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Respiratory 230 > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

What is the best diagnosis for ILD?

A

Surgical biopsy with histological exam for precise diagnosis.

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2
Q

What is interstitial lung disease

A

heterogenous group of diffuse parenchymal lung disease with a variety of causes, known and unknown

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3
Q

How many diseases does ILD have

A

extremely diverse group of acute and chronic disorders

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4
Q

How is ILD categorized

A

By varying degrees of inflammation and fibrosis of the lung parenchyma

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5
Q

What is the definition of ILD

A

ILD/DPLD are diseases affect the tissue and space around the alveoli, causing progressive scarring of the lung tissue through inflammation and fibrosis

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6
Q

How many types of ILD are there

A

200 different subtypes

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7
Q

How is ILD diagnosed

A

By clinical, pathophysiological, immunological, and imaging

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8
Q

Clinical history of ILD

A

Slow progression of dyspnea on exertion or at rest
nonproductive cough
Duratón can last months to years

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9
Q

What are the classifications of ILD?

A

Major subgroups of ILD are now broadly defined as:
Idiopathic interstitial pneumonias (IIPs)
Granulomatous ILD (e.g., sarcoidosis, hypersensitivity pneumonitis)

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10
Q

What are the classifications of ILD? #2

A

ILD with known associations (e.g., occupational and drug exposures, connective tissue diseases)
Miscellaneous ILD (e.g., pulmonary Langerhans cell histiocytosis [PLCH])

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11
Q

Clinical history of ILD #1

A

Fever, chills, and night sweats

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12
Q

Clinical history of ILD #2

A

Prior medications used and irradiation

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13
Q

Clinical history of ILD #3

A

Patient/ Family history- most critical step in evaluation

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14
Q

Clinical history of ILD #4

A

Detail work history- occupational exposures

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15
Q

Clinical history of ILD #5

A

Travel history

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16
Q

Clinical history of ILD#6

A

Enviromental exposures- due to hobbies

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17
Q

Clinical history of ILD #7

A

Smoking history

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18
Q

Clinical history of ILD #8

A

Past systemic conditions

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19
Q

Clues from initial eval to suggest ILD

A

Dyspnea/ all ILD

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20
Q

Clues from initial eval to suggest ILD

A

Nonproductive cough/ most ILD, productive cough is unusual

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21
Q

Clues from initial eval to suggest ILD

A

Fatigue/ most ILD and present with dyspnea
Fever, chills, weight loss- interstitial pulmonary infections, collagen vascular disorder

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22
Q

Clues from initial eval to suggest ILD

A

Inspiratory crackles/ most ILD
Wheezing- uncommon mostly with hypersensitivity pneumonia
Pleural rub- rheumatoid arthritis, systemic lupus
Digital cubbing- interstitial pulmonary fibrosis

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23
Q

Clues from initial eval to suggest ILD

A

Tachypnea- 15% of all interstitial pulmonary lung diseases
Chest pain- uncommon, pleuritic chest pain/ systemic lupus, rheumatoid arthritis, and pneumothorax

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24
Q

Clues from initial eval to suggest ILD

A

Arthralgias, arthritis- CTD’s sarcoidosis

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25
Clues from initial eval to suggest ILD
Hemoptysis- diffuse alveolar hemorrhage syndrome, LAM
26
Clues from initial eval to suggest ILD
Eye symptoms- sarcoidosis, CTD, polyangiitis, and granulomatosis Salivary gland enlargement- sarcoidosis, Sjogren's disease
27
Clues from initial eval to suggest ILD
Abnormal gastroesophageal reflux, dysphagia- CTD (especially SCL), IPF
28
Clues from initial eval to suggest ILD
Peripheral lymph adenopathy- Sarcoidosis, lymphoid interstitial pneumonitis, ILD with connective tissue disorders.
29
Clues from initial eval to suggest ILD
Hepatosplenomegaly- Sarcoidosis, eosinophilic granuloma, chronic cor pulmonale
30
Clues from initial eval to suggest ILD
Neurologic manifestations- Tuberous sclerosis, systemic vasculitis, sarcoidosis, eosinophilic granuloma
31
Clues from initial eval to suggest ILD
Rapid onset or worsening- Acute interstitial pneumonia, acute hypersensitivity pneumonitis, cryptogenic organizing pneumonia, CTD, diffuse alveolar hemorrhage
32
Etiology (cause) of ILD
Drugs: ACE inhibitors, amiodarone, and nonsteroidal inflammatory drugs
33
Etiology (cause) of ILD
Illicit drugs- cocaine heroin
34
Etiology (cause) of ILD
Inorganic material - asbestos, coffee, farming material hot tub, bird droppings
35
Etiology (cause) of ILD
Infections- aspergillosis, histoplasmosis, parasite infection, mycobacterial and viral infection
36
Etiology (cause) of ILD
Connective tissue disorders- system sclerosis, autoimmune myosis, Goodpasture syndrome, and systemic lupus erythematosus
37
Etiology (cause) of ILD
Vasculitis- eosinophilic granulomatosis with polyangiitis,
38
Etiology (cause) of ILD
Miscellaneous causes- amyloidosis, chronic aspiration, sarcoidosis, tuberous, sclerosis, lipoid pneumonia
39
Etiology (cause) of ILD
#0% of persons with ILD have no clear-cut cause that is easily identifiable. This affects the diagnosis a precise etiology for ILD,
40
Etiology (cause) of ILD
30% of persons with ILD have no clear-cut cause that is easily identifiable. This affects the diagnosis a precise etiology for ILD,
41
Epidemiology
ILD is PREDOMINIANTELY AN ADULT disease; HOWEVER, IT DOES occur in CHILDREN. Certain ILDS such as sarcoidosis, PLHC, and autoimmune-associated lung disease develop in adults.
42
Epidemiology
The prevalence is 10 times greater in African Americans 34 cases per 100,000
43
Epidemiology
Occurs in middle age toolder adults
44
Epidemiology
Occurs in middle age to older adults
45
Pathogenesis (manner of development)
ILD dominated by inflammation: Organizing pneumonia Nonspecific interstitial pneumonitis ILD dominated by fibrosis: Characterized by fibroblastic foci
46
Pathogenesis (manner of development)
Lung fibrotic disorders Characterized by accumulation of fibroblasts, myofibroblasts, and extracellular matrix leading to chronic respiratory failure. Origins and activation of fibroblasts unknown.
47
Etiology (cause) of ILD
Major subgroups of ILD are now broadly defined as: Idiopathic interstitial pneumonias (IIPs)
48
Etiology (cause) of ILD
Granulomatous ILD (e.g., sarcoidosis, hypersensitivity pneumonitis)
49
Etiology (cause) of ILD
ILD with known associations (e.g., occupational and drug exposures, connective tissue diseases)
50
Etiology (cause) of ILD
Miscellaneous ILD (e.g., pulmonary Langerhans cell histiocytosis [PLCH])
51
Epidemiology
31.5 new cases diagnosed per 100,000 men/yr and 26.1 new cases diagnosed per 100,000 women/yr
52
Pathogenesis
ILD dominated by inflammation: Organizing pneumonia Nonspecific interstitial pneumonitis
53
Pathogenesis
ILD dominated by fibrosis: Characterized by fibroblastic foci
54
Pathogenesis
Lung fibrotic disorders Characterized by accumulation of fibroblasts, myofibroblasts, and extracellular matrix leading to chronic respiratory failure. Origins and activation of fibroblasts unknown
55
What are some risk factors to ILD?
Smoking Bronchiolitis associated ILD Desquamative interstitial pneumonia Pulmonary Langerhans cell histiocytosis
56
What are some risk factors to ILD?
COPD Higher risk for development of ILD
57
What are some risk factors to ILD?
Rheumatoid arthritis Older age and male gender increases risk
58
What are some risk factors to ILD?
Exposure to radiation therapy
59
What type of laboratory test are done for ILD?
CBC, calcium, creatine, BNP. Liver function test, and urine sample. Additional blood tests include those to detect the presence of autoantibodies, precipitating immunoglobulins against organic antigens, and serum angiotensin converting enzyme.
60
Lung function tests
usually show restrictive disease, useful but not diagnostic
61
What results would LFT show for ILD
Reduced values, Normal or elevated FEV1/FVC, Reduced diffusing capacity (DLCO) Due to V/Q mismatch
62
What will ABG result show?
Early ILD Normal or mild hypoxemia/respiratory alkalosis at rest
63
What will ABG result show?
Late ILD Hypercapnea
64
What will ABG result show?
Exercise test Six-minute walk (6MWT) with pulse oximetry Cardiopulmonary exercise test Overnight pulse oximetry
65
Why is cardiac assessment done?
Need to rule out heart failure as differential diagnosis. If HF or PH is suspected, BNP is measured
66
What will x-ray reveal?
CXR will show changes when symptoms are present. Can be normal with some cases when lung involvement is mild.
67
Which is more accurate CT or x-ray?
High-resolution CT Provides greater accuracy. Most appropriate protocol for ILD
68
What typical patterns will be seen?
Reticulation Honeycombing Ground glass appearance Consolidation Micronodules
69
Is tissue sampling (biopsy) effective?
Most likely method of definitive diagnosis is surgical lung biopsy.
70
What treatment should provide a plan for each of the following?
What pharmacological agent(s) are appropriate, if any? How will disease progression be monitored? Should a lung transplant referral be made? Is the disease end stage? Is the disease likely to respond to therapy? Is supportive, palliative care for the patient the best approach?
71
What treatment or management is needed?
Dependent on underlying disease and primary histologic classification May consist of immunosuppressive drugs
72
What treatment or management is needed?
Corticosteroids Nintedanib Intracellular inhibitor Pirfenidone Antifibrotic, anti-inflammatory, and antioxidant
73
Does treatment include O2 therapy?
Oxygen Hypoxemia is a common finding with ILD
74
Pulmonary rehab
Not well studied Can improve quality of life
75
How can it be prevented?
Vaccinations/avoidance of infection
76
For long term survival what is a permanent treatment?
Lung transplant May be required for ongoing survival Potentially curative option Timely referral needed Rate of decline in FVC and DLCO predictor of requirement
77
What is the prognosis?
Depends on type of ILD and response to therapy Better chance of survival if: Female Younger Have less dyspnea More significant response with lavage at the onset of symptoms Overall prognosis poor chance of survival is less

Respiratory 230 (4 decks)

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