Obstructive vs. Restrictive lung disease Flashcards

1
Q

Obstructive features

A
  • Blockage of the airways or bronchi and/or bronchioles
  • Caused by secretions, inflammation or bronchoconstriction
  • normal lung volume but decreased expiratory function
  • FEV/FVC % decreased
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2
Q

Restrictive features

A
  • Lung disease that does not allow full expansion of the lungs.
  • Multiple causes.
  • Decreased total lung volumes.
  • shrunk FEV/FVC
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3
Q

Obstructive lung diseases

A
Asthma
Inhalant occupational asthma
Bronchitis
Emphysema
COPD=Chronic Obstructive Pulmonary Disease
Bronchiectiasis
Cystic Fibrosis
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4
Q

Restrictive

A
  • Goodpasture’s Syndrome
  • Wegner’s Granulomatosis
  • Kyphoscoliosis
INTERSTITIAL LUNG DISEASE
-Inhaled Lung Diseases
       Inorganic dust
       Pneumoconiosis
Asbestosis
Silicosis
Coal Worker’s Pneumoconiosis

-Organic dust
Hypersensitivity pneumonitis
Eosinophilic pneumonia

INTERSTITIAL LUNG DISEASE

  • Sarcoidosis
  • Connective Tissue
  • Rheumatoid Arthritis
  • Scleroderma
  • Idiopathic interstitial pneumonias
  • Idiopathic pulmonary fibrosis (usual -interstitial pneumonia or UIP)
  • Cryptogenic organizing pneumonia formerly called (BOOP)
  • Acute interstitial pneumonia (Hamman-Rich Syndrome)
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5
Q

Goodpasture’s Syndrome

A

-Restrictive lung disease
-Progressive autoimmune disease of the lungs and kidneys
-Produces intra-alveolar hemorrhage and glomerulonephritis
E: caused by anti-glomerular basement membrane (anti-GBM) antibodies
S/Sx: hemoptysis and dyspnea
Lab: +anti-GBM antibody in serum or biopsy
Dx:
-CXR: bilateral alveolar infiltrates
-PFT: restrictive pattern on spirometry
-Hypoxia
Tx:
-Plasmapheresis and Corticosteroids
-Nephrectomy and dialysis in severe cases

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6
Q

Wegener’s Granulomatosis

A
  • Restrictive lung disease
  • Small vessel vasculitis with predilection for the respiratory tract and kidney
  • Immunologic injury of vessels with secondary inflammatory changes
  • Classic triad: upper respiratory vasculitis, sinus or nasal polyps, lower respiratory vasculitis and glomerlonephritis
  • Lab: +ANCA, Increased ESR
  • Tx: Cyclophosphamide (decreases immune response) +/- prednisone
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7
Q

Kyphoscoliosis

A

-Skeletal abnormality that leads to chronic deterioration in lung function
-Causes increased stiffness of the chest wall
-PFT-reduced lung functions; restrictive lung pattern, cant fully expand chest wall to get a whole breath
S/Sx: dyspnea, use of accessory muscles
-Chronic hypercapnic respiratory failure may occur
-Tx: possible mechanical ventilatory support, BiPAP (help with CO2), corrective intervention when angulation >40degrees.

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8
Q

Interstitial Lung Disease (ILD)

A
  • AKA Diffuse Parenchymal Lung Disease
  • Alveolitis leading to diminished lung compliance and restrictive lung disease
  • E: infectious, drug-related, occupational
  • S/Sx: dyspnea, cough
  • CXR: “honeycomb lung”, ground-glass infiltrates
  • Dx: biopsy
  • Tx: +/- corticosteroids
  • Diffuse parenchymal lung damage effecting interstitium, alveoli, and airways.
  • Diseases are classified together because of similar clinical presentation, chest xray findings and physiologic or pathologic manifestations.
  • The most common identifiable causes of ILD are exposure to occupational and environmental agents; especially inorganic or organic dusts (bacteria or fungi), drug-induced pulmonary toxicity and radiation-induced lung injury.
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9
Q

Pneumoconiosis

A

3 kinds: Asbestosis, Silicosis, Coal worker’s Pneumoconiosis

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10
Q

Asbestosis

A
  • Diffuse interstitial cellular and fibrotic reaction of the lung to inhaled asbestos fibers
  • Asbestos found in old homes, plumbing, floors, insulation, brakes
  • S/Sx: breathlessness, digital clubbing, crackles in the lower lobes, may cause pleurisy with effusion
  • CXR: hazy infiltrates in lower lung zones, interstitial fibrosis, thickened pleura, pleural plaques appear on the diaphragms or lateral chest wall

Cancer causing

  • Mesothelioma-diffuse interstitial cell disease
  • Bronchogenic carcinoma
  • Increased risk of lung cancer with Asbestos exposure and smoking history 16 fold, 1ppd. Increase risk of lung cancer 9 fold, <1ppd. Increased risk of lung cancer with asbestos exposure alone,
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11
Q

Silicosis

A

-Diffuse fibrotic reaction of the lungs to inhalation of free crystalline silica
S/Sx:
-Silica found in sand, quartz, and talc
-Progression can lead to myobacterial or mycotic infections
-CXR: numerous small, rounded opacities scattered throughout the lungs and hilar lymph nodes may be calcified.

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12
Q

Coal Worker’s Pneumoconiossis

A
  • Chronic fibrotic lung disease caused by inhalation of coal dust
  • S/Sx: dyspnea, cough, inspiratory crackles, clubbing, cyanosis
  • CXR: small opacities are prominent in the upper lung fields
  • Dx: PFT with restrictive dysfunction and reduced diffusion capacity
  • Tx: supportive, possibly corticosteroids, smoking cessation
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13
Q

Hypersensitivity Pneumonitis

A
  • Also called extrinsic allergic alveolitis.
  • Abnormal pulmonary lung function testing with restrictive pattern and reduced diffusing capacity.
  • Immunologic reaction to an inhaled agent.
  • Agricultural dusts, bioaerosols and certain reactive chemicals.
  • Most common is Farmer’s Lung-farming, vegetable or dairy cattle workers.
  • Exposure to ventilation systems and water reservoirs.
  • Bird and poultry handlers.
  • Animal handlers
  • Grain and flour processing
  • Lumber mill and construction
  • Plastics, paint/epoxy, electronic industries
  • Textile workers

Treatment-eliminate or decrease allergen exposure
-Protective devices dust filters, mask
-Oral or IV steroids for subacute and chronic
Don’t always help!

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14
Q

Acute Hypersensitivity Pneumonitis Clinical presentation

A
  • Usually abrupt onset (4-6 hrs after exposure) with fever, chills, malaise, nausea, cough, chest tightness and dyspnea.
  • PE: tachypnea and diffuse fine crackles
  • Xray- transient patchy, micronodular opacities
  • Removal for exposure improves symptoms 12 hours to several days with resolution within several weeks.
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15
Q

Subacute Hypersensitivity Pneumonitis

A
  • Gradual development of productive cough, dyspnea, fatigue, anorexia and weight loss
  • Xray more extensive changes with infiltrates and nodular opacities
  • Symptoms usually resolve but slower than acute.
  • Systemic steroids usually necessary.
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16
Q

Chronic Hypersensitivity Pneumonitis

A
  • May lack history of acute episodes
  • Usually report with insidious onset of cough, dyspnea, fatigue, and weight loss.
  • Digital clubbing may be seen.
  • Disabling and irreversible respiratory findings due to pulmonary fibrosis and may be difficult to differentiate between idiopathic pulmonary fibrosis (IPF).
  • Diagnosis by video-assisted thoracoscopic lung biopsy (VATS)
17
Q

Eosinophillic Pneumonia

A
  • Rare disease, can occur at any age.
  • Patients usually between 20 and 40 yrs old.
  • Men are affected approximately twice as often as women.
  • S/Sx: acute febrile illness of less than three weeks. Nonproductive cough and dyspnea. Malaise, myalgias, night sweats, pleuritic chest pain.
  • PE: fever (often high), tachypnea, bibasilar inspiratory crackles and occasionally rhonchi. Low SpO2 levels or hypoxia.
  • Labs not too helpful, may see elevated neutrophils with increased ESR
  • Xray-subtle reticular or ground glass opacities
  • Bronchoalveolar lavage with high eosinolphils
  • PFTs with restrictive lung pattern and reduced diffusing capacity
  • Lung biopsy but diagnosis based usually on clinical presentation and chest xray.
  • Treatment with steroids and respiratory support as needed.
18
Q

ILD - connective tissue disease

A
  • Polymyositis/dermatomyositis (ILD in 10% of cases)
  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Scleroderma (70% of patients have ILD)
  • Mixed connective tissue disease
19
Q

Idiopathic causes of ILD

A
  • Sarcoidosis
  • Idiopathic interstitial pneumonias
  • Idiopathic pulmonary fibrosis (usual interstitial pneumonia or UIP)
  • Cryptogenic organizing pneumonia formerly called (BOOP)
  • Acute interstitial pneumonia (Hamman-Rich Syndrome)
20
Q

Sarcoidosis

A

-Multi-organ disease of unknown cause
-Incidence 10-20 in 100,000 persons with diagnosis between age 10-40 70% of the time.
-3-4x increase in black individuals and 2.4% chance of having sarcoidosis vs 0.85% in whites
-Characterized by noncaseating granulomatous inflammation in affected organs
-S/Sx: nonproductive cough, dyspnea on exertion, insidious onset, chest discomfort
+/- fever, malaise
-Lab: leukopenia, eosiophilla, increased ESR,
-Hypercalcemia, hypercalcuria, increased ACE levels
-CXR: bilateral hilar and right paratracheal adenopathy and diffuse reticular infiltrates
-Dx: transbronchial biopsy confirms diagnosis
-Assoc: erythema nodosum, enlarged parotid gland, arthritis, cardiomyopathy, uveitis, renal stones
-Tx: 90% respond to steroids; SLOW taper

21
Q

Idiopathic Pulmonary Fibrosis (UIP)

A

-Inflammation and fibrosis of the alveolar walls and air spaces without known cause
S/Sx:
-Exertional dyspnea and dry non-productive cough
-Digital clubbing
-Fever, fatigue, anorexia, weight loss
Dx:
-CXR-diffuse ground-glass, nodular, reticular or reticulonodular infiltrates
-PFT: restrictive pattern with decreased FVC and FEV1
Tx: +/- steroids, immunosuppressive agents, Mucomyst

22
Q

Cryptogenic organizing pneumonia orBronchiolitis Obliterans Organizing Pneumonia (BOOP)

A
  • a rare lung condition
  • small airways (bronchioles) and air exchange sac (alveoli) become inflamed with connective tissue.
  • usually starts with a flu-like illness associated with fever, malaise, fatigue and cough.
  • cough may be persistent and troubling.
  • shortness of breath with exertion and weight loss occurs in about half of patients.
  • form of interstitial [within the fine supporting tissue of the lung] pneumonia of unknown origin.
  • Very similar interstitial pneumonias can be seen in association with connective tissue diseases such as lupus erythematosis, several drug exposures and malignancies.
  • PE: other interstitial pneumonias but the chest x-ray and chest CT scan are distinctive
  • findings are not definitive and a lung biopsy is recommended for confirmation. -Pulmonary function tests are nonspecific.
  • tends to be persistent and not self limited.
  • Current therapy involves relatively high doses of corticosteroids [e.g. prednisone] for several months depending upon the response.
  • Other immunosuppressive drugs [e.g. cyclophosphamide] may also be used. -Treatment usually results in significant improvement.
  • recurrences are common and patients should be periodically monitored with chest radiography, especially in the first year after treatment.
23
Q

Acute Interstitial Pneumonia(Hamman-Rich Syndrome)

A
  • Rare and fulminant form of diffuse lung injury.
  • Acute interstitial pneumonia with the most acute onset of interstitial pneumonias and rapidly progressive course.
  • Presents similarly to acute respiratory distress syndrome (ARDS)
  • Effects previously healthy people without history of lung disease. Frequency equal in men and women.
  • No association with smoking.
  • Most patients are over age 40 with mean age 50-55 yrs old.
24
Q

Acute Interstitial Pneumonia

A
  • Chest xray- diffuse alveolar damage
  • Cause is unknown
  • S/Sx: Prodromal illness with muscle aches, chills, arthralgias and malaise 7-14 days.
  • Most present with fever, cough and progressive, SEVERE shortness of breath. Usually will have hypoxemia at presentation. Most require intubation and ventilator support for a few days.
  • PE: Tachypnea, low SpO2, diffuse crackles
  • Severe hypoxemia on ABGs.
  • Bronchoscopy (to ruleout other causes) VATS lung biopsy (identifies diffuse alveolar damage-pathology)
  • Treatment-supportive care. High dose IV steroids. Empiric antibiotics often given.
  • Lung transplant