Respiratory Pathology

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jknell
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Respiratory Pathology
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2013-04-01 16:24:41
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  1. Rhinitis
    • Inflammation of the nasal mucosa
    • Adenovirus is the most common cause
    • Presentation: sneezing, congestion, runny nose
    • Allergic rhinitis: type I hypersensitivity reaction
    • - eosinophils
    • - associated with asthma and eczema
  2. Nasal polyp
    • Protrusion of edematous, inflamed nasal mucosa
    • Secondary to repeated bouts of rhinitus, CF, aspirin-intolerant asthma
  3. Angiofibroma
    • Benign tumor of nasal mucosa
    • -large blood vessels and fibrous tissue
    • Presentation: profuse epistaxis
    • Males >> females
  4. Nasopharyngeal carcinoma
    • Malignant tumor of nasopharyngeal epithelium
    • Associated with EBVAfrican children, Chinese adults
    • Bx: pleomorphic keratin-positive epithelial cells
  5. Acute epiglottitis
    • INflammation of epiglottis
    • H. influenzae type b is most common (non-immunized children)
    • Presentation: fever, sore throat, drooling with dysphagia, muffled voice, inspiratory stridor
    • Risk of airway obstruction
  6. Laryngotracheobronchitis (Croup)
    • Inflammation of the upper airway
    • Parainfluenza virus is most common cause
    • Presentation: hoarse, "barking" cough, inspiratory stridor
  7. Vocal cord nodule (Singer's nodule)
    • Nodule that arises on the true vocal cord
    • Cause: excessive use
    • Usually bilateral
    • Degenerative (Myxoid) connective tissue
    • resolves with rest
  8. Laryngeal papilloma
    • benign papillary tumor of the vocal cord
    • HPV 6 and 11
    • Usually single in adults, multiple in children
    • Presentation: hoarseness
  9. Laryngeal carcinoma
    • Squamous cell carcinoma
    • Origin: epithelial lining of the vocal cord
    • Risk factors: alcohol, tobacco
    • Presentation: hoarseness, cough, stridor
  10. Primary Pulmonary Hypertension
    • Epidemiology:
    • -young adult females

    • Presentation:
    • -exertional dyspnea
    • -R-sided heart failure

    • Pathophysiology:
    • -PAH ≥ 25 mmHg or > 35 mmHg during exercise
    • -results in arteriosclerosis, medial hypertrophy and intimal fibrosis of pulmonary arteries
    • -inactivating mutation in BMPR2 gene (normally inhibits vascular SM proliferation)

    • Complications:
    • -RVH
    • -eventual cor pulmonale

    • Prognosis:
    • -poor prognosis
  11. Secondary Pulmonary Hypertension
    • Pathophysiology:
    • -COPD (lung parenchyma destruction)
    • -Mitral stenosis (increase resistance leads to increased pressure)
    • -Recurrent thromboemboli (decrease cross-sectional area of vascular bed)
    • -Autoimmune disease (systemic sclerosis: inflammation → intimal fibrosis → medial hypertrophy)
    • -Left to Right Shunt (increase shear stress → endothelial injury)
    • -Sleep apnea/living at high altitude (hypoxic vasoconstriction)
  12. Deep Venous Thrombosis
    • Pathophysiology:
    • -most common source is femoral, iliac, or popliteal vein
    • -predisposed by Virchow's Triad
    •      -Stasis
    •      -Hypercoagulability
    •      -Endothelial damage

    • Presentation:
    • -Homans' Sign: calf pain with dorsiflexion of foot

    • Complications:
    • -can lead to PE

    • Treatment:
    • -heparin for prevention and acute management
    • -warfarin for long-term prevention of recurrence
  13. Pulmonary Emboli
    • Presentation: most often clinically silent
    • -sudden-onset dyspnea
    • -chest pain
    • -tachypnea
    • -may present as sudden death

    • Pathophysiology:
    • -95% arise from DVTs

    • Diagnosis:
    • -CT pulmonary angiography

    • Pathology:
    • -Fat (long bone fractures and liposuction)
    • -Air
    • -Thrombus
    • -Bacteria
    • -Amniotic fluid
    • -Tumor
    • "An embolus moves like a FAT BAT"
    • -lines of Zahn (platelets/fibrin and RBCs): only happen premortem

    • Fat Emboli:
    • -(long bone fractures and liposuction)
    • -Triad: hypoxemia, neurologic abnormalities, petechial rash

    • Amniotic Fluid Emboli:
    • -can lead to DIC, especially post partum
  14. Obstructive Lung Diseases
    -basic principles
    -PFTs
    -types
    • Pathophysiology:
    • -obstruction of air flow resulting in air trapping in the lungs
    • -airways close prematurely at high lung volumes
    • -V/Q mismatch

    • PFTs:
    • -↑ RV
    • -↑ TLC
    • -↓ FVC
    • -↓↓ FEV1
    • -↓ FEV1/FVC ratio

    • Types:
    • -Chronic Bronchitis
    • -Emphysema
    • -Asthma
    • -Bronchiectasis
  15. Chronic Bronchitis
    "Blue Bloater"

    • Pathophysiology:
    • -highly associated with smoking

    • Pathology:
    • -form of COPD
    • -hypertrophy of mucus-secreting glands in bronchi
    • -Reid index > 50% (thickness of gland layer/total wall thickness)

    • Presentation:
    • -productive cough for > 3 months per year for > 2 years
    • -wheezing
    • -crackles
    • -cyanosis (early onset hypoxemia due to shunting)
    • -late-onset dyspnea
  16. Emphysema
    -Centriacinar
    -Panacinar
    "Pink Puffer"

    • Pathophysiology:
    • -↑ elastase activity
    • -↑ lung compliance due to loss of elastic fibers
    • -destruction of alveolar air sacs

    • Centriacinar:
    • -associated with smoking
    • -inflammation leads to protease mediated damage
    • -mostly in upper lobes

    • Panacinar:
    • -α1-antitrypsin deficiency
    • -lack of antiprotease
    • -mostly in lower lobes
    • -also get liver cirrhosis

    • Presentation:
    • -dyspnea and cough (minimal sputum)
    • -pursed lip expiration
    • -weight loss
    • -barrel-chest
    • -late-onset hypoxemia
  17. Asthma
    -Pathophysiology
    -Diagnosis
    -Presentation
    -Pathology
    • Pathophysiology:
    • -bronchial hyperresponsiveness
    • -reversible bronchoconstriction
    • -can be triggered by viral URIs, allergens and stress
    • -most often due to type I hypersensitivity

    • Diagnosis:
    • -methacholine challenge

    • Presentation:
    • -cough
    • -wheezing
    • -tachypnea
    • -dypsnea
    • -hypoxemia
    • -↓ I/E ratio
    • -pulsus paradoxus
    • -mucus plugging

    • Pathology:
    • -smooth muscle hypertrophy
    • -Curschmann's spirals (shed epithelium forms mucus plugs)
    • -Charcot-Leyden crystals (formed from breakdown of eosinophils in sputum)
  18. Bronchiectasis
    -Pathophysiology
    -Causes
    -Presentations
    -Complications
    • Pathophysiology:
    • -chronic necrotizing infection of bronchi
    • -causes permanently dilated airways

    • Causes:
    • -Cystic Fibrosis
    • -Smoking (poor ciliary motility)
    • -Kartagener's Syndrome
    • -Allergic bronchopulmonary aspergillosis
    • -Bronchial obstruction

    • Presentation:
    • -purulent sputum (foul smelling)
    • -recurrent infections
    • -hempotysis

    • Complications:
    • -hypoxemia with cor pulmonale
    • -secondary AA amyloidosis
  19. Restrictive Lung Disease
    -Pathophysiology-PFTs
    -Types
    • Pathophysiology:
    • -restricted lung expansion causes decreased lung volumes

    • PFTs:
    • -↓ FVC
    • -↓ TLC
    • -FEV1/FVC > 80%

    • 1. Poor Breathing Mechanics
    • -Pathophysiology: extrapulmonary, periperal hypoventilation, normal A-a gradient
    • -poor muscular effort: polio, myasthenia gravis
    • -poor structural apparatus: scoliosis, morbid obesity

    • 2. Interstitial Lung Diseases
    • -Pathophysiology: pulmonary, lowered diffusing capacity, ↑ A-a gradient)
    • -ARDS
    • -Neonatal respiratory distress syndrome
    • -Pneumoconioses (anthracosis, silicosis, asbestosis)
    • -Sarcoidosis (bilateral hilar adenopathy, noncaseating granulomas, elevated ACE and Ca2+)
    • -Idiopathic pulmonary fibrosis
    • -Goodpasture's Syndrome
    • -Wegener's
    • -Langerhans cell histiocytosis (eosinophilic granuloma)
    • -Hypersensitivity pneumonitis
    • -Drug toxicity (bleomycin, busulfan, amiodarone, methotrexate)
  20. Pneumoconioses
    types, risk, lung region
    ↑ risk of cor pulmonale and Caplan's syndrome

    • Anthracosis: coal miner's lungs, "black lung" (upper lobes)
    • -mild exposure results in anthracosis; not clinically significant

    • Silicosis: sand blasting, foundries, mines (upper lobes)
    • -"Eggshell" calcification of hilar lymph nodes
    • -Macrophages respond to silica and release fibrogenic factors
    • -↑ susceptibility to TB
    • -↑ risk of bronchogenic carcinoma

    • Berylloisis: beryllium miners, workers in aerospace
    • -Noncaseating granulomas in the lung, hilar lymph nodes, systemic organs
    • -↑ risk for lung cancer

    • Asbestosis: ship building, roofing, plumbing (lower lobes)
    • -"Ivory white" calcified pleural plaques are pathognomonic of exposure, not precancerous
    • -↑ incidence of bronchogenic carcinoma and mesothelioma
    • -long, golden-brown fibers with associated iron  (asbestos bodies)
  21. Neonatal respiratory distress syndrome
    cause, risk factors, tx
    • Cause/features:
    • -Surfactant deficiency → ↑ surface tension, resulting in alveolar collapse
    • -Lecithin:sphingomyelin ratio <1.5 in amniotic fluid is predicitive
    • -Persistently low O2 tension → risk of PDA
    • -Diffuse granularity of lungs ("ground glass" appearance on CXR)

    • Risk factors:
    • -Prematurity
    • -Maternal diabetes (elevated fetal insulin)
    • -Cesarean delivery (↓ release of fetal glucocorticoids)

    • Tx:
    • -maternal steroids before birth
    • -artificial surfactant for infants
    • -*Supplemental O2 can result in retinopathy of prematurity and bronchopulmonary dysplasia
  22. Acute respiratory distress syndrome (ARDS)
    aka Adult respiratory distress syndrome
    Diffuse alveolar damage → ↑ alveolar capillary permeability → protein-rich fluid leakage into alveoli

    • Features:
    • -hyoxemia
    • -cyanosis
    • -respiratory distress
    • -"White-out" on chest X-ray

    • Cause: initial damage due to release of neutrophilic substances toxic to alveolar wall, activation of coag cascade, oxygen derived free radicals...
    • -Trauma
    • -Sepsis
    • -Shock
    • -Gastric aspiration
    • -Uremia
    • -Acute pancreatitis
    • -Amniotic fluid embolism

    • Histology:
    • -Formation of intra-alveolar hyalin membrane

    • Tx:
    • -Treat underlying cause
    • -Ventilation with PEEP
    • -**Most have normal lungs 1yr following
  23. Normal lung
    • Obstructive lung volumes > normal
    • ↑ TLC, ↑ FRC, ↑ RV
    • FEV1 is more dramatically reduced compared to FVC
    • Restrictive lung disease
    • Volumes < normal
  24. Sleep apneaa
    • -Repeated cessation of breathing > 10 seconds during sleep → disrupted sleep → daytime somnolence
    • -Hypoxia → ↑ EPO release → ↑ erythropoiesis
    • Central sleep apnea: no respiratory effort
    • Obstructive sleep apnea: respiratory effort against airway obstruction
    • -Associated with obesity, loud snoring, systemic/pulmonary hypertension, arrhythmias, and possibly sudden death

    Tx: weight loss, CPAP, surgery
  25. Pleural effusion
    • ↓ breath sounds
    • Dull to percussion
    • ↓ fremitus
    • no tracheal deviation
  26. Atelectasis (bronchial obstruction)
    • ↓ breath sounds
    • Dull to percussion
    • ↓ fremitus
    • Tracheal deviation toward side of lesion
  27. Spontaneous pneumothorax
    • ↓ breath sounds
    • Hyperresonant to percussion
    • ↓ fremitus
    • Tracheal deviation toward side of lesion
  28. Tension pneumothorax
    • ↓ breath sounds
    • Hyperresonant to percussion
    • ↓ fremitus
    • Tracheal deviation away from side of lesion
  29. Consolidation
    (lobar pneumonia, pulmonary edema)
    • Bronchial breath sounds; late inspiratory crackles
    • Dull to percussion
    • ↑ fremitus
    • No tracheal deviation
  30. Lung cancer
    presentation, complications, smoking
    • -Leading cause of cancer death
    • -Metastatic cancer is most common cause
    • (breast, colon, prostate, bladder cancer)
    • -Sites of metastases: adrenals, brain, bone (pathologic fracture), liver (jaundice, hepatomegaly)
    • Presentation:
    • -Cough
    • -hemoptysis
    • -bronchial obstruction
    • -wheezing
    • -weight loss
    • -pneumonic "coin" lesion on CXR
    • -noncalcified nodule on CT

    • ComplicationsSPHERE
    • -Superior vena cava syndrome
    • -Pancoast tumor
    • -Horner's syndrome
    • -Endocrine (paraneoplastic)
    • -Recurrent laryngeal symptoms (hoarseness)
    • -Effusions (pleural or pericardial)

    • Smoking:
    • -All are associated with smoking except brronchioalveolar and bronchial carcinoid
  31. Lung cancer
    Small cell vs non-small cell
    • Small cell carcinoma: 15%
    • -no surgical resection
    • -treat with chemotherapy

    • Non-small cell carcinoma: 85%
    • -Adenocarcinioma (40%)
    • -Squamous cell carcinoma (30%)
    • -Large cell carcinoma (10%)
    • -Carcinoid tumor (5%)
  32. Lung cancer
    Adenocarcinoma
    • Location: peripheral
    • -Most common lung cancer in nonsmokers and females
    • -Activating mutations in k-ras common
    • -Associated with hypertrophic osteoarthropathy (clubbing)

    • Bronchioloalvolar subtype: CXR often shows hazy infiltrates similar to pneumonia; excellent prognosis
    •  -Histology: grows along alveolar septa → apparent "thickening" of alveolar walls
  33. Squamous cell carcinoma
    • Location: Central
    • Hilar mass arising from bronchus
    • Cavitation
    • Cigarrettes (linked to smoking)
    • hyperCalcemia (produces PTHrP)
    • Most common tumor in male smokers
    • Histology: keratin pearls and intercellular bridges
  34. Small cell (oat cell) carcinoma
    • Central
    • Undifferentiated → very aggressive
    • May produce ACTH, ADH, or Antibodies against presynaptic calcium channels (Lambert-Eaton syndrome)
    • Amplification of myc oncogenes common
    • Male smokers
    • Tx: Inoperable; treated with chemotherapy
    • Histology: neoplasm of neuroendocrine Kulchitsky cells → small dark blue cells
  35. Large cell carcinoma
    • Location: peripheral (or central)
    • Highly anaplastic undifferentiated tumor (no keratin pearls, intracellular bridges, glands or mucin)
    • poor prognosis
    • less responsive to chemotherapy
    • surgical resection
    • Smoking
    • Histology: Pleomorphic giant cells
  36. Bronchial carcinoid tumor
    • Location: -
    • Excellent prognosis; metastasis is rare
    • Not smoking related
    • Sx usually due to mass effect
    • Occasionally carcinoid syndrome (serotonin secretion → flushing, diarrhea, wheezing)
    • Histology: Nets of neuroendocrine cells; chromogranin positive
  37. Mesothelioma
    • Location: Pleural
    • Malignancy of the pleura associated with asbestosis
    • Results in hemorrhagic pleural effusions and pleural thickening
    • Histology: Psammoma bodies
  38. Pancoast tumor
    • Carcinoma that occurs in apex of lung may affect cervical sympathetic plexus
    • Horner's syndrome: ipsilateral ptosis, miosis, anhidrosis
  39. Superior vena cava syndrome
    • Obstruction of the SVC that impairs blood drainage from:
    • -head ("facial plethora")
    • -neck (jugular venous distention)
    • -upper extremities (edema)

    • -Commonly caused by malignancy and thrombosis from indwelling catheters
    • -Medical emergency!
    • -Can raise intracranial pressure (if obstruction severe) → headaches, dizziness, and ↑ risk of aneurysm/rupture of cranial arteries
  40. Lobular Pneumonia
    organism, characteristics
    • LobarS. pneumoniae most frequently, Klebsiella
    • -Intra-alveolar exudates → consolidation
    • -May involve entire lung
    • -Phases: congestion → red hepatization → gray hepatization → resolution
  41. Bronchopneumonia
    organism, characteristics
    • S. pneumoniae, S. aureus, H. influenzae, Klebsiella
    • Acute inflammatory infiltrates from bronchioles into adjacent alveoli
    • Patchy distribution involving ≥ 1 lobe
  42. Interstitial (atypical) pneumonia
    organisms, characteristics
    • Viruses (influenza, RSV, adenovirus), Mycoplasma, Legionella, Chlamydia
    • Diffuse patchy inflammation localized to interstitial areas at alveolar walls
    • Distribution involving ≥ 1 lobe
    • Generally follows a more indolent course
    • -Atypical presentation: mild upper respiratory symtoms
  43. Aspiration pneumonia
    • Seen in pts at risk for aspiration (alcoholics and comatose patients)
    • Bacteria of the oropharynx (e.g., Bacteroides, Fusobacterium, Peptococcus)
  44. Lung abscess
    • Localized collection of pus within parenchyma
    • Causes:
    • -bronchial obstruction (e.g., cancer)
    • -aspiration of oropharyngeal contents (pts predisoposed to loss of consciousness - alcoholics, epileptics)
    • -Air-fluid levels seen on CXR
    • -S. aureus or anaerobes (Bacteroides, Fusobacterium, Peptostreptococcus)
  45. Tuberculosis
    Mycobacterium tuberculosis

    • Primaryfocal, caseating necrosis in the lower lobes and hilar LNs
    • -Generally asymptomatic

    • Secondary: reactivation in the apex of lung (high O2 tension)
    • -Cavitary foci of caseous necrosis
    • -may progress to miliary TB or TB bronchopneumonia
    • Features: nigh sweats, fevers, cough with hemoptysis, weight loss
    • -Bx: caseating granulomas
    • -AFB stain +
  46. Hypersensitivity pneumonitis
    • Mixed type III/IV hypersensitivity reaction to environmental antigen
    • Granulomatous reaction to inhaled organic antigens
    • Presentation: dyspnea, cough, chest tightness, headache
    • Who: farmers, those exposed to birds
    • Chronic exposure leads to interstitial fibrosis
  47. Pleural effusion
    transudative vs exudative
    Transudate: ↓ protein content

    -Due to CHF, nephrotic syndrome, hepatic cirrhosis

    Exudate: ↑ protein content, cloudy

    • -Due to malignancy, pneumonia, collagen vascular disease, trauma (occurs in states of ↑ vascular permeability)
    • -Must be drained in light of risk of infection
  48. Pleural effusions
    Lymphatic
    • aka chylothorax
    • Due to thoracic duct injury from trauma, malignancy
    • Milky-appearing fluid
    • ↑ triglycerides
  49. Pneumothorax
    presentation
    • Unilateral chest pain and dyspnea
    • unilateral chest expansion
    • ↓ tactile fremitus
    • hyperresonance
    • diminished breath sounds
  50. Spontaneous pneumonthorax
    • Accumulation of air in the pleural space
    • Most frequently in tall, thin, young males (caused by rupture of apical blebs)
    • Trachea deviates toward affected lung
  51. Tension pneumothorax
    • Usually occurs in setting of trauma or lung infection
    • Air is capable of entering pleural space, but not exiting
    • Trachea deviates away from affected lung

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