Chapter 23: Restrictive Pulmonary Disorders Flashcards Preview

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Flashcards in Chapter 23: Restrictive Pulmonary Disorders Deck (48):

Fibrotic Interstitial Lung Disease

- Group of disorders (more than 180 disease entities)

- Characterized by acute, subacute, or chronic infiltration of alveolar walls by cells, fluid, and connective tissue

- If left untreated, may progress to irreversible fibrosis


Diffurse Interstitial Lung Disease

- Also called diffuse interstitial pulmonary fibrosis and other names

- Characterized by thickening of alveolar interstitium


Pathogenesis of and pathological patterns of Diffuse Interstitial Lung Disease

- Begins with injury to alveolar epithelial or capillary endothelial cells

- Persistent alveolitis leads to obliteration of alveolar capillaries, reorganization of lung parenchyma, irreversible fibrosis

- Leads to large air-filled sacs (cysts) with dilated terminal and respiratory bronchioles (honey-comb lung)

- Pathologic patters include inflammation, fibrosis, and destruction


Clinical Manifestations of Diffuse Interstitial Lung Disease

- Progressive dyspnea with irritating, nonproductive cough

- Rapid-shallow breathing

- Clubbing of nail beds

- Bibasilar end-expiratory crackles

- Cyanosis (late finding)

- Anorexia, weight loss

- Inability to increase cardiac output with exercise


Diagnosis of Diffuse Interstitial Lung Disease

- Chest x-ray

- PFTs (decreased VC,

- TLC, diffusing capacity)

- Open lung biopsy

- Transbronchial biopsy

- Gallium-67 scan

- High resolution computed tomography (HRCT) and bronchoalveolar lavage: primary tests


Treatment of Diffuse Interstitial Lung DIsease

- Smoking cessation

- Avoid environmental exposure to cause

- Anti-inflammatory agents

- Immunosuppressive agents

- Oxygen for hypoxemia

- Lung transplant


Sarcoidosis (Etiology)

- Acute or chronic systemic disease of unknown cause

- Immunologic basis is most likely cause

- Activation of alveolar macrophage to unknown trigger

- First degree relative increases risk 5 fold


Pathogenesis of Sarcoidosis

- Development of multiple, uniform, noncaseating epithelioid granulomas

- Affects multiple organs

- Abnormal T cell function


Clinical Manifestations of Sarcoidosis

- Malaise, fatigue

- Weight loss

- Fever

- Dyspnea of insidious onset

- Dry, nonproductive cough

- Erythema nodosum

- Macules, papules, hyperpigmentation, and subcutaneous nodules

- Hepatosplenomegaly, lymphadenopathy


Diagnosis of Sarcoidosis

- Leukopenia, anemia

- Increased eosinophil count, elevated sedimentation rate
Increased Ca++ levels

- Elevated liver enzymes

- Elevated angiotensin-converting enzyme in active disease

- Gallium-67 scan (Localize areas of granulomatous infiltrates)

- PFTs

- Transbronchial lung biopsy (Noncaseating granulomas (definitive diagnosis))

- Bronchoalveolar lavage

- Stages 0-4 (Progressing from normal to advanced fibrosis with evidence of honeycombing, hilar retraction, bullae, cysts, and emphysema)


Treatment of Sarcoidosis

- Management of symptoms

- Corticosteroids

- Immunosuppressive agents

- Hydroxychloroquine: for disfiguring skin lesions, hypercalcemia, and neurologic involvement


Acute (Adult) Respiratory DIstress Syndrome (ARDS) Etiology

- Damage to the alveolar-capillary membrane (Causes widespread protein-rich alveolar infiltrates and severe dyspnea)

- Occurs in association with other pathophysiologic processes

- More than 150,000 cases/year in United States

- Mortality rate 30% to 63%

- Associated with a decline in the Pao2 that is refractory (does not respond) to supplemental oxygen therapy


Causes of ARDS

- Severe trauma

- Sepsis (>40%)

- Aspiration of gastric acid (>30%)

- Fat emboli syndrome


Characteristics of ARDS

- Increased permeability of the pulmonary vasculature

- Flooding of the alveoli with proteinaceous fluid

- Leads to development of protein-rich pulmonary edema (noncardiogenic pulmonary edema)

- Triggers the immune system to activate the complement system and to initiate neutrophil sequestration in the lung


Pathogenesis of ARDS

- Injury to alveoli from a wide variety of disorders

- Changes in alveolar diameter

- Injury to pulmonary circulation

- Atelectasis and decrease in lung compliance from lack of surfactant

- Fibrosis (hyaline membrane)

- Diffuse, fluffy alveolar infiltrates

- Disruptions in O2 transport and utilization (Severe hypoxemia)


Clinical Manifestations of ARDS

- History of a precipitating event that has led to a low blood volume state (“shock” state) 1 or 2 days prior to the onset of respiratory failure

- Early: Sudden marked respiratory distress, Slight increase in pulse rate,Dyspnea
Low PaO2, Shallow, rapid breathing

- Late: Tachycardia, Tachypnea
Hypotension, Marked restlessness, Frothy secretions,
Crackles, rhonchi on auscultation, Use of accessory muscles, Intercostal and sternal retractions, Cyanosis


Diagnosis of ARDS

- Hallmark is hypoxemia refractory to increased levels of supplemental O2

- ABG (Hypoxia, Acidosis, Hypercapnia)

- Chest Xray (Normal with progression to diffuse “whiteout”)

- PFTs (Decrease in FVR, Decreased lung volumes, Decreased lung compliance, VA/Q mismatch with large right-to-left shunt)

- Open-Lung biopsy shows (Atelectasis, Hyaline membranes, Cellular debris,
Interstitial and alveolar edema)


Treatments of ARDS

- Mostly supportive (Enhance tissue oxygenation until inflammation resolves)

- Identify and treat underlying cause

- Maintain fluid and electrolyte balance (Increased fluid administration can produce or intensify pulmonary edema)

- Volume ventilator using pressure support

- Mechanical ventilation with positive end-expiratory pressure (PEEP) (Increases FRV and prevents alveolar collapse at end-expiration, Forces edema out of alveoli)

- Supplemental O2 (>60% contributes to ARDS related to absorption atelectasis, FIO2 reduced as soon as possible)

- High-frequency jet ventilation (HFJV)

- Inverse ratio ventilation (IRV)

- Inhaled nitric oxide


Pneumothorax Etiology

- Accumulation of air in the pleural space

- Primary pneumothorax (Spontaneous, Occurs in tall, thin men 20 to 40 years, No underlying disease factors, Cigarette smoking increases risk)

- Secondary Pneumothorax (Result of complications from preexisting pulmonary disease)

- Catamenial pneumothorax (Associated with menstruation, Primarily in right hemothorax, Associated with endometriosis)

- Tension Pneumothorax (Traumatic origin, Results from penetrating or nonpenetrating injury, May also be from iatrogenic causes, Medical emergency)


Pathogenesis of Pneumothorax

- Primary (Rupture of small subpleural blebs in apices; Air enters pleural space, lung collapses, and rib cage springs out)

- Secondary (Result of complications from an underlying lung problem, May be due to rupture of cyst or bleb)

- Tension (Results form buildup of air under pressure in pleural space; Air enters pleural space during inspiration but cannot escape during expiration; Lung on ipsilateral (same) side collapses and forces mediastinum toward contralateral (opposite) side; Decreases venous return and cardiac output)

- Open "sucking" chest wall wound (Air enters during inspiration but cannot escape during expiration leads to shift of mediastinum)


Clinical Manifestations of Pneumothorax

- Small pneumothoraces (


Diagnosis of Pneumothorax

- ABG (Decreased PaO2, acute respiratory alkalosis)


- Chest Xray (Expiratory films show better demarcation of pleural line than inspiratory;
Depression of hemidiaphragm on side of pneumothorax)


Treatment of Pneumothorax

- Management depends on severity of problem and cause of air leak

- Lung collapse 15% to 25% (Chest tube placement with H2O seal and suction; Oxygen)

- Chemical pleurodesis (Promotes adhesion of visceral pleura to parietal pleura to prevent further ruptures; For recurrent spontaneous pneumothorax)

- Thoracotomy (Permits stapling or laser pleurodesis of ruptured blebs)


Pleural Effusion Etiology

- Pathologic collection of fluid or pus in pleural cavity as result of another disease process

- Normally, 5-15 ml of serous fluid is contained in pleural space

- Constant movement of pleural fluid from parietal pleural capillaries to pleural space

- Reabsorbed into parietal lymphatics


Five major types of pleural effusion

- Transudates (Low in protein (ratio 0.5 mg/dl); Causes: malignancies, infections, pulmonary embolism, sarcoidosis, postmyocardial infarction syndrome, pancreatic disease)

- Empyema due to infection in the pleural space (High-protein exudative effusion)

- Hemothorax (Presence of blood in pleural space;
Result of chest trauma; Contains blood and pleural fluid: hemorrhagic)

- Chylothorax or lymphatic
(Exudative process that develops from trauma)


Causes of Pleural Effusion

- Changes in pleural capillary hydrostatic pressure, colloid oncotic pressure, or intrapleural pressure

- Imbalance in pressure associated with fluid formation exceeding fluid removal


Pathogenesis of Pleural Effusion

- Transudates (Increased hydrostatic or decreased oncotic pressure)

- Exudates increase production of fluid r/t (Increased permeability of pleural membrane; Impaired lymphatic drainage)


Clinical Manifestations of Pleural Effusion

- Vary depending on cause and size of effusion

- May be asymptomatic with


Diagnosis of Pleural Effusion

- Chest X-Ray (Pleural-based, densities, infiltrates,Signs of CHF, Hilar adenopathy, and Location of fluid

- Thoracentesis (Analyze fluid and reduce amount of fluid)

- CT or Ultrasonographic Tests (Assist in complicated effusions, and Distinguish mass from large effusion)


Treatment of Pleural Effusion

- Directed at underlying cause and relief of symptoms

- Tension and spontaneous pneumothorax are medical emergencies requiring treatment to remove pleural air and re-expand lung

- Closed chest tube drainage (adults) (Controversial in pediatrics)

- Thoracentesis, if large amount of effusion (Ultrasound useful for thoracentesis guidance)

- Thoracotomy (Control bleeding (>200 ml/hr))


Guillian Barre Syndrome (Acute Polyneuritis) etiology

- Immunologic basis

- Demyelination of peripheral nerves

- History of recent viral or bacterial illness (66% of cases) followed by ascending paralysis (Infection involving Campylobacter jejuni often precedes diagnosis)


Clinical Manifestations of Guillian-Barre Syndrome

- Weakness and paralysis begin symmetrically in lower extremities and ascend proximally to upper extremities and trunk

- Severe cases show Respiratory muscle weakness accompanied by limb and trunk symptoms

- Usually have full recovery

- Minor residual motor deficits (15% to 20%)


Myasthenia Gravis

- Weakness and fatigue of voluntary muscles

- Those innervated by cranial nerves

- Peripheral and respiratory muscles can be affected

- Hallmark finding: weakness worse with exercise and better with rest

- 2-5 cases/year/million persons in U.S.

- Females more than males (3:2)

- Primary abnormality at neuromuscular junction

- Transmission from nerve to muscle impaired from decreased number of receptors on muscle

- Symptoms often managed by appropriate therapy

- Respiratory failure can be due to increasing severity of illness or overmedication

- Individual episodes of respiratory failure are potentially reversible



- Inflammatory reaction in the alveoli and interstitium caused by an infectious agent

- Causes include Aspiration of oropharyngeal secretions composed of normal bacterial flora or gastric contents (25% to 35%), Inhalation of contaminants, and Contamination from the systemic circulation


Classifications of Pneumonia

- Community acquired

- Hospital acquired

- Bacterial

- Atypical

- Viral


Those who are at high risk for Pneumonia

- Elderly

- Those with a diminished gag reflex

- Seriously ill

- Hospitalized patients

- Hypoxic patients

- Immune-compromised patients


Types of Pneumonia

- Anaerobic bacteria: present as a lung abscess, necrotizing pneumonia, or empyema; usually caused by aspiration of normal oral bacteria into the lung

- Mycoplasmal pneumonia: commonly seen in the summer and fall in young adults; common between the ages of 5 and 20

- Legionnaires Disease (Organism lives in H2O;
Transmitted by portable H2O, condensers, cooling towers;
Fever, diarrhea, abdominal pain, liver and kidney failure, pulmonary infiltrates; Treatment: macrolide antibiotic)

- Opportunistic Pneumonias (Pneumocystis jiroveci & Aspergillus)

- Pneumocystis jiroveci
(Opportunistic fungal infection,
Common in patients with cancer or HIV)

- Aspergillus (Opportunistic fungal infection, Released from walls of old buildings under reconstruction)


Pathogenesis of Pneumonia

- Acquired when normal pulmonary defense mechanisms are compromised

- Organisms enter lung, multiply, and trigger pulmonary inflammation

- Inflammatory cells invade alveolar septa

- Alveolar air spaces fill with exudative fluid (Consolidates and difficult to expectorate)

- Viral pneumonia doesn’t produce exudative fluids


Clinical Manifestations of Pnemonia

- Severity of disease and patient age cause variation in symptoms

- Crackles (rales) and bronchial breath sounds over affected lung tissue

- Chills

- Fever

- Cough, purulent sputum

- Viral (Upper respiratory prodrome: Fever, nonproductive cough, hoarseness, coryza accompanied by wheezing/rales)

- Chlamydia Pneumonia (Cough, tachypnea, rales, wheezes, and no fever)

- Mycoplasma (Fever, Cough, Headache, Malaise)


Diagnosis of Pneumonia

- Chest x-ray (Parenchymal infiltrates (white shadows) in involved area)

- Sputum C&S (Sputum from deep in lungs)

- CURB-65

- Laboratory (WBC >15,000 (acute bacterial)


Treatment of Pneumonia

- antibiotic therapy based on sensitivity of culture


Pulmonary Tuberculosis Etiology

- Estimated 10 million infected in United States

- High-risk individuals include those with Prior infection (90%), Malnourishment, immunosuppression, Living in overcrowded condition, Incarcerated persons, Immigrants, and Elderly

- Multidrug-resistant TB

- Cases are reported to local and state health departments


Causes of Pulmonary Tuberculosis

- Mycobacterium tuberculosis (Acid-fast aerobic bacillus Infects lungs and lymph nodes)

- Infection (Inhalation of small droplets containing bacteria;
Droplets expelled with cough, sneeze, or talking)

- Involvement of distant organ systems (Hematogenous spread during primary or reactivation phase)

- Disseminated disease
(Miliary tuberculosis causes hematogenous dissemination of organisms)


Classifications of Pulmonary Tuberculosis

- Primary (usually clinically/radiographically silent)
(May lie dormant for years or decades)

- Reactivating (May occur many years after primary infection;
Impaired immune system causes reactivation; HIV, corticosteroid use, silicosis, and diabetes mellitus have been found to be associated with reactivation)


Pathogenesis of Pulmonary Tuberculosis

- Entry of mycobacteria into lung tissue

- Alveolar macrophages ingest and process microorganisms (Microorganisms destroyed OR persist and multiply)

- Lymphatic and hematogenous dissemination (T-cells and macrophages surround organisms in granulomas)

- Reactivation occurs if immunosuppressed

- Pathologic manifestation is Ghon tubercle or complex


Clnical Manifestations of Pulmonary Tuberculosis

- History of contact with infected person

- Low-grade fever

- Chronic cough (most common); as disease progresses becomes productive with purulent sputum

- Night sweats

- Fatigue

- Weight loss

- Malaise

- Anorexia

- Apical crackles (rales)

- Bronchial breath sounds over region of consolidation

- Malnourished


Diagnosis of Pulmonary Tuberculosis

- Sputum culture (definitive diagnosis) (Three consecutive, morning specimens; Identify slow-growing acid-fast bacillus:
Takes 1-3 weeks for determination)

- DNA or RNA amplification techniques (diagnosis)

- PFTs

- Chest x-ray (Nodules with infiltrates in apex and posterior segments)

- TB skin test (Mantoux or PPD test) (Doesn’t distinguish between current disease or past infection; False-positive PPD results may occur in persons with other mycobacterial infections or if they have received bacille Calmette-Guérin vaccine)


Treatment of Pulmonary Tuberculosis

- Administer multiple drugs (antibiotics) to which organism is susceptible (Therapy is for 9-12 months for active disease;
Therapy shorter in persons exposed with no active disease)

- Add at least 2 new agents to drug regimen when treatment failure is suspected

- Providing safest, most effective therapy for shortest period of time

- Ensuring adherence to therapy by using directly observed therapy (Nonadherence to therapy is major cause of treatment failure; Negative pressure isolation room to prevent spread)