Restrictive Lung dz packet - Ms. Anna Flashcards

(62 cards)

1
Q

What is restrictive lung dz characterized by?

A

Decreased lung volume and decreased lung capacity due to either alteration in the lung parenchyma or disease of the pleura, chest wall, or neuromuscular apparatus

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2
Q

Describe how alteration in lung parenchyma relates to restrictive lung dz?

A

“intrinsic” or “interstitial” dz
Disease causes inflammation, scarring, or results in filling of air spaces with exudate or debris

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3
Q

Describe how diseases of pleura, chest wall, or neuromuscular apparatus relate to restrictive lung disease?

A

“extrinsic”
Chest wall, pleura, or respiratory muscles are not functioning normally

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4
Q

What are some examples of extrinsic disease that involve the chest wall?

A

Trauma
Kyphoscoliosis
Ankylosing spondylitis
Neuromuscular disease like myasthenia gravis/guillain barre
morbid obesity
scleroderma

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5
Q

What are some drug induced causes of interstitial lung disease?

A

Chemotherapy
Methotrexate
Amiodarone
Macrobid

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6
Q

What are some autoimmune related causes of interstitial lung disease?

A

RA
SLE
Scleroderma
Polymyositis
Dermatomyositis
Sjogrens syndrome

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7
Q

What is the most common hypothesized etiology of interstitial lung disease?

A

Both environmental and genetic factors contribute
Usually idiopathic
Most identifiable causes include infectious, drug-related, or environmental/occupational

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8
Q

What does interstitial lung disease cause?

A

thickening of the interstitium, a part of the lung’s structure
Inflammation, scarring, or extra fluid can result in the thickening
Can be acute or chronic

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9
Q

What are the pathophysiological changes in ILD?

A

After injury/exposure:
-A repair process is initiated resulting in progressive, patchy, and diffuse thickening of the alveolar walls with connective tissue and inflammatory cells

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10
Q

What is the functional abnormality of ILD?

A

Reduced lung volume/capacity

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11
Q

What are the symptoms of ILD?

A

Dyspnea**
-usually insidious and progressive
Dry chronic cough
Extrapulmonary symptoms may be noted depending on dz

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12
Q

What are the signs of ILD?

A

Early - normal
Advanced:
-tachypnea
-bilateral crackles
-clubbing
-right sided heart failure

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13
Q

What all should be in the workup for ILD?

A

CXR
PFTs
CT chest
Blood tests
Bronchoscopy with biopsy
Surgical biopsy

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14
Q

What are the common CXR findings in ILD?

A

Low lung volumes
Patchy “ground glass” appearance
Hallmark: reticular or nodular opacities

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15
Q

What are the common Chest CT findings in ILD?

A

HRCT:
Reticular opacities
interlobular septal thickening
Honeycombing (late)
Nodules
Traction bronchiectasis

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16
Q

What will be seen on spirometry in ILD?

A

Decrease lung volumes
Decrease vital capacity

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17
Q

What is the use of Bronchoscopy with biopsy and surgical biopsy for ILD?

A

Bronchoscopy with biopsy is diagnostic for sarcoidosis
Surgical biopsy is the definitive diagnostic study

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18
Q

What are the pharmacological treatment options for ILD?

A

Corticosteroids
Cytotoxic agents
Immunosuppressive agents

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19
Q

What are the non-pharmacological treatment options for ILD?

A

Smoking cessation
Supplemental O2 therapy
Avoid exposure/DC meds
Tx pulmonary infections
Pulmonary rehabilitation
Lung transplant (definitive)

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20
Q

Define idiopathic pulmonary fibrosis?

A

A subtype of interstitial lung disease that results in fibrosis of the lungs without a known cause

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21
Q

What is the etiology of idiopathic pulmonary fibrosis?

A

Cause is unknown
More commonly effects older adults (65+)
Hypothesized genetic link

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22
Q

What is the pathogenesis of idiopathic pulmonary fibrosis?

A

An inciting incident disrupts homeostasis of alveolar epithelial cells causing diffuse epithelial cell activation and abnormal cell repair

Exaggerated accumulation of extracellular matrix - destruction of the lung parenchyma

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23
Q

Idiopathic pulmonary fibrosis clinical manifestations?

A

limited to lungs - systemic symptoms rare
Onset is gradual and not often diagnosed for 1-2 years
Most common complaint is dyspnea, 2nd is non-productive cough

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24
Q

What is found on PE for idiopathic pulmonary fibrosis?

A

End-respiratory crackles

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25
What is found on CXR for idiopathic pulmonary fibrosis?
Diffuse reticular opacities; not diagnostically specific
26
What is seen on HRCT for idiopathic pulmonary fibrosis?
Diffuse reticular pattern, honeycombing
27
Describe use of a 6-minute walk test in idiopathic pulmonary fibrosis?
If O2 drops below 88%, mortality risk is increased If HR fails to decline 1-2 min after exercise, indicates poor prognosis
28
What is a helpful diagnostic test for idiopathic pulmonary fibrosis?
Bronchoalveolar lavage with fluid analysis
29
What is the diagnostic criteria for idiopathic pulmonary fibrosis?
>65 years old Idiopathic dz Restrictive pattern on PFT Progressive scarring on CXR honeycombing on HRCT
30
How is idiopathic pulmonary fibrosis treated?
Treat underlying diseases Supplement O2 if sat <88% on room air Smoking cessation Vaccinations Symptom management Drug therapy - anti-fibrotic drugs, immunosuppressants Lung transplant is the ONLY TX to prolong survival
31
What is the prognosis for idiopathic pulmonary fibrosis?
Poor 2-5 years after diagnosis
32
What is the etiology of asbestosis?
A group of naturally occurring, heat-resistant fibrous silicates that are found: -shipyards -construction sites -pipe fitters -insulation -cement -shingles -vinyl floor -textiles
33
What is the typical presentation of asbestosis?
about 10-20 years after exposure: -Chronic SOB -Clubbing -Inspiratory crackles
34
What are the CXR findings of asbestosis?
Linear streaking at bases Diffuse infiltrate Pleural calcifications
35
What is to be noted about CT of chest in asbestosis?
Best imaging method
36
What are the complications for asbestosis?
Pulmonary HTN Right sided HF Progressive pulmonary insufficiency Malignancy - lung and mesotheliomas (6x)
37
What is the etiology of silicosis?
Inhalation of free silica: rock mining stone cutting sand blasting pottery
38
What are the CXR findings of silicosis?
Small round opacities Calcifications of hilar nodes (strong indication) Eggshell or popcorn calcification
39
What is the prognosis of silicosis?
Simple silicosis is usually asymptomatic with no effects of PFTs
40
What are the complications of silicosis?
Higher incidence of pulmonary TB Chronic exposure can lead to scarring or fibrosis Acute silicosis can be rapidly progressive and lead to cardiorespiratory failure
41
What is the etiology of coal worker's pneumoconiosis?
Chronic inhalation of dust from high-carbon coal and rarely graphite Typically 20+ Alveolar macrophages ingest dust leading to coal macules 2-5mm in diameter
42
What is found of CXR for coal worker's pneumoconiosis?
Diffuse small opacities prominent un upper lobes nonspecific Caplan syndrome: Necrotic nodules in lung tissue - RA or CWP
43
What is the prognosis for coal worker's pneumoconiosis?
Can be asymptomatic with little to no change in PFT Prognosis depends on severity and length of exposure Severity of disease may depend on the type of coal
44
What is the etiology of hypersensitivity pneumonitis?
Immune system disorder that occurs in some people after they breathe in certain organic antigens triggering inflammation
45
What are specific triggers for hypersensitivity pneumonitis?
Non-asthmatic, nonatopic inflammatory pulmonary disease: Bacteria or mycobacteria Fungi or molds Bird excrements/feathers Animal furs Mold in air conditioners or ventilation Bacteria in hot tub Moldy hay Contaminated food
46
What are the clinical manifestations for hypersensitivity pneumonitis?
Sudden onset of fever/chills, malaise, cough, SOB, nausea 4-8 hours after exposure Crackles, tachypnea, tachycardia, and possibly cyanosis *very sick patients
47
What imaging findings are associated with hypersensitivity pneumonitis?
CXR: Diffuse reticular consolidation pattern Fibrosis in late/chronic stages HRCT: ground glass appearance
48
What is the treatment/prognosis for hypersensitivity pneumonitis?
Severe - steroids Based on many factors including identification and avoidance of agents If not diagnosed properly or poorly controlled, disease can lead to irreversible lung damage
49
What is sarcoidosis?
A multisystem disorder characterized by accumulation of noncaseating granulomas in involved tissues Bilateral hilar lymphadenopathy = sarcoidosis
50
What is the etiology of sarcoidosis?
Unknown Both genetic and environmental factors likely involved It is not malignant
51
What is the epidemiology for sarcoidosis?
AA F>M northern european caucasians Onset usually in third or fourth decade
52
General manifestations of sarcoidosis?
Malaise, fever, and dyspnea Arthralgias anorexia
53
What are the pulmonary manifestations of sarcoidosis?
Dyspnea on exertion cough chest pain ***Crackles are UNCOMMONWh
54
What are some other manifestations of sarcoidosis?
Ocular and skin involvement Cardiac involvement Hepatosplenomegaly Encephalopathy Renal involvement
55
What are the key points of sarcoidosis clinical manifestions?
Multiorgan disease that primarily effects the lung tissue Granulomas can infiltrate any organ leading to pathology Some patients may be asymptomatic
56
What is the skin finding in sarcoidosis?
lupus pernio
57
What is the ocular finding in sarcoidosis?
Granulomatous uveitis
58
How is sarcoidosis worked up?
exclude other disorders and determine what body systems are affected Labs: -CBC leukopenia -elevated CSR/ACE levels -R/O TB -Hypercalciuria may be present
59
What is the imaging/diagnostic procedure for sarcoidosis?
Bilateral hilar adenopathy Transbronchial biopsy via fiberoptic bronchoscopy is diagnostic
60
How are asymptomatic sarcoidosis patients treated?
Need close monitoring: -Serial eye examination -CXR -EKG -Labs
61
How should mod-severe sarcoidosis be treated?
oral corticosteroid (prednisone) -long term therapy over months to years -serum ACE levels usually fall with clinical improvement
62
Describe the stages of sarcoidosis?
Stage 0: no sign of granulomas Stage 1: Granulomas in lymph nodes only Stage 2: Granulomas in both lymph nodes and lungs Stage 3: Granulomas present in the lungs only Stage 4: Scarring of the lung tissue and permanent damage