Medicine Marrow Pg 231-240 (Pulmonology)
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Questions and Answers

Which of the following is a cause of primary ARDS?

  • Smoke inhalation
  • Non-pulmonary sepsis
  • Pulmonary sepsis (correct)
  • Drowning
  • The fibrotic vasoconstriction phase occurs between 14-21 days after the onset of ARDS.

    True

    What is the hallmark of the acute exudative phase of ARDS?

    Eosinophilic hyaline membrane deposition

    The loss of surfactant in ARDS leads to __________ of the alveoli.

    <p>collapse</p> Signup and view all the answers

    Match the following phases of ARDS with their descriptions:

    <p>Acute exudative phase = Interstitial + alveolar edema and eosinophilic hyaline membrane Fibroproliferative phase = Repair processes and fibrosis begin Fibrotic vasoconstriction = Vascular changes and reduced compliance</p> Signup and view all the answers

    Which of the following conditions is NOT prone to develop interstitial lung disease (ILD)?

    <p>Asthma</p> Signup and view all the answers

    Blameycin is known to be a drug that can induce interstitial lung disease.

    <p>True</p> Signup and view all the answers

    What pattern of ILD is most common in rheumatoid arthritis?

    <p>UIP</p> Signup and view all the answers

    Eosinophilic granulomatosis polyangiitis is associated with ___ lobe ILD.

    <p>upper</p> Signup and view all the answers

    Match the following ILD types with their characteristics:

    <p>UIP = Idiopathic lung disease often seen in rheumatoid arthritis NSIP = Subacute restrictive lung disease more common in young females COP = Most common in inflammatory muscle diseases LIP = Associated with Sjogren's syndrome and HIV</p> Signup and view all the answers

    What does elevated $P_{aCO_2}$ indicate in the diagnosis of respiratory failure?

    <p>Hypoventilation</p> Signup and view all the answers

    A V/Q ratio of infinity indicates a shunt.

    <p>False</p> Signup and view all the answers

    What should be evaluated when there is an elevated $P_{aO_2}$?

    <p>Possible V/Q mismatch or shunting</p> Signup and view all the answers

    If the patient is hypoventilating and the $P_{O_2}$ is low and corrected with oxygen, this may indicate _____ altitude.

    <p>high</p> Signup and view all the answers

    Match the hypoventilation types with their corresponding indicators:

    <p>Type I = Likely hypoventilation Type II = Not corrected with oxygen Type III = Other underlying mechanism</p> Signup and view all the answers

    Which of the following is a symptom specific to bronchiectasis?

    <p>Wheeze</p> Signup and view all the answers

    Hypoxia is a common sign of ILD.

    <p>True</p> Signup and view all the answers

    What is the most significant factor for prognosis in ILD?

    <p>Pulmonary hypertension</p> Signup and view all the answers

    CXR findings in ILD typically display bilateral bibasal __________ infiltrates.

    <p>subpleural reticular/reticulo-nodular</p> Signup and view all the answers

    Match the following conditions with their corresponding features:

    <p>Asbestosis = Pleuritic pain Sarcoidosis = Erythema nodosum Neurofibromatosis = Cafe-au-lait spots Scleroderma = Raynaud's phenomenon</p> Signup and view all the answers

    What is the typical V/Q ratio in healthy lungs?

    <p>0.8</p> Signup and view all the answers

    A patient with type III respiratory failure has decreased functional residual capacity.

    <p>True</p> Signup and view all the answers

    List two common etiologies of type III respiratory failure.

    <p>Obesity, Supine position</p> Signup and view all the answers

    In type III respiratory failure, the pathology includes decreased functional residual capacity, which is comprised of residual volume and _______.

    <p>expiratory reserve volume</p> Signup and view all the answers

    Match the type of respiratory failure with its characteristic features:

    <p>Type III = Decreased functional residual capacity Type IV = Hypotension Type I = Oxygenation failure due to ARDS Type II = Ventilatory failure</p> Signup and view all the answers

    What imaging technique is primarily used to determine the pattern and prognosis of interstitial lung disease?

    <p>High-Resolution Computed Tomography (HRCT)</p> Signup and view all the answers

    Ground glass opacification is a feature that may be present in Usual Interstitial Pneumonia (UIP).

    <p>False</p> Signup and view all the answers

    What pulmonary function test finding is associated with the initial phase of interstitial lung disease?

    <p>Impaired DLCO, Normal FEV, FVC</p> Signup and view all the answers

    Traction bronchiectasis is a finding associated with ________________.

    <p>Usual Interstitial Pneumonia (UIP)</p> Signup and view all the answers

    Match the following drugs with their action in treating UIP-ILD:

    <p>Pirfenidone = Anti-TGF Nintedanib = Anti-FDGF, anti-VEGF, anti-PDGFR</p> Signup and view all the answers

    What does the abbreviation DAD stand for in the context of lung injury?

    <p>Diffuse Alveolar Damage</p> Signup and view all the answers

    The pulmonary capillary wedge pressure (PCWP) is used to measure right atrial pressure.

    <p>False</p> Signup and view all the answers

    What is one significant indicator of non-cardiogenic pulmonary edema?

    <p>Pulmonary capillary wedge pressure (PCWP)</p> Signup and view all the answers

    The condition recognized by terms such as shock lung and acute alveolar injury is known as __________.

    <p>ARDS</p> Signup and view all the answers

    Match the following terms with their descriptions:

    <p>Non-cardiogenic pulmonary edema = Fluid accumulation in the lungs without heart failure DAD = Diffuse Alveolar Damage AECC = American European Consensus Conference PCWP = Measurement of left atrial pressure</p> Signup and view all the answers

    Which of the following is NOT characteristic of Non-Specific Interstitial Pneumonia (NSIP)?

    <p>Destruction of lung parenchyma</p> Signup and view all the answers

    Amiodarone can cause interstitial lung disease with an NSIP pattern.

    <p>True</p> Signup and view all the answers

    What is the best treatment for Non-Specific Interstitial Pneumonia (NSIP)?

    <p>Mycophenolate mofetil (MMF)</p> Signup and view all the answers

    In Bronchiolitis Obliterans Organising Pneumonia, the presence of polypoid plugs is referred to as __________.

    <p>Masson's buds</p> Signup and view all the answers

    Match the following lung conditions with their primary associations:

    <p>LAM = Tuberous sclerosis LCH = Smoking LIP = Sjogren's syndrome COP/BOOP = Polymyositis</p> Signup and view all the answers

    Which symptom is NOT typically associated with acute respiratory distress syndrome (ARDS)?

    <p>Hypertension</p> Signup and view all the answers

    Mechanical ventilation is considered the mainstay of treatment for respiratory failure.

    <p>True</p> Signup and view all the answers

    Which of the following is the most common type of idiopathic interstitial pneumonia?

    <p>Idiopathic pulmonary fibrosis/usual interstitial pneumonia (UIP)</p> Signup and view all the answers

    What are bilateral crackles indicative of in patients with respiratory distress?

    <p>Diffuse pulmonary infiltrates</p> Signup and view all the answers

    Sarcoidosis is classified as a type of idiopathic interstitial pneumonia.

    <p>False</p> Signup and view all the answers

    In VILI, high positive end expiratory pressure (PEEP) is used to treat _________.

    <p>Atelectrauma</p> Signup and view all the answers

    What is the primary pathogenic fibrogenic cytokine associated with interstitial lung disease?

    <p>TGF-β</p> Signup and view all the answers

    Match the following conditions with their differential diagnoses of acute breathlessness:

    <p>Pulmonary edema = Unless proven otherwise Pulmonary embolism = Possible obstruction of blood flow Pneumothorax = Air in the pleural space Acute exacerbation of asthma = Reversible airway obstruction</p> Signup and view all the answers

    The connective tissue that holds the lungs together is called the __________.

    <p>interstitium</p> Signup and view all the answers

    Match the following types of interstitial lung disease with their characteristics:

    <p>Idiopathic pulmonary fibrosis = Most common idiopathic interstitial pneumonia Hypersensitivity pneumonitis = Inflammatory response to inhaled organic particles Cryptogenic organizing pneumonia = Third most common type of idiopathic interstitial pneumonia Desquamative interstitial pneumonia = Associated with smoking</p> Signup and view all the answers

    Study Notes

    Etiology of ARDS

    • Primary ARDS: caused by conditions directly affecting the lungs, including pulmonary sepsis, massive blood transfusion, acute pancreatitis, and transfusion-associated lung injury (TRALI)
    • Secondary ARDS: caused by conditions outside the lungs, such as smoke inhalation, drowning, post-surgery complications, non-pulmonary sepsis, and major trauma or burns

    Pathogenesis of ARDS

    • Alveolar epithelial injury: initiated by toxins, sepsis, or other insults
    • Inflammatory mediators: release of interleukin-1 (IL-1), IL-6, tumor necrosis factor-alpha (TNF-α), and other cytokines
    • Neutrophil recruitment: attraction of neutrophils to the injured area
    • Exudation: accumulation of proteinaceous fluid and debris in the alveolar space
    • Surfactant loss: reduced surfactant production leads to alveolar collapse
    • Impaired gas exchange: decreased lung compliance results in ineffective gas exchange
    • Capillary endothelial injury: damage to the blood vessels in the lungs

    Respiratory Failure Diagnostic Flowchart

    • Elevated PaCO2P_{aCO_2}PaCO2​​: indicates hypercapnic respiratory failure, requiring further investigation
    • Elevated PaO2P_{aO_2}PaO2​​ - PaO2P_{aO_2}PaO2​​: suggests V/Q mismatch or shunting
      • V/Q = 0: Shunt (e.g., problem in blood flow)
      • V/Q = ∞: Dead space (e.g., problem in ventilation)
    • Hypoventilation: indicates a problem with breathing mechanics, further classification needed
      • Type II Hypoventilation: PO2P_{O_2}PO2​​ low but correctable with oxygen, may be due to high altitude or other underlying mechanisms
      • Type I Hypoventilation: other hypoventilation mechanisms
    • V/Q mismatch: further analysis required to determine the underlying cause

    Autoimmune ILD

    • ILD can develop in individuals with autoimmune conditions:
      • Rheumatoid arthritis
      • Diffuse systemic sclerosis
      • Polymyositis/dermatomyositis
      • Mixed connective tissue disease (MCTD)
      • Sjogren's syndrome
      • Ankylosing spondylitis

    Miscellaneous ILDs

    • Occupational ILDs:
      • Asbestosis: lower lobe ILD
      • Silicosis
      • Berylliosis
      • Coal-worker’s pneumoconiosis
    • Vasculitis-related ILD:
      • Eosinophilic granulomatosis polyangiitis (EGPA)/Churg-Strauss syndrome: upper lobe ILD
    • Inherited forms of ILD:
      • Neurofibromatosis
      • Tuberous sclerosis
    • Drug-induced ILD:
      • Bleomycin
    • Langerhan’s cell histiocytosis (LCH): Associated with smoking.
    • Lymphangiomyomatosis (LAM):

    Key Characteristics of ILD Patterns

    • UIP/IPF:
      • Idiopathic
      • Males > females
      • Age: 50-70 years
      • Associated with smoking
    • NSIP:
      • Young females
      • Subacute restrictive process
      • Associations: Drug, Connective tissue disease
    • COP:
      • Most common in inflammatory muscle disease: Polymyositis, Dermatomyositis
    • LIP:
      • Associated with Sjogren syndrome and HIV

    Clinical Features of ILD

    • Symptoms:
      • Dry, non-productive cough
      • Dyspnea on exertion
      • Fever and arthralgia (Sarcoidosis)
      • Wheezing (Bronchiectasis)
      • Pleuritic pain (Asbestosis)
      • Hemoptysis (Rare)
    • Signs:
      • Auscultation:
        • ILD: Bilateral bibasal, fine “velcro crackles” at the end of expiration
        • Bronchiectasis: Unilateral, coarse crackles heard early in inspiration
      • Other:
        • Cafe-au-lait spots (Neurofibromatosis)
        • Erythema nodosum (Sarcoidosis)
        • Raynaud's phenomenon (Scleroderma)
        • Hypoxia

    Investigations for ILD

    • CXR: Bilateral bibasal subpleural reticular/reticulo-nodular infiltrates
    • HRCT: Investigation of choice to determine pattern and prognosis of ILD

    The Interstitium

    • Connective tissue that holds the lungs together, including alveolar, subpleural, lobar, vascular, axial, and lung parenchyma

    ILD Classification

    • Idiopathic Interstitial Pneumonia (most common)
    • Autoimmune ILD
    • Hypersensitivity pneumonitis
    • Sarcoidosis
    • Miscellaneous

    Idiopathic Interstitial Pneumonia Types

    • Idiopathic pulmonary fibrosis/usual interstitial pneumonia (UIP): Most common
    • Idiopathic nonspecific interstitial pneumonia (NSIP):
    • Cryptogenic organizing pneumonia (COP): Third most common
    • Respiratory bronchiolitis interstitial lung disease (RB-ILD)
    • Desquamative interstitial pneumonia (DIP)
    • Acute interstitial pneumonia:
    • Lymphocytic interstitial pneumonia (LIP)

    Type 3 Respiratory Failure

    • Pathology: Decreased Functional Residual Capacity (Residual volume + Expiratory Reserve Volume)
    • Etiology:
      • Supine position, obesity, ascites
      • Residual effects of anesthesia
      • Upper abdominal incision
      • Airway secretions
      • Collapse (Post op Atelectasis)

    Hypoxemic Respiratory Failure - V/Q Matching

    • Normal V/Q Ratio: 0.8
    • Upper Zone: >1 (Non-dependent area → ↓v, ↓↓Q)
    • Lower Zone: <1 (Dependent area → ↑v, ↓Q)

    Clinical Features of ARDS

    • Rapid onset dyspnea
    • Hypoxemia
    • Bilateral crackles
    • Tachypnea, tachycardia, diaphoresis
    • Pulmonary hypertension

    ARDS Management

    • Mechanical ventilation:
      • Inverse ratio ventilation
      • Prone ventilation
      • Permissive hypercapnia

    VILI (Ventilation Induced Lung Injury)

    • Pathogenesis:
      • Volume Trauma: Increased lung volume
      • Barotrauma: Increased transpulmonary pressure
      • Atelectrauma: Collapse and decollapse of alveoli during breathing
    • Treatment:
      • Low tidal volume ventilation
      • Low plateau pressure
      • High positive end expiratory pressure (PEEP)

    Differential Diagnosis of Acute Breathlessness

    • Respiratory:
      • Pulmonary edema
      • Pulmonary embolism
      • Pneumothorax
      • Acute exacerbation of asthma
    • Cardiovascular:
      • Aortic dissection
      • Myocardial infarction

    PFT Findings in ILD

    • Initial Phase: Impaired DLCO (Diffusion capacity of lung for carbon monoxide); Normal FEV (Forced Expiratory Volume in 1 second); FVC (Forced Vital Capacity)
    • Advanced Disease: Reduced FVC; Normal or increased FEV/FVC ratio

    HRCT Findings in ILD

    • UIP:

      • Suggestive Features:
        • Linear shadows (Intralobular septal thickening)
        • Reticular shadows (Net of linear opacities)
        • Subpleural, basal predominant
        • Honeycombing (Small, polyhedral cystic spaces)
        • Loss of lung architecture
        • Traction bronchiectasis
        • Nodules
      • Absent Features:
        • Ground glass opacification
    • Other Findings:

      • Honeycombing
      • Reticular shadows
      • Linear shadows
      • Traction bronchiectasis
    • New Drugs for UIP-ILD:

      • Pirfenidone (Anti-TGF)
      • Nintedanib (Anti-FDGF, anti VEGF, anti PDGFR)

    Characteristics of NSIP

    • B/L posterior-basal ground-glass appearance
    • No destruction of lung parenchyma
    • No honeycombing
    • No cystic spaces
    • Prognosis: NSIP > UIP

    Drugs Causing ILD (NSIP Pattern)

    • Amiodarone
    • Bleomycin
    • Busulfan
    • Bromocriptine
    • Nitrofurantoin
    • Cyclophosphamide
    • Methotrexate
    • Anti-TNF
    • Carmustine
    • Mitomycin

    Treatment of NSIP

    • MMF (best)
    • Steroids
    • Azathioprine

    Features of COP/BOOP

    • Elderly males (>60-70 years)
    • Polypoid plugs in bronchioles (Masson's buds)
    • Pneumonia-like presentation
    • Associations: Polymyositis/Dermatomyositis
    • HRCT: Consolidations (Peribronchial, perilobar opacities)
      • Reverse-halo sign: Ground glass opacification (GGO) surrounded by consolidation
    • Types: Restrictive, Constrictive

    Different Cystic Appearances on HRCT

    • LAM: Young females, association with tuberous sclerosis and angiomyolipoma; Complications: Spontaneous pneumothorax, Chylous effusion; Treatment: Everolimus (mTOR inhibitor)
    • LCH: Young males, upper lobe fibrosis; Complications: Spontaneous pneumothorax; Pathology: CDIA (+), Birbeck granules (+); Associations: Smoking, Lytic lesions, Diabetes insipidus
    • LIP: Associated with Sjogren's and HIV

    AECC Criteria for ARDS

    • Non-cardiogenic pulmonary edema:
      • Pulmonary capillary wedge pressure (PCWP) <18 mmHg
      • Bilateral infiltrates on CXR
      • PaO2/FiO2 ≤ 200 mmHg

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    This quiz explores the etiology and pathogenesis of Acute Respiratory Distress Syndrome (ARDS). It covers both primary and secondary causes, the mechanism of alveolar injury, and the role of inflammatory mediators. Test your knowledge on the complexities of ARDS and its impact on lung function.

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