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FunMed: Pulmonology > Restrictive Interstitial Lung Disease > Flashcards

Restrictive Interstitial Lung Disease Flashcards

(71 cards)

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1
Q

Common Presentation

A

Dyspnea

Dry, Nonproductive Cough

Inspiratory Crackles on Exam (except Sarcoidosis)

May or may not have digital clubbing

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2
Q

Common Chest X-ray and CT Findings

A

nodular oppacties - diffuse white spots

reticular opacities - irregular spider webs that represent scarring

honeycombs (CT) - alveoli are broken and surrounded by thick scar tissue, limiting the passage of gasses across the alveolar-capillary barrier

traction bronchiectasis (CT) - too much negative pressure pulls tissue away from bronchioles

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3
Q

FVC

A

low

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4
Q

FEV1/FVC

A

normal to high

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5
Q

DLCO

A

low

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6
Q

TLC

A

low

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7
Q

Common Pathophysiology

A

injury and damage occurs to the alveolar epithelial or capillary endothelial cells leading to inflammation (alveolitis) and eventually fibrosis

the inflammation and scarring limits the passage of gases across the alveolar-capillary barrier

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8
Q

Lungs with interstitial lung disease …

A

show areas of fibrosis (irreversibly enlarged, damaged bronchioles and distorted alveoli) that alternate with areas of normal lung

the areas of fibrosis between the alveoli greatly decreases the gas exchange, reducing oxygen transferred to the bloodstream

honeycombing results – clustered cystic air spaces

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9
Q

Idiopathic Pulmonary Fibrosis

A

also called Usual Interstitial Pneumonia

fibrotic scarring of the lung interstitium of unknown cause

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10
Q

Idiopathic Pulmonary Fibrosis: Epidemiology

A

men

55-60 yo

white

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11
Q

Idiopathic Pulmonary Fibrosis: Signs and Symptoms

A

insidious onset of dry, hacking cough

SOB

inspiratory crackles on exam

slow onset; may seem normal to patient

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12
Q

Idiopathic Pulmonary Fibrosis: Characteristic Histologic Pattern

A

alternating areas of normal lung, interstitial inflammation, fibroblast focial, and honeycomb change (+/-)

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13
Q

Idiopathic Pulmonary Fibrosis: Chest X-Ray

A

reticular opacities with a basilar predominance (lower lobes)

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14
Q

Idiopathic Pulmonary Fibrosis: CT

A

basilar and subpleural areas of reticular changes, honeycombing, and traction bronchiectasis

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15
Q

Idiopathic Pulmonary Fibrosis: Diagnosis

A

must rule out other insterstitial diseases in order to be truly idiopathic

can be made on H&P and imaging alone as long as all features are present

may require tissue pathology via Bronchoalveolar Lavage or Transbronchial Biopsy or Surgical Lung Biopsy

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16
Q

Idiopathic Pulmonary Fibrosis: Treatment

A

none; lung transplant is definitive with a 50% 5 year survival rate

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17
Q

Sarcoidosis

A

a multisystem granulomatous disorder of unknown etiology

multisystem inflammatory disorder characterized by noncaseating granuloma formation

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18
Q

Sarcoidosis: Epidemiology

A

geographical and familial clusters

10-20 per 100,000

African Americans

Female

20-30 yo

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19
Q

Sarcoidosis: Signs and Symptoms

A

pulmonary findings in 90%: onset of dry hacking cough and shortness of breath can be subacute (weeks to months) or chronic (months to years)

NO inspiratory crackles

Imaging: reticular opacities are in the middle and upper lungs

hilar adenopathy

extrapulmonary findings

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20
Q

Sarcoidosis: Extrapulmonary Findings

A

may have fatigue, malaise, fever, or weight loss

skin lesions

cervical lymphadenopathy

visual changes, dry eyes

dry mouth, parotid swelling

joint pain and swelling

muscle weakness

hepatomegaly and tenderness

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21
Q

Sarcoidosis: Diagnosis

A

based on H&P (no historical features indicative of other similar presenting disorders) and imaging findings

MUST have tissue pathology that shows noncaseating granulomas

CBC to rule out lymphomas

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22
Q

Sarcoidosis: Treatment

A

glucocorticoids (Prednisone)

immunosuppressive therapy if ineffective (Methotrexate)

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23
Q

Pneumoconiosis

A

occupational lung disease secondary to inhalation of inorganic dusts

a group of chronic fibrotic lung diseases that result from inhalation of inorganic dusts typically associated with specific occupations

treatment is supportive

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24
Q

Coal Miners Lung Disease: Pathophysiology

A

coal dust is ingested by macrophages, which then become coal macules that show as 2-5 mm radio-opaque nodules on chest x-ray

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25
Coal Miners Lung Disease: Chest X-Ray
2-5 mm radio-opaque nodules that are primarily in the upper lung fields in “simple” may become more widespread in “complicated coal worker’s pneumoconiosis” with progressive massive fibrosis
26
Coal Miners Lung Disease: Treatment
supportive care; no other treatment is shown to help; prevent further exposure
27
Asbestos Pneumoconiosis
secondary to inhalation of asbestos fibers, which contain fibrous magnesium silicate
28
Who is most at risk for developing Asbestos Pneumoconiosis?
asbestos mining and milling workers are most at risk, but also people doing insulation work, shipbuilding, construction, pipe fitting, and textile work
29
Asbestos Pneumoconiosis: Symptoms
dry, nonproductive cough; dyspnea; and inspiratory crackles that presents decades after exposure
30
Asbestos Pneumoconiosis Imaging
reticular and small nodular opacities in the lower lung field calcified pleural plaques in the lower lungs pleural effusions
31
Asbestos Pneumoconiosis: Pathology Lab
pathology is not necessary but can aid diagnosis by identifying asbestos fibers and ferruginous bodies
32
Asbestos Pneumoconiosis: Complications
respiratory failure for a minority of patients Malignancy: bronchogenic adenocarcinoma, malignant mesothelioma, concomitant smoking increases risk
33
Asbestos Pneumoconiosis: Treatment
supportive and preventative; O2
34
Silicosis Pneumoconiosis
secondary to inhalation of crystalline silica (SiO2) in quartz, grant, or sandstone can be acute or chronic
35
Who is most at risk for developing Silicosis Pneumoconiosis?
occupations at risk include mining, quarrying, drilling, potters, masonry, and sandblasting
36
Acute Silicosis Pneumoconiosis
rare; after exposure to high concentrations of crystalline silica symptoms begin within a few weeks to years appears like chronic silicosis on radiograph, but shows sooner poor prognosis: progresses to complicated and frequently respiratory failure less than four year survival rate from onset of symptoms
37
Chronic Silicosis Pneumoconiosis
often decades after cessation of job associated with exposure includes Simple Silicosis, which can progress to Complicated Silicosis (Progressive Massive Fibrosis) can be asymptomatic and only noted on chest x-ray insidious onset of cough and dyspnea with inspiratory crackles on exam
38
Silicosis Pneumoconiosis: Imaging
Simple Silicosis appears as innumerable, small, rounded opacities (<10mm) in the dorsal, upper lung fields Complicated Silicosis shows larger nodules (>1cm) that evolve into conglomerate masses the small nodules of simple have coalesced conglomerate masses can appear like malignancies
39
Berylliosis Pneumoconiosis
a chronic, immunologically mediated, granulomatous disease caused by exposure to beryllium your body makes an antibody to beryllium, and the immune response leads to disease
40
Beryllium
a metal used in a number of industrial applications
41
Who is at most risk for developing Berylliosis Pneumoconiosis?
occupations at risk include metal and metal allow machine shops, electronics manufacture and assembly, defense industry, beryllium extraction, automotive industry, computer industry, aerospace industry (more often)
42
Berylliosis Pneumoconiosis: Symptoms
insidious onset of dry, nonproductive cough; shortness of breath; crackles in the lungs on exam inhalation causes pulmonary pathology, but skin contact can also cause cutaneous nodules cutaneous nodules are on exposed areas of skin and smaller than those in Sarcoidosis onset of exposure to symptoms varies from 3 months to 30 years
43
Berylliosis Pneumoconiosis: Imaging
varies, but more common in the upper lung fields
44
Berylliosis Pneumoconiosis: Special Testing
Beryllium Lymphocyte Proliferation Test (BeLPT) blood test, low sensitivity, high specificity repeat negative or borderline two negatives is a ‘negative; also perform Bronchoalveolar Lavage (BAL) effluent
45
Berylliosis Pneumoconiosis: Tissue Pathology
shows noncaseating granulomas
46
Berylliosis Pneumoconiosis: Management
mild disease or Beryllium sensitization but no frank Berylliosis: None with onset of bothersome dyspnea or decrease in lung volumes: Prednisone chronically if fail glucocorticoids then may try Methotrexate or other immunosuppressive agents but no clear evidence showing efficacy
47
Berylliosis Pneumoconiosis: Prognosis
course is variable; may live chronically on glucocorticoids some patients experience progressive deterioration of lung function and development of pulmonary fibrosis no increased risk of cancer
48
Hypersensitivity Pneumonitis
an immunologic reaction to an inhaled organic antigen that is often animal or plant related, such as agricultural dusts, bioaerosols, reactive chemical species requires initial exposure to sensitize the immune system; subsequent exposures can then result in disease
49
Occupations and hobbies most at risk for Hypersensitivity Pneumonitis are related to....
farming, bird and poultry handling, and ventilation and water-related contamination
50
Farming Environmental Source and Causative Agent related to Hypersensitivity Pneumonitis
moldy hay, grain, and silage thermophilic actinomycetes
51
Poultry Handling Environmental Source and Causative Agent related to Hypersensitivity Pneumonitis
parakeets, budgerigars, pigeons droppings, feathers, serum proteins
52
Ventilation and Water-Related Environmental Sources and Causative Agents related to Hypersensitivity Pneumonitis
humidifier fever – thermoactinomyces hot-tub lung – myobacterium complex lifeguard lung – endotoxin from pool-water sprays
53
Acute Hypersensitivity Pneumonitis: Symptoms
abrupt onset (w/in 4-6 hrs) of fever, chills, malaise, nausea, cough, chest tightness, dyspnea; exam with basilar crackles and tachypnea
54
Acute Hypersensitivity Pneumonitis: Imaging
CXR frequently normal
55
Acute Hypersensitivity Pneumonitis can be confused with?
potentially confused with a viral or bacterial infection and given antibiotics
56
Acute Hypersensitivity Pneumonitis: Treatment
remove from environment where exposure occurred and not return symptoms subside within 12hrs to several days. disease may recur with re-exposure
57
Subacute Hypersensitivity Pneumonitis: Symptoms
gradual development of productive cough, dyspnea, fatigue, anorexia and weight loss; exam with tachypnea and diffuse crackles
58
Subacute Hypersensitivity Pneumonitis: PFTs
restrictive pattern and decreased DLCO
59
Subacute Hypersensitivity Pneumonitis: Imaging
CXR normal to micronodular or reticular opacities; HRCT with diffuse micronodules
60
Subacute Hypersensitivity Pneumonitis: BAL
Bronchalveolar Lavage (BAL) often performed and shows marked lymphocytosis
61
Subacute Hypersensitivity Pneumonitis: Tissue Pathology
shows noncaseating granulomas
62
Subacute Hypersensitivity Pneumonitis: Treatment
removal from exposure; glucocorticoids may be required. improvement in symptoms and function takes weeks to months
63
Chronic Hypersensitivity Pneumonitis: Symptoms
insidious onset of cough, dyspnea, fatigue, weight loss
64
Chronic Hypersensitivity Pneumonitis: Imaging
similar to IPF; differentiation from IPF may be difficult
65
Chronic Hypersensitivity Pneumonitis: BAL
Bronchoalveolar Lavage (BAL) is often necessary - low CD4 to CD8 ratio
66
Chronic Hypersensitivity Pneumonitis: Tissue Pathology
noncaseating granulomas, destruction of alveoli, bronchiolitis obliterans
67
Chronic Hypersensitivity Pneumonitis: Treatment
chronic glucocorticoids removal from exposure still necessary but doesn’t help as much
68
Byssinosis
an obstructive, asthma-like, disorder caused by inhalation of cotton fibers presents with chest tightness, cough, wheezing and dyspnea usually worse on Mondays or first day back after a break/vacation if left untreated and without job change, could lead to chronic bronchitis/COPD
69
Sillo-filler's Disease
toxic lung injury due to nitrogen dioxide inhalation bacterial conversion of nitrates in fresh grains produces N02; his gas is heavier than air and accumulates in silos inhalation of large amounts causes toxic injury to airways and alveoli leads to increased tissue permeability → pulmonary edema
70
Sillo-filler's Disease: Symptoms
dyspnea, cough, crackles on exam, occasionally wheezing as well
71
Sillo-filler's Disease: Treatment
glucocorticoids ASAP to prevent progression to bronchiolitis obliterans, a common late complication.

FunMed: Pulmonology (6 decks)

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