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Sunday, February 7, 2016

Pulmonology Review for the End of Rotation Exams

Pulmonology for the End of Rotation Exams
Peak Expiratory Flow - use a peak flow meter
  • If <350 L/min, perform PFTs to screen for obstruction
Pulmonary Function Testing
  • FEV1: amount of air that can be forced out of the lungs in 1 second
    • Airway obstruction diagnosed by: normal/increased TLC with decreased FEV1
      • FEV1/FVC < 0.7
Disease
FEV1
FVC
FEV1/FVC
FEF25-75
FET
Peak Exp Flow
Obstructive
Decreased
<80%
Normal
<80%
Decreased
< 0.7
Decreased
<60%
Increased
Low
Restrictive
Normal or
Decreased
<80%
Decreased
<80%
Normal or Increased
0.7
Normal
>60%
Normal
Normal
Mixed
Decreased
<80%
Decreased
<80%
Decreased
< 0.7
Decreased or Normal
<60%
Increased or Normal

    • Tiffeneau index (FEV1/FVC x 100): % of FVC expired in one second
    • FET = Forced Expiratory Time
  • Pay Attention Here: The important thing to know about how to differentiate an obstructive vs. restrictive lung disease is based on their TLC, not the vital capacity, which will be decreased in obstructive lung diseases (this can be misleading).
Disease
TLC
FRC
TV
RV
VC
Obstructive
Increased
Increased
N
Increased
Decreased
Restrictive
Decreased
Decreased
N
Decreased
Decreased
  • Total Lung Capacity: volume of air in lungs after maximum inspiration
  • Functional Residual Capacity: volume of air in lungs after normal expiration
  • Residual Volume: volume of air in the lungs at maximal expiration
  • Tidal Volume: volume of air breathed in and out of lungs during quiet breathing
  • Vital Capacity: volume of air expelled from the lungs during a maximum expiration
1221_lung_volume.gif

  • Obstructive vs. Restrictive Lung Diseases
    • Obstructive Lung Diseases - COPD, asthma, cystic fibrosis, bronchiectasis, and bronchiolar diseases (constrictive bronchiolitis, bronchiolitis obliterans syndrome)
    • Restrictive Lung Diseases - interstitial lung diseases (ILD): sarcoidosis, chronic beryllium disease, hypersensitivity pneumonitis, pneumoconiosis, asbestos
Plethysmography Pattern
Feature
DLCO
Likely Dx
Obstructive
Hyperinflation (TLC >120%)
Decreased
Emphysema
Obstructive
Hyperinflation
Normal or Increased
Asthma
Obstructive
Normal lung volume
Normal
Chronic Bronchitis
Restrictive
Low RV
Decreased
Scar (Sarcoid or fibrosis)
Restrictive
Normal RV
Normal
Neuromuscular disease
  • DLCO (Diffusing capacity of the lung for carbon monoxide) = CO2 into lungs - CO2 out of lungs
    • We use carbon monoxide because we can maximize diffusion because of the affinity of Hgb for it → Volume = Area/Thickness x (P1-P2) x constant
    • In emphysema, tissue is destroyed, reducing surface area for diffusion → decreased area causes volume to decrease
    • In sarcoidosis and fibrosis, lung thickness increases, also driving volume to decrease
    • In chronic bronchitis - mucous and inflammation creates a barrier for air to get into lungs, low P1-P2 drives the volume downward


Acute Bronchitis
Etiology: Viruses (most)
Cough (+/- sputum) - lasts 1-2 weeks
Chest discomfort
Shortness of breath
+/- Fever

Labs not indicated, unless pneumonia suspected → CXR
Antibiotics not necessary - most viral
Cough suppressants - Codeine-containing cough meds
Bronchodilators (albuterol)

Asthma
  1. Characteristics
    1. Airway inflammation
    2. Airway hyperresponsiveness
    3. Reversible airflow obstruction
  2. May begin at any age
  3. Dyspnea common when exposed to rapid changes in temperature and humidity
Extrinsic
Intrinsic
  • Atopic: produce IgE to environmental triggers (eczema, hay fever)
  • Become asthmatic at young age
  • Not related to atopy or environmental triggers
  • PFTs (obstructive): decreased expiratory flow, low FEV1, and decreased FEV1/FVC ratio
    • Increased FEV1 > 12% with albuterol
    • Decrease in FEV1 >20% with methacholine or histamine
    • Increase in diffusion capacity of lung for carbon monoxide


Triggers: pollens, house dust, molds, cockroaches, cats, dogs, cold air, viral infections, tobacco smoke, meds (BB, aspirin), exercise
Intermittent: SOB, wheezing, chest tightness, cough
-Occurs in 30 mins to exposure to triggers
-Symptoms worse at night
-Wheezing (inspiration AND expiration) is most common finding
-PFTs: required to DX
-Spirometry before and after bronchodilators: increase in FEV1 or FVC by 12%

-SABA (albuterol) for acute attacks
  • Onset 2-5 min, duration is 4-6 hours
-LABA (salmeterol) for nighttime asthma and exercise induced asthma
-Inhaled Corticosteroids: mod to severe asthma
  • Use regularly to decrease airway hyperresponsiveness and exacerbations
-Montelukast: prophylaxis for mild exercise induced asthma and control of mild-mod
  • Allows for reduction in steroid and B2

-Cromolyn sodium: prophylaxis before exercise

AVOID Beta-BLOCKERS in asthmatics
Acute Asthma Exacerbation
Tachypnea
Sweating, wheezing, speaking in incomplete sentences, use of accessory muscles

-Paradoxical movement of the abdomen and diaphragm on inspiration
-Peak exp flow: low
  • Severe <60
-ABG: increased A-a gradient
-CXR: r/o pneumonia, pneumothorax
-Inhaled B2 agonist via nebs or MDI
-Corticosteroids: IV or orally
-IV magnesium: can help prevent bronchospasm
Complications:
-Status asthmaticus: does not respond to standard meds
-ARDS: resp muscle fatigue
-Pneumothorax, atelectasis, pneumomediastinum


Monitor peak flow (peak expiratory flow rate) - measures airflow obstruction
  • Adult normal ranges: 450-650 L/min (men) vs. 350-500 L/min (women)
  • Mild >300, Mod-Severe 100-300, Severe <100
  • Mild persistent asthma: monitor periodically, increase dose of inhaled steroid if peak flow decreases
  • Moderate persistent asthma: daily monitoring required. Increase dose of inhaled steroid if peak flow decreases.
  • Severe persistent: daily monitoring; initiate prednisone if peak flow decreases


Severity
Long-term Control Medications
Mild intermittent (symptoms <=2 times/week)
None
Mild persistent (symptoms 2+ times/week, not everyday)
Low dose inhaled corticosteroid
Moderate persistent (daily sx, frequent exacerbation)
Daily inhaled corticosteroid (low dose) or cromolyn/nedocromil, methylxanthine, or antileukotriene
Severe persistent (continued sx, frequent exacerbation, limited physical activity)
Daily corticosteroid (high dose)
+ LABA or methylxanthine
+ oral corticosteroids


Bronchoprovocation test - useful when asthma suspected, but PFTs non-diagnostic
  • Measures lung function before and after inhalation of increasing doses of methacholine; hyperresponsive airways → obstruction at low doses


Chest XR - severe asthma reveals hyperinflation
  • Only get with severe asthma to r/o pneumonia, pneumothorax, pneumomediastinum, foreign body


Arterial Blood Gas (ABG) - if significant respiratory distress
  • Hypocarbia (common) with hypoxemia
  • If PaCO2 normal or increased, respiratory failure may ensue - asthmatics have increased respiratory rate, which should cause PaCO2 to DECREASE


Chronic Obstructive Pulmonary Disease - 4th leading cause of death in US
  • Coexisting bronchitis and emphysema, rarely one or the other by itself
  • Leads to chronic respiratory acidosis with metabolic alkalosis as compensation
  • Risk factors and causes
    • Smoking (tobacco)
    • Alpha-antitrypsin deficiency - worse with smoking
    • Environmental factors - second hand smoke
    • Chronic asthma - independent risk factor?


Chronic Bronchitis
Emphysema - elastase (protease) excess and overinflation (“pink puffers”, barrel chest, pursed lips)
Excess mucus production narrows airways → productive cough
Scarring and inflammation → enlargement of glands → smooth muscle hyperplasia = obstruction
Centrilobular - most common; seen in SMOKERS; destruction limited to bronchioles (upper lungs)
Panlobular - patients with alpha-1 antitrypsin deficiency; destruction both proximal and distal acini (lung bases)
Elastase - released from PMNs and macrophages, ingesting lung tissue (normally inhibited by alpha-1 antitrypsin)
Tobacco smoke - increases PMNs and macrophages, inhibits antitrypsin, and increases oxidative stress on lung
Cough, sputum production, dyspnea (on exertion or at rest)

Clinical diagnosis: Chronic cough, productive sputum > 3 months, at least 2 consecutive years
Signs:
-Prolonged forced expiratory time (takes longer to get all air out)
-During auscultation: end-expiratory wheezes on forced expiration, decreased breath sounds, inspiratory crackles
-Tachypnea, tachycardia
-Cyanosis
-Use of accessory muscles
-Hyperresonance on percussion
-Signs of cor pulmonale
Pathologic diagnosis: permanent enlargement of air spaces distal to terminal bronchioles due to destruction of alveolar walls
CXR - low sensitivity; useful in acute exacerbation to r/o pneumonia or pneumothorax
  • Hyperinflation, flat diaphragm, enlarged retrosternal space
  • Diminished vascular markings
Alpha-antitrypsin levels - FH premature emphysema?
ABG - chronic pCO2 retention, decreased pO2
PFTs (spirometry): diagnostic
FEV1/FEV <0.75
FEV1 is decreased
Total lung capacity (TLC), residual volume, FRC increased (indicates air trapping)
Vital capacity decreased - extra air that does come in is not useful → becomes residual volume (dead space)
  • Smoking cessation (most important): smokers have faster decline in FEV1 (3-4x)
  • Inhaled B2 agonists (albuterol): symptomatic relief
    • Long term agents (salmeterol) for frequent use
  • Inhaled anticholinergics (ipratropium bromide): slower onset of action, lasts longer
  • Combination of B-agonist and anticholinergic (helps with adherence to therapy)
  • Inhaled corticosteroids (budesonide, fluticasone): anti-inflammatory; slows decline in FEV1 over time
  • Oxygen therapy: get an ABG to determine need for oxygen; long-standing hypoxemia → pulmonary hypertension and cor pulmonale
  • Pulmonary rehab: improves exercise tolerance
  • Vaccinations: influenza (annual) and streptococcus pneumoniae every 5-6 years (>65)
  • Surgery: lung resection vs. transplant
  • Smoking cessation and home O2 are only interventions shown to lower mortality
  • Give steroids and antibiotics for acute exacerbations: increased sputum production or change in character or worsening SOB
    • Not responsive to bronchodilators
    • IV methylprednisolone if hospitalized
    • Azithromycin or Levofloxacin
    • O2 > 90%, nasal cannula
    • NPPV: BiPAP or CPAP
    • Can lead to ARDS
  • Criteria for continuous or intermittent long-term O2:
    • PaO2 55 mmHg
    • O2 saturation <88% (pulse ox) either at rest or during exercise
    • PaO2 55-59 mmHg + polycythemia or cor pulmonale
  • Look for nocturnal hypoxemia; give CPAP or O2 as needed
  • Clinical Monitoring
    • Serial FEV1 measurements (highest PPV)
    • Pulse oximetry
    • Exercise tolerance testing
  • Acute Exacerbations
    • Most common causes: infection, non-compliance, cardiac disease
    • Secondary polycythemia (Hct >55% men, 47% women) - response to chronic hypoxemia
    • Pulmonary HTN and cor pulmonale - severe, longstanding COPD
  • COPD Staging - Global initiative for chronic Obstructive Lung Disease Criteria


GOLD I Mild
No symptoms
Cough, sputum
Limited exercise capacity
Infrequent exacerbations
1. Bronchodilator in MDI (with spacer)
2. Anticholinergics and/or B-agonists (first line)
FEV1 > 80% predicted
GOLD II Moderate
Significant limitation in exertional capacity
Limited ADLs

3. Inhaled glucocorticoids
4. Theophylline (optional)
50% < FEV1 < 80%
GOLD III Severe
SOB, even at rest
5. Continuous O2
6. Pulmonary rehab
7. Triple inhaler therapy
30% < FEV1 <  50%
GOLD IV
Very Severe
Frequent, severe exacerbations

FEV1 < 30% predicted


Pneumonia
  • In alcoholics, think Klebsiella; in immigrants, think TB
  • In nursing home residents, think nosocomial and predilection for upper lobes (Pseudomonas)
  • HIV (+) patients - Pneumocystis carinii and Mycobacterium tuberculosis
  • Legionella - common in organ transplant pts, renal failure, chronic lung disease, smokers
  • Ventilator Associated Pneumonia - cannot cough, hard to clear mucous and positive pressure impairs ability to clear colonization
    • DX: bronchoalveolar lavage (BAL)
    • TX: all three
      • Cephalosporin (Cefepime or ceftazidime) OR penicillin (Zosyn)
      • Fluoroquinolone or Aminoglycoside
      • Vancomycin or Linezolid


Community Acquired Pneumonia (CAP)
Nosocomial Pneumonia (Hospital Acquired Pneumonia)
-Occurs in first 72 hours of hospitalization
-Typical or atypical
-MC pathogen: S. pneumoniae (60%), H. flu, anaerobic GNR (Klebsiella and other Enterobacteriaceae), S. aureus
-Atypical: Mycoplasma pneumo, Chlamydia pneumo, Legionella (not visible on gram stain and not culturable)
-Bacteremic pneumonia: S. pneumoniae (66%), H. flu, influenza virus, Legionella
-Occurs during hospitalization after first 72 hours
-MC pathogens gram negative rods (E. Coli, Pseudomonas) and Staphylococcus aureus
-Classic CAP: acute onset chills followed by fever, pleuritic pain (pleural effusion), productive cough (thick, purulent sputum), dyspnea
  • Most caused by aspiration of gastric contents
-Atypical: sore throat, headache, non-productive dry cough, dyspnea, fatigue, myalgias (fever/chills uncommon)
-Typical Signs: tachycardia, tachypnea, late inspiratory crackles, bronchial breath sounds, increased tactile and vocal fremitus, dullness on percussion, pleural friction rub (pleural effusion)
-Atypical signs: normal pulse in setting of high fever, wheezing, rales, rhonchi

-Typical CXR: lobar consolidation (multilobular = serious)
-Atypical CXR: diffuse reticulonodular infiltrates, absent or minimal consolidation
-PA and lateral CXR required to confirm DX - if not suggestive, do not treat → after TX, changes lag behind clinical response (up to 6 weeks); false negatives with neutropenia, dehydration, infection with PCP and early disease (<24 h)
-CBC w/ diff, BUN/Cr, glucose, electrolytes
-O2 saturation
-2 pre-treatment blood cultures
-Sputum culture with gram stain - controversial; order in all hospitalized patients (>25 PMNs and <10 epithelial cells); culture if hospitalized
  • Acid-fast stain - MTB
  • Silver stain - fungi, PCP for HIV/immunocompromised
-Urinary antigen for Legionella - very sensitive for up to weeks

1. Decision to hospitalize (pneumonia severity index) - if patient hypoxic or hypotensive ADMIT
2. Outpatient treatment - cover atypicals and gram (+)
  • Macrolides (azithro or clarithromycin) or doxycycline
  • Fluoroquinolones (ciprofloxacin)
  • Elderly with comorbidities (typical CAP) or prior TX with antibiotics - levofloxacin or moxifloxacin
  • TX for 5 days, do not stop until afebrile 48 h
3. Inpatient treatment - Fluoroquinolone alone OR 3rd generation cephalosporin + macrolide (ceftriaxone + azithromycin)
1. Treat for Gram Negative Rods - any of the three
  • Cephalosporin with pseudomonal coverage: Ceftazidime or Cefepime
  • Carbapenems: imipenem
  • Piperacillin/tazobactam (Zosyn)
Complications:
-Parapneumonic effusion (50%) - most resolve with treatment with antibiotics; thoracentesis if effusion >1 cm on lateral decubitus → send fluid for gram stain, culture, pH, cell count, glucose, protein, LDH
Complications:
-Parapneumonic effusion (50%) - most resolve with treatment with antibiotics; thoracentesis if effusion >1 cm on lateral decubitus → send fluid for gram stain, culture, pH, cell count, glucose, protein, LDH


Carcinoid Tumor
  • Originate from neuroendocrine cells and secrete serotonin
  • Most common site - appendix, but can be found in a variety of locations (small bowel, rectum, bronchus, kidney, pancreas)
  • Carcinoid syndrome (10%): excess serotonin secretion
    • Cutaneous flushing, diarrhea, sweating, wheezing, abdominal pain, heart valve dysfunction
    • Risk factors of mets increased with size: ileal tumors have greatest likelihood
    • TX: surgical resection of tumor


Pulmonary Neoplasm (Lung Cancer)
  • Risk Factors
    • Cigarette smoking (>85%) - more pack years, high risk
      • Adenocarcinoma - lowest association of smoking
    • Passive smoke
    • Asbestos - common in shipbuilding and construction, car mechanics, painting
      • Smoking + asbestos = high risk
    • Radon - high levels in basements
    • COPD - independent risk factor
  • Metastatic disease - Brain, Bone, Adrenal glands, Liver



Small cell lung cancer (SCLC) - 25%
Non-small cell lung cancer (NSCLC) - 75%
  • Squamous cell carcinoma, adenocarcinoma, large cell carcinoma, bronchoalveolar cell carcinoma



Symptoms
&
Signs
-Recurrent pneumonia
-Constitutional SX (advanced disease) : anorexia, weight loss, weakness
-Superior Vena Cava syndrome (SCLC): obstruction of SVC by mediastinal tumor, facial fullness, facial and arm edema, dilated veins over anterior chest, arms, face; JVD
-Phrenic nerve palsy - hemidiaphragmatic paralysis
-Recurrent laryngeal nerve palsy - hoarseness
-Horner’s syndrome: invasion of cervical sympathetic chain by apical tumor → unilateral facial anhidrosis (no sweating), ptosis, miosis
-Malignant pleural effusion
-Eaton-Lambert syndrome (most common in SCLC): similar to myasthenia gravis (proximal muscle weakness/fatigue, diminished DTRs, paresthesias (lower ext)
-Digital clubbing
-Airway involvement (SCC) = cough, hemoptysis, obstruction, wheezing
-Pancoast’s syndrome (SCC): superior sulcus tumor → shoulder pain, radiates down arm; pain and upper extremity weakness due to brachial plexus invasion, Horner’s (60%) of time  
Paraneoplastic syndromes
  • SIADH: with SCC
  • Ectopic ACTH - SCC
  • PTH-like secretion - SCC
  • Hypertrophic pulmonary osteoarthropathy - adenocarcinoma and SCC
Diagnosis
-CXR most important for DX, but not used for screening
-CT chest with IV contrast (use to stage)
-Tissue biopsy - determine histologic type
-Cytologic exam of sputum - DX central tumors, not peripheral lesions
-Fiberoptic bronchoscope - DX central tumors, not peripheral lesions
-PET scan
-Transthoracic needle biopsy - suspicious masses, highly accurate for peripheral lesions
-Mediastinoscopy - advanced disease
CXR shows pleural effusion (tap it for malignant cells)
Always perform a biopsy for intrathoracic lymphadenopathy
Treatment
- Limited: chemo + radiation
-Extensive: chemo only → if responsive, radiation therapy
Surgery - best option
  • If metastatic outside chest, not a candidate
  • May recur after surgery
Radiation - important adjunct to surgery
-Chemotherapy - uncertain benefit
Prognosis
Limited: 10-13% 5-year survival
Extensive: 1-3% 5-year survival

Staging
Limited - confined to CHEST + supraclavicular nodes (not cervical or axillary)
Extensive - outside chest and supraclavicular nodes  
Primary TNM system


Solitary Pulmonary Nodule - single, well-circumscribed nodule seen on CXR (incidental) with no associated mediastinal or hilar lymph node involvement (decide if malignant)
  • Low probability - serial CT scan
  • Intermediate (<1 cm) - serial CT scan
  • Intermediate (>1 cm) - PET scan → if positive, excise
    • May also perform transthoracic needle aspiration biopsy or fiberoptic bronchoscopy
  • High - excise


Benign
Malignant
  • Young (<50)
  • Size: small (<1 cm)
  • Borders: smooth, discrete
  • Calcification: dense, central calcification
  • CXR: stable for >2 years
  • Older age (>50)
  • Smoking
  • Size: large (> 2 cm)  
  • Borders: irregular
  • Calcification: eccentric, asymmetric
  • Change in size: enlarging
Follow every 3 months
Biopsy and resect
  • Testing
    • Flexible bronchoscopy - central lesions
    • Transthoracic needle biopsy
    • PET scan - determines if malignant


Bronchiectasis - permanent, abnormal dilation and destruction of the bronchial walls; cilia damaged; onset in childhood (usually)
  • Infection with airway obstruction or impaired defense/drainage precipitates disease
  • Causes: cystic fibrosis (most common), infection, immunodeficiency, airway obstruction
  • Features:
    • Chronic cough with large amounts of mucopurulent, foul smelling sputum
    • Dyspnea (SOB)
    • Hemoptysis - mild, self limited
    • Recurrent or persistent pneumonia
  • Diagnosis - high resolution CT (gold standard), PFTs (obstructive pattern), CXR (abnormal, nonspecific)
  • Treatment
    • Antibiotics - acute exacerbations
    • Bronchial hygiene - fluids, chest physiotherapy, inhaled bronchodilators


Hypoventilation Syndrome - condition in which severely overweight people fail to breathe rapidly or deeply enough, resulting in low blood O2 and high CO2 levels
  • May result in OSA (periods of frequent absence of breathing for short periods of time) resulting in partial awakenings at night and sleepiness during the day
  • This may lead to eventual heart failure symptoms, such as leg swelling
  • Clinical features: obesity (BMI > 30), hypoxemia during sleep, and hypercapnia resulting from hypoventilation  
  • TX: weight loss and continuous positive airway pressure (CPAP)


Obstructive sleep apnea (90%)
Sleep hypoventilation syndrome (10%)
5+ episodes of apnea, hypopnea, or respiratory related arousals per hour (high AHI) during sleep
Rise of CO2 by 10 mmHg after sleep compared to awake measurements and overnight drops in O2 levels without apnea or hypopnea
Snoring
Apnea - cessation of breathing
Interrupted sleep and excessive daytime sleepiness; worsened by high CO2 levels (CO2 narcosis)
Other: depression, hypertension, headaches worse in AM
Snoring
Apnea - cessation of breathing
Interrupted sleep and excessive daytime sleepiness; worsened by high CO2 levels (CO2 narcosis)
Other: depression, hypertension, headaches worse in AM
BMI > 30 kg/m^2 (weight/height^2)
ABG CO2 > 45 mmHg
-Polysomnography (required): EEG, EKG, pulse oximetry
-Screen for TSH (hypothyroid) and CBC (polycythemia)
-CXR or CT scan, spirometry performed
Complications: cor pulmonale (33%)
BMI > 30 kg/m^2 (weight/height^2)
ABG CO2 > 45 mmHg
-Polysomnography (required): EEG, EKG, pulse oximetry
-Screen for TSH (hypothyroid) and CBC (polycythemia)
-CXR or CT scan, spirometry performed
Complications: cor pulmonale (33%)
TX: CPAP
If O2 < 90%, switch to BiPAP (higher pressure during inspiration, lower pressure during expiration)
If both ineffective, add O2 therapy
Last resort: tracheostomy
Acetazolamide - reduces bicarbonate levels


Pulmonary Hypertension - mean pulmonary arterial pressure greater than 25 mmHg at rest or 30 mmHg during exercise
  • Passive type - resistance to pulmonary venous drainage
    • Ex. mitral stenosis, LVHF, atrial myxoma, pulmonary veno-occlusive disease
  • Hyperkinetic - high pulmonary blood flow (L to R shunts)
    • Ex. VSD, ASD, PDA
  • Obstructive type - resistance to flow through large pulmonary arteries
    • Ex. PE, pulmonary artery stenosis
  • Obliterative type - resistance to flow through small pulmonary vessels (arterioles) due to parenchymal inflammation leading to fibrosis
    • Ex. primary pulmonary hypertension, collagen vascular disease, CREST syndrome
  • Vasoconstrictive type - resistance to flow due to hypoxia-induced vasoconstriction
    • Ex. chronic hypoxemia, COPD, OSA
  • Increased intrathoracic pressure - transmitted to pulmonary vasculature
    • Ex. mechanical vent with PEEP, COPD
  • Increased blood viscosity - polycythemia vera


Primary Pulmonary Hypertension (PPH) - pulmonary HTN in absence of disease of heart or lung; diagnosis of exclusion
  • Abnormal increase in pulmonary arterial resistancethickening of pulmonary arterial walls
  • Cause: unknown


Primary Pulmonary Hypertension (PPH)
-Dyspnea on exertion
-Fatigue
-Chest pain (exertional)
-Syncope (exertional) in 50%

Young or middle-aged women
-Loud pulmonic P2 sound, subtle lift of sternum
-JVD, hepatomegaly, ascites, peripheral edema
-Diagnosis of exclusion
-EKG: right ventricular hypertrophy, right axis deviation and right atrial abnormality
Echo: dilated pulmonary artery, dilation and hypertrophy of RA/RV, abnormal movement of IV septum
Right heart cath: gold standard; increased PA pressure
-CXR: clear lungs, enlarged RV, central pulm arteries
-PFTs: restrictive
-ABGs, serology
-V/Q scan: PE vs. PPH
1. IV prostacyclins (epoprostenol) and CCB: lower pulm vasc resistance
2. Anticoagulation with Warfarin (goal: 2.0) due to venous stasis, physical inactivity, risk of thrombosis
3. Lung transplant


Prognosis: poor, mean survival 2-3 years from DX
Cor Pulmonale
- Decreased exercise tolerance
-Parasternal lift
-Cyanosis
-Clubbing
-JVD, hepatomegaly, ascites, peripheral edema
-Polycythemia (if COPD present)
CXR: enlarged RA, RV, and pulm-arteries
EKG: right axis deviation, P-pulmonale (peaked P waves), right vent hypertrophy
1. Treat underlying pulmonary disorder
2. Use diuretic therapy cautiously, pt. may be preload dependent
3. Apply continuous long-term O2 therapy if hypoxic
4. Digoxin - if coexistent LV failure


Cor Pulmonale - right ventricular hypertrophy with eventual RV failure, resulting from pulmonary HTN, secondary to pulmonary disease
  • Most commonly secondary to COPD
    • Other causes: recurrent PE, ILD, asthma, CF, OSA, pneumoconiosis


Interstitial Lung Diseases: inflammatory process involving the alveolar wall (resulting in widespread fibroelastic proliferation and collagen deposition) that can lead to irreversible fibrosis, distortion of lung architecture, and impaired gas exchange.
  • History: medications (chemotherapy, gold, amiodarone, penicillamine, nitrofurantoin)
    • Previous jobs: asbestos (electrical or building insulation), beryllium, coal, silicone
  • Symptoms: dyspnea (first with exertion → rest)
    • Cough (non-productive)
    • Fatigue
  • Signs: rales at the bases (common), digital clubbing (IPF), signs of pulmonary HTN and cyanosis in advanced disease
  • Diagnosis: CXR (reticular, reticulonodular, ground glass, honeycombing)
    • High resolution CT: shows extent of fibrosis better
    • PFTs (restrictive): increased FEV1/FVC, lung volumes low, FEV1 and FVC are low but FVC lower
    • O2 desaturation with exercise
    • Bronchoalveolar lavage (culture/cytology): controversial, variable results
    • Tissue biopsy: required in most
    • Urinalysis: if signs of glomerular injury (Goodpasture’s syndrome and Wegener’s granulomatosis)


Interstitial Lung Diseases Associated with Granulomas
Sarcoidosis - chronic systemic granulomatous disease characterized by noncaseating granulomas, often involving multiple organ systems; lungs most involved
  • Etiology unknown
  • Most often occurs in African Americans, especially in women
  • 75% of cases occur when <40
  • Good prognosis for most patients

Clinical Features
Diagnosis
Treatment
Sarcoidosis
Constitutional SX: malaise, fever, anorexia, weight loss
-Lungs: Dry cough, dyspnea on exertion
-Musk (25-50%): arthralgias, arthritis, bone lesions
-Skin (25%): erythema nodosum
-Eyes (25%): anterior uveitis (75%), posterior uveitis (25%), conjunctivitis
-Heart (5%): arrhythmias, heart block, sudden death
-Nervous (5%): CN VII (Bell’s palsy), optic nerve dysfunction, papilledema, peripheral neuropathy

CXR: bilateral hilar adenopathy (hallmark, nonspecific, 50%)
Serum Angiotensin-converting enzyme (ACE): elevated (50-80%, not sens/spec)
Hypercalciuria, hypercalcemia (common)

Definitive DX: transbronchial biopsy - must see noncaseating granulomas (not diagnostic by itself)

PFTs:
-Decrease VC/TLC
-Decreased DLCO
-Decreased FEV1/FVC ratio

Most resolve spontaneously in 2 years, no treatment
1. Systemic (PO) steroids: if symptomatic, active lung disease, PFT deterioration, conduction disturbances, severe skin or eye involvement
2. Methotrexate - if refractory


Staging
  • Honeycombing: scarred shrunken lung; air space dilated, fibrous scarring in interstitium; associated with a poor prognosis
Stage 1
Stage 2
Stage 3
Stage 4
Bilateral hilar adenopathy WITHOUT
Parenchymal infiltrates
Hilar adenopathy
WITH
Parenchymal infiltrates
NO hilar adenopathy +
Diffuse parenchymal infiltrates
Pulmonary fibrosis with honeycombing +
Fibrocystic parenchymal changes


Pneumoconiosis (Environmental Lung Disease): accumulation of dust in the lungs, and the tissue reaction to its presence
  • Ex. silica, beryllium, asbestos, coal dust, graphite, carbon black, aluminium, talc  


Coal Worker’s
Asbestosis
Silicosis
Berylliosis
Inhalation of coal dust (carbon + silica)
Diffuse interstitial fibrosis caused by inhalation of asbestos fibers (lower lobes)
Localized and nodular peribronchial fibrosis (upper lobes)

Sources: mining, stone cutting, glass manufacturing
Acute and chronic forms
Acute: diffuse pneumonitis by massive exposure to beryllium
Chronic: similar to sarcoidosis with granulomas, skin lesions, and hypercalcemia
Simple: no respiratory disability
Complicated: fibrosis (restrictive lung disease)
Insidious development (>15-20 years) post-exposure
-Nonspecific ILD findings
Exertional dyspnea (MC)
Cough with sputum production


Clinical diagnosis, HX of exposure to asbestos
Restrictive pulmonary function findings
Beryllium lymphocyte proliferation test

CXR: pleural plaques, hazy infiltrates and bilateral linear opacities
CXR: “egg shell” calcifications


No specific treatment

Increased risk of bronchogenic carcinoma (smoking synergistic) and malignant mesothelioma
Removal from exposure to silica

Increased risk of tuberculosis
Glucocorticoid therapy for both acute/chronic


Malignant Mesothelioma
  • Most cases secondary to asbestos exposure
  • Dyspnea, weight loss, cough, bloody effusion (common)
  • Prognosis is dismal


Idiopathic Pulmonary Fibrosis
  • Etiology: unknown, more common in male smokers
  • Prognosis: variable, but mean survival is 3-7 years after first diagnosis

Clinical Features
Diagnosis
Treatment
IPF
-Gradual onset of progressive dyspnea, nonproductive cough
CXR: ground glass or honeycombed appearance; may be normal
Exclude other cause of ILD
No effective treatment
->70% do not respond and experience gradual respiratory failure
-Supplemental O2, Steroids with or without cyclophosphamide, lung transplant


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