Pulmonology Review for Rotation Exams

Pulmonology for 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

• 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 resistance → thickening 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