in increased vascular resistance and pulmonary artery pressure

Vasoconstriction of the pulmonary arteries, proliferation of the cellular elements within the arterial wall obliteration of the vascular lumen. Plexiform lesions

Inability to recruit vessels during exercise leading to increased pulmonary pressures. Right heart unable to maintain cardiac output during exercise causing progressive Dyspnea, dizziness, syncope. Eventually right failure, dilation, arrhythmias

Familial PAH associated with the bone morphogenic protein receptor 2 (BMPR2).

93% of cases are sporadic form

Therapies

Oxygen

Coumadin, no randomized control trials, historical controls suggest improved survival

Digoxin for RV failure, not validated

Prostacyclin analogs

Epoprostenol (flolan)

Treprostinil (remodulin)

Iloprost (ventavis)

Endothelin-1 Receptor Antagonist

Bosentan (tracleer) A and B receptor

Ambrisetan (letairis) A receptor

Phosphodiesterarse Inhibitors

Sildenafil (Revatio)

Tadafinil

Increases NO by decreasing the degradation of cyclic guanosine 3’-5’ monophosphate (cGMP)

Diurectics, low dose

Atrial septostomy

Lung transplant

Per NIH registry of PAH patients in the 1980’s, the median survival was 2.8 years

Poor prognosis:

Advanced NYHA functional class

Poor exercise tolerance (6 minute walk distance)

Elevated right atrial pressure and pulmonary artery pressure

Increase PVR, decrease cardiac index

Chronic thrombo-embolic disease

Most patients lung perfusion returns to normal or near normal.

Some (4%) develop CTEPH

Proximal involvement of the pulmonary arteries may cause elevated pulmonary vascular resistance, cor pulmonale and right ventricular dysfunction

Exercise tolerance decreases and may have dizziness or syncope as the RV fails.

Obstruction of the vasculature not longer resembles a blood clot, it an organized web with extensive scarring. Walls become thicken and progressively narrowed. Angio shows tapering of vessels with luminal irregularities

Risk factors: previous PE, younger age, large perfusion defect, idiopathic PE

V/Q scan for diagnosis, followed by pulmonary angiography for confirmation and to evaluate if

Echo to evaluate RV dysfunction and pulmonary HTN

Pulmonary thromboendarteretomy in specific centers

Pulmonary Malignancies

Mesothelioma

Cancer of the pleura and peritoneum (90%/10%)

Estimated 3000 cases/year

5-6 more common in men

Long latency period (40 years)

Strong asbestosis exposure (90%)

Increased risk with incrased exposure

Ship building, (navy ship yards) Sand balsting, house building/destruction, mines, factories

Asbestosis increases risk of bronchogenic cancer

Smoking plus asbestosis 40-fold increases risk of bronchogenic cancer

Smoking does not increase the risk of Mesothelioma

Chest pain, shortness of breath, pleural effusion, pleural thicking, malais, weight loss

Surgical biopsy for diagnosis

Pleural biopsy is usually inadequate tissue

Placing of chest tube never finishes draining effusion and tumor will grow out the chest tube or biopsy site

VATS 80% positive, open thoractomy 90%

Distinguish from adenoca—negative PAS satin for mucin, negative TTF-1

Mature mestothelial cells

Poor respone to chemotherapy, radiation therapy. Extrapleural pneumonectomy

Survival 10% at 2 years

Solitary Pulmonary nodule

Single discrete intrapulmonary density less than 3 cm in diameter completely surrounded by normal aertated lung

Bronchogenic CA, mets to the lung, granulomas, benign lung tumors, AVM’s, COP, pulmonary infarcts

Estimated changes of malignancy based on size, age, smoking history, previous malignancies, nodule characteristics (Fleischer’s Recommnedation)

Malignant nodules have speculated margins, little or no calcifications, doubling times 30-500 days

Old films are key: benign lesions have either very long double times (200 years) or very short (30 days)

Benign lesions have smooth margins, central, diffuse or laminated calcifications

PET scan show uptake in >90% of malignant nodules >1cm in size, but can be false negative in bronchoaveloar carcinoma and false positive in inflammatory lesions

Initial evaluation: monitor with serial radiographs if low concern for cancer vs attempted transthoracic needle aspiration (must be >1cm). transbronchial biopsies unrealiable. Or surgical resection/biopsy.

Assess comorbidities. Lung function test. If possible straight to resection/lobectomy

Bronchogenic cancer

Second most common cancer, but the most common cause of cancer death.

Overall survival is 14-16% at 5 years. Not much improved from 20 years ago

80-90% related to cigarette smoking

Smoking cessation reduces risk to near baseline risk at 10 years

Other risk: second hand smoke, radon, asbestos, age and COPD

Screening by sputum cytology and chest radiography in the 1980’s failed to show reduction in lung cancer mortality. Increased detection

CT scan increases detection but also causes increased cost and morbitity for work up for bening lesions. Has not shown improvement in survival benefit

85% are non-small cell type. (adenoca, Squamous cell ca, large cell/undifferentiated)

15 % small cell type. 70% metastatic at the time of diagnosis.

Asymptomatic in only 5-15% of patinents

Present with cough (most common), dyspnes, chest pain, and Hemoptysis (indicates endobronchial component)

1/3 present with features suggestive of metastatic disease (bone pain, headache)

10% present with paraneoplastic syndromes: SIADH, clubbing, hypercalcemia, ACTH, hypertrophic osteoarthropathy

Diagnosis with bronchoscopy if central lesions (up to 90%), TTNA if peripheral (50-90%), sampling a metastatic sites: lymph nodes, pleural fluid. Most easily accessible site. Mediastinoscopy or surgical biopsy/resection

Staging

CT scan of chest, abdomen (liver and adrenals), bone scan, MRI of brain

Mediastinal biopsy of enlarged lymph nodes >1cm or distant sites

PET scan, not readily available at all sites, so not uniformly recommended

Resectability?

Operability? Nutrition, performace status, PFT’s

Metastatic to lung: lbs kg

Thyroid: teeny tiny nodules, calcify

Breast: nodules, pleural effusions

Renal: pleural effusions, bloody, large masses, endobronchial mets

Melanoma: hemorrhagic nodules, hemoptysis

Colon, to liver first

Lymphoma: lymphadenopathy, or nodules

Osteosarcoma: large calcified mets

Lymphangitic carcinomatosis

Benign neoplasm of the lung

Hamartoma

Normal tissue in abnormal place

Cartilage, fat, fibromyoid stroma

Very slow growing, eccentric “popcorn” calfications