Pulmonology Diseases Flashcards by J S

Pulmonary Edema

Diseases of Impaired Gas Exchange

Pathophysiology: Alveoli fill with fluid, usually because of increased hydrostatic pressure
Etiologies: Left-sided heart failure
Symptoms: Dyspnea
Signs/Diagnostic Criteria: Congested, heavy, wet, red lungs
Treatment: Diuretics, O2, treat heart failure

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Pneumonia

Diseases of Impaired Gas Exchange

Pathophysiology: Inflammation of lung, usually infectious
Etiologies: Infection
Signs/Diagnostic Criteria: Consolidation, PMNs (polymorphonuclear leukocytes) in alveoli
Symptoms: Dyspnea
Treatment: Antibiotics, O2

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Pulmonary Hemorrhage

Diseases of Impaired Gas Exchange

Pathophysiology: Alveoli fill with blood, +/- fibrin, becomes hemosiderin if chronic
Etiologies: Goodpasture’s syndrome; Wegener’s granulomatosis
Symptoms: Dyspnea
Signs/Diagnostic Criteria: Blood-filled, very dark red lungs
Treatment: O2, treat underlying cause
Epi: premie

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Deep Vein Thrombosis (DVT)

Diseases of Vasculature

Pathophysiology: Thrombi form in deep calf veins, popliteal/femoral/iliac veins, UE (central line or Paget-Schroetter syndrome)…massive is +stroke or worse, >30% mortality
Etiologies: Genetic risk factors, Virchow’s triad (stasis, endothelial injury, hypercoagulability); lack of prophylaxis
Symptoms: Pain, swelling, erythema
Signs/Diagnostic Criteria: Asymmetry, warmth, edema, ischemia; Wells Score, D-dimer, ultrasound, venography
Treatment: Unfractionated Heparin (UFH)-can reverse with protamine > Low Molecular Weight Heparin (LMWH)/Fondapiranux; transition to oral warfarin > 3 months; for massive, can try catheter-directed thrombolysis or IVC filter
Epi: hospital, pregnant, casted

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Pulmonary Embolism (PE)
Diseases of Vasculature

Pathophysiology: Deep vein thrombi move to pulmonary arteries, lodge there, platelets release serotonin in response causing wheeze via vasoconstriction
Etiologies: Genetic risk factors, Virchow’s triad (stasis, endothelial injury, hypercoagulability); lack of prophylaxis
Symptoms: Dyspnea, pleuritic pain, cough, wheezing
Signs/Diagnostic Criteria: Modified Wells Score, D-dimer, CT angiography; hypoxemia, hypocapnia, respiratory alkalosis
Treatment: Low Molecular Weight Heparin (LMWH) for hemodynamically stable patients; Unfractionated Heparin (UFH) in unstable patients, or with high risk of bleeding; warfarin > 3 months
Epi: Hospital, pregnant, casted

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Pulmonary Hypertension

Diseases of Vasculature

Pathophysiology: Mean Pulmonary Arterial pressure > 25 mm Hg at rest; due to either increased Cardiac Output, increased Left Atrial Pressure, or increased Pulmonary Vascular Resistance
Etiologies: Small Pulmonary Artery vasculopathy (PAH) most common cause
Symptoms: Dyspnea, fatigue, chest/abdominal pain, syncope
Signs/Diagnostic Criteria: Hypoxemia, tachycardia, hypotension, right heart hypertrophy, no rales
Treatment: Calcium Channel Blockers if vasoreactive, Prostanoids, PDE-5 inhibitors (Phosphodiesterase 5 inhibitors), endothelin receptor antagonists, surgery
Epi: Connective tissue disorders

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Vasculitis

Diseases of Vasculature

Pathophysiology: Inflammation of pulmonary vessels
Etiologies: Primary: autoimmune; Secondary: infectious
Symptoms:
Signs/Diagnostic Criteria: Macro nodules
Treatment: Treat underlying cause

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Cystic Fibrosis

Diseases of Ion Channel Defect

Pathophysiology: CFTR gene defect on chromosome 7q, commonly deltaF508, inheritance = AR (1/30 Caucasians are carriers), Cl- channel is defective –> secretion of thick mucous in lungs, pancreas, liver, obstructive ventilatory defect
Etiologies: Genetic
Symptoms: Recurrent respiratory infections (P.Aeruginosa), productive cough, weight loss, dyspnea
Signs/Diagnostic Criteria: Lung inflammation, end-stage bronchiectasis, obstruction, clubbing, V/Q mismatch, really decreased RV/TLC (residual volume/total lung capacity), hyperinflation, air trapping, salty sweat, pancreatic insufficiency
Treatment: Accredited CF center! Mechanical airway clearance, Pulmozyme (recombinant DNAase mucolytic), inhaled hypertonic saline, tobramycin/aztreonam, steroids/ibuprofen/azithromycin, CFTR potentiators/correctors, gene therapy?
Epi: white folks

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Exudative Pleural Effusion

Pleural Diseases

Pathophysiology: Either infection (empyema), malignancy (primary mesothelioma or secondary metastatic breast cancer), or chyle (chylothorax)/blood(hemothorax)/ascites (hepatic hydrothorax) gets into the pleural space
Etiologies: Infection: bacterial or TB; Malignancy: Other fluids from trauma or collagen vascular disease
Symptoms: Pleuritic chest pain, dyspnea, dizziness, trapped lung
Signs/Diagnostic Criteria: Air-fluid level on chest x-ray, free-flowing fluid, soft tissue windows on CT, high metabolic activity on PET
Treatment: Thoracentesis, chest tube drainage, direct pleural examination

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Transudative Pleural Effusion

Pleural Diseases

Pathophysiology: Imbalance of pleural fluid production
Etiologies: Heart/renal/liver failure
Symptoms: Pleuritic chest pain, dyspnea, dizziness, trapped lung
Signs/Diagnostic Criteria: Air-fluid level on chest x-ray, free-flowing fluid, soft tissue windows on CT, high metabolic activity on PET
Treatment: Thoracentesis, chest tube drainage, direct pleural examination

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Pneumothorax

Pleural Diseases

Pathophysiology: Leakage of air into pleural space
Etiologies: Trauma
Symptoms: Pleuritic chest pain, dyspnea, dizziness, trapped lung
Signs/Diagnostic Criteria: Collapsed lung on chest x-ray, increased heart rate, respiratory rate, deviated trachea
Treatment: thoracentesis, chest tube drainage, direct pleural examination

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Obstructive Sleep Apnea (OSA)

Sleep-Related Diseases

Pathophysiology: Respiratory effort vs. airway obstruction
Etiologies:
Symptoms: Fatigue, daytime sleepiness, snoring, poor sleep quality/insomnia, awakening with choking/gasping, cerebrovascular accident (CVA), hypertension, decreased left ventricular function, death
Signs/Diagnostic Criteria: cessation of airflow > 10 seconds
Treatment: weight loss, sleep positioning, alcohol/sedative avoidance, oral devices, CPAP (continuous positive airway pressure)
Epi: obesity, hypertension

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Overlap Syndrome (COPD + Obstructive Sleep Apnea)
Sleep-Related Diseases

Pathophysiology: respiratory effort vs. airway obstruction and also severe hypoxemia, paradoxical breathing, worse outcomes vs. obstructive sleep apnea alone
Etiologies: COPD + OSA (obstructive sleep apnea)
Symptoms: fatigue, daytime sleepiness, snoring, poor sleep quality/insomnia, awakening with choking/gasping, cerebrovascular accident (CVA), hypertension, decreased left ventricular function, death
Signs/Diagnostic Criteria: cessation of airflow > 10 seconds
Treatment: nocturnal O2/ventilation
Epi: obesity, hypertension

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Cheyne-Stokes Breathing with Central Sleep Apnea (CSB/CSA)

Sleep-related diseases

Pathophysiology: periods of no respiratory effort, arousal from hyperventilation, disrupted sympathovagal balance
Etiologies: CHF stimulates vagal receptors in lung
Symptoms: fatigue, daytime sleepiness, snoring, poor sleep quality/insomnia, awakening with choking/gasping, cerebrovascular accident (CVA), hypertension, decreased LV function, death
Signs/Diagnostic Criteria: cessation of airflow >10 seconds
Treatment: treat CHF, give O2, CPAP (continuous positive airway pressure), maybe CO2, drugs, Left ventricular assist device (LVAD)
Epi: obesity, hypertension

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Central Hypoventilation Syndrome (CHS)

Sleep-related diseases

Pathophysiology: periods of no respiratory effort, extremely irregular
Etiologies: rare congenital
Symptoms: fatigue, daytime sleepiness, snoring, poor sleep quality/insomnia, awakening with choking/gasping, cerebrovascular accident (CVA), hypertension, decreased left ventricular function, death
Signs/Diagnostic Criteria: cessation of airflow > 10 seconds
Treatment: ventilation
Epi: obesity, hypertension

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Hypopnea

Sleep-related diseases

Pathophysiology: obstructive or non-obstructive
Etiologies:
Symptoms: fatigue, daytime sleepiness, snoring, poor sleep quality/insomnia, awakening with choking/gasping, cerebrovascular accident (CVA), hypertension, decreased left ventricular function, death
Signs/Diagnostic Criteria: decreased airflow for > 10 seconds
Treatment:

Chronic Bronchitis
Obstructive diseases (COPD)

Pathophysiology: hypertrophy of mucous glands + goblet cells in bronchioles, basement membrane thickening
Etiologies: smoking exacerbates
Symptoms: wheeze, productive cough, dyspnea (especially exertional), muscular wasting
Signs/Diagnostic Criteria: airway edema + fibrosis, cyanosis, hyperinflation, decreased breath sounds, black lung fields on CT, reduced FEV1/FVC ratio (<70% expiration), impaired gas exchange (V/Q mismatch), increased dead space, hypoventilation
Treatment: Stage I: risk factor reduction, flu/pneumo vaccine, SABA (short-acting beta agonists). Stage II: LABA (long-acting beta2 agonists), rehab. Stage III: inhaled corticosteroids. Stage IV: long-term O2 therapy, surgery. SMOKING cessation.
Epi: Smokers

Emphysema (Centriacinar)
Obstructive Diseases (COPD)

Pathophysiology: destruction of acinar walls by increased elastase activity –> loss of radial traction, increased lung compliance
Etiologies: smoking, mostly affects upper lobe, spares alveoli
Symptoms: wheeze, productive cough, dyspnea (especially exertional), muscular wasting
Signs/Diagnostic Criteria: airway edema + fibrosis, cyanosis, hyperinflation, decreased breath sounds, black lung fields on CT, reduced FEV1/FVC ratio (<70% expiration), impaired gas exchange (V/Q mismatch), increased dead space, hypoventilation
Treatment: Stage I: risk factor reduction, flu/pneumo vaccine, short-acting beta agonists; Stage II: long-acting beta agonists, rehab; Stage III: inhaled corticosteroids; Stage IV: long-term O2 therapy, surgery. SMOKING cessation
Epi: smokers

Bronchiectasis
Obstructive diseases (COPD)

Pathophysiology: chronic necrotizing infection of bronchi –> permanently dilated airways, hemoptysis
Etiologies: bronchial obstruction, Kartagener’s, smoking, CF
Symptoms: hemoptysis, productive cough
Signs/Diagnostic Criteria: “soap bubble” appearance on chest xray, polymorphonuclear leukocytes (PMNs) and mucous in airways on histology
Treatment: treat underlying cause

alpha1-antitrypsin (AAT) deficiency (aka Panacinar Emphysema)
Obstructive diseases (COPD)

Pathophysiology: congenital cause of COPD; autosomal codominant, mutation in SERPINA1 gene leads to excess protease activity, increased lung compliance
Etiologies: mostly affects lower lobes
Symptoms: wheeze, productive cough, dyspnea (especially exertional), muscular wasting
Signs/Diagnostic Criteria: airway edema + fibrosis, cyanosis, hyperinflation, decreased breath sounds, black lung fields on CT, *reduced FEV1/FVC ratio (<70% expiration), impaired gas exchange (V/Q mismatch), increased dead space, hypoventilation
Treatment: Same + IV pooled alpha1 antitrypsin
Epi: 2% of COPD patients

Bronchiolitis
Obstructive diseases (COPD)

Pathophysiology: Inflammation of bronchioles, fibrosis
Etiologies: RSV, allergic/hypersensitivity
Symptoms:
Signs/Diagnostic Criteria: evidence of fibrosis, inflammation on histology
Treatment: treat underlying cause

Bronchiolitis Obliterans
Obstructive diseases (COPD)

Pathophysiology: Inflammation of bronchioles
Etiologies: Lung transplant, connective tissue disease
Symptoms:
Signs/Diagnostic Criteria: evidence of fibrosis, inflammation on histology
Treatment: treat underlying cause

Asthma
Obstructive diseases (COPD)

Pathophysiology: airflow obstruction (reversible), bronchial hyperresponsiveness, V/Q mismatch during attacks, alveolar hyperventilation during attacks (hypoventilation if severe, due to fatigue + dead space); Th2 phenotype
Etiologies: Genetic (FCeR1, CD14, IL-4, IFN, etc.), environment (URIs, stress, allergen re-exposure), prenatal exposures
Symptoms: wheeze, cough, chest tightness during attacks
Signs/Diagnostic Criteria: decline in FEV1 of 20% from baseline, pulsus paradoxus, hypoxemia, hyperinflation, thick wall/narrow lumen, mucous plugging
Treatment: inhaled corticosteroids, long-acting beta agonists; O2/intubation if severe; Omalizumab is useful in high-risk patients, binds all circulating IgE, blocks mast cell cross-linking without affecting complement
Epi: clean kids

Idiopathic Pulmonary Fibrosis (IPF)/Usual Interstitial Pneumonia (UIP)
Restrictive diseases (ILD)

Pathophysiology: fibrosis and thickening of alveolar walls and interstitium, honeycomb lung appearance, bases > apices
Etiologies: idiopathic; can also have drug-induced, radiation-related, or connective tissue disease-related ILD
Symptoms: dyspnea (exertional), dry cough
Signs/Diagnostic Criteria: clubbing, hypoxemia, bibasilar crackles, impaired gas exchange, dermatomyositis, Raynaud’s, sclerodactyly, positive six-minute walk test, ground glass opacity/reticular opacity/honeycombing, TLC < 75% expected
Treatment: O2, pulmonary rehab, avoid lung injury/antigen, anti-inflammatory therapy (corticosteroids), lung transplant
Epi: older male smoker with family history

``` Pneumonoconiosis Restrictive diseases (ILD) ```

Pathophysiology: inorganic antigen leads to inflammation Etiologies: silicosis, asbestosis Symptoms: dyspnea (exertional), dry cough Signs/Diagnostic Criteria: clubbing, hypoxemia, bibasilar crackles, impaired gas exchange, dermatomyositis, Raynaud's, sclerodactyly, positive six-minute walk test, ground glass opacity/reticular opacity/honeycombing, *TLC < 75% expected* Treatment: O2, pulmonary rehab, avoid lung injury/antigen, anti-inflammatory therapy (corticosteroids), lung transplant Epi: Workers

``` Sarcoidosis Restrictive disease (ILD) ```

Pathophysiology: systemic non-necrotizing granulomatous disease, usually near airways; multi-system, can also get skin plaques, lupus pernio, uveitis with synechiae, hilar adenopathy on chest xray Etiologies: unknown; often reversible Symptoms: dyspnea (exertional), dry cough Signs/Diagnostic Criteria: clubbing, hypoxemia, bibasilar crackles, impaired gas exchange, dermatomyositis, Raynaud's, sclerodactyly, positive six-minute walk test, ground glass opacity/reticular opacity/honeycombing, *TLC < 75% expected* Treatment: O2, pulmonary rehab, avoid lung injury/antigen, anti-inflammatory therapy (corticosteroids), lung transplant Epi: Northern Europe, African-American

``` Hypersensitivity Pneumonitis Restrictive diseases (ILD) ```

Pathophysiology: CD4+ Th1 humoral immune response overreacts, leading to Type III + IV hypersensitivity, centrilobular nodules, airway thickening + fibrosis Etiologies: birds, fungi, bacteria, chemicals Symptoms: dyspnea (exertional), dry cough Signs/Diagnostic Criteria: clubbing, hypoxemia, bibasilar crackles, impaired gas exchange, dermatomyositis, Raynaud's, sclerodactyly, positive six-minute walk test, ground glass opacity/reticular opacity/honeycombing, *TLC < 75% expected* Treatment: O2, pulmonary rehab, avoid lung injury/antigen, anti-inflammatory therapy (corticosteroids), lung transplant Epi: Birds

``` Acute Respiratory Distress Syndrome (ARDS) Restrictive diseases (respiratory failure) ```

Pathophysiology: diffuse alveolar damage, increased alveolar capillary permeability, inflammatory injury from neutrophils, necrosis lead to hyaline membranes Etiologies: pulmonary infection, shock, sepsis, burns, near-drowning, etc. Symptoms: acute onset tachypnea, dyspnea Signs/Diagnostic Criteria: hyaline membranes, hypoxemia, diffuse crackles, bilateral infiltrates, consolidation Treatment: treat underlying cause, lung protective ventilation (6 cc/kg VT), max plateau of 30 cm H2O, conservative fluids, ECMO (extracorporeal membrane oxygenation)

``` Neonatal Respiratory Distress Syndrome Restrictive diseases (respiratory failure) ```

Pathophysiology: congenital surfactant deficiency, alveolar collapse Etiologies: maternal diabetes, C-section, premature Symptoms: dyspnea, hypoxia Signs/Diagnostic Criteria: hyaline membranes, hypoxemia, diffuse crackles, bilateral infiltrates, consolidation Treatment: maternal steroids, artificial surfactant Epi: premie

``` Guillain-Barre Restrictive diseases (normal lungs) ```

Pathophysiology: for a variety of reasons, muscles responsible for breathing are dystrophied or wasted or not innervated properly, resulting in increased work of breathing, failure to ventilate/oxygenate Etiologies: Symptoms: respiratory failure, restrictive ventilatory defect, hypoxia, *hypercapnia*, respiratory acidosis Signs/Diagnostic Criteria: respiratory failure, restrictive ventilatory defect, hypoxia, *hypercapnia*, respiratory acidosis Treatment: supplemental oxygen, mechanical ventilation, non-invasive ventilation

``` ALS (Amyotrophic Lateral Sclerosis) Restrictive diseases (normal lungs) ```

Pathophysiology: for a variety of reasons, muscles responsible for breathing are dystrophied or wasted or not innervated properly, resulting in increased work or breathing, failure to ventilate/oxygenate Etiologies: Symptoms: dyspnea, tachypnea Signs/Diagnostic Criteria: respiratory failure, restrictive ventilatory defect, hypoxia, *hypercapnia*, respiratory acidosis Treatment: supplemental oxygen, mechanical ventilation, non-invasive ventilation

``` Multiple Sclerosis Restrictive diseases (normal lungs) ```

Pathophysiology: for a variety of reasons, muscles responsible for breathing are dystrophied or wasted or not innervated properly, resulting in increased work of breathing, failure to ventilate/oxygenate Etiologies: Symptoms: dyspnea, tachypnea Signs/Diagnostic Criteria: respiratory failure, restrictive ventilatory defect, hypoxia, *hypercapnia*, respiratory acidosis Treatment: supplemental oxygen, mechanical ventilation, non-invasive ventilation

``` Muscular Dystrophy Restrictive diseases (normal lungs) ```

Pathophysiology: for a variety of reasons, muscles responsible for breathing are dystrophied or wasted or not innervated properly, resulting in increased work of breathing, failure to ventilate/oxygenate Etiologies: Symptoms: dyspnea, tachypnea Signs/Diagnostic Criteria: respiratory failure, restrictive ventilatory defect, hypoxia, *hypercapnia*, respiratory acidosis Treatment: supplemental oxygen, mechanical ventilation, non-invasive ventilation

``` Myasthenia Gravis Restrictive diseases (normal lung) ```

Pathophysiology: for a variety of reasons, muscles responsible for breathing are dystrophied or wasted or not innervated properly, resulting in increased work of breathing, failure to ventilate/oxygenate Etiologies: Symptoms: dyspnea, tachypnea Signs/Diagnostic Criteria: respiratory failure, restrictive ventilatory defect, hypoxia, *hypercapnea*, respiratory acidosis Treatment: supplemental oxygen, mechanical ventilation, non-invasive ventilation