Interstitial Lung Disease Flashcards by Nesha Barnett | Brainscape

Q

What is the best diagnosis for ILD?

A

Surgical biopsy with histological exam for precise diagnosis.

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

What is interstitial lung disease

A

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

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

How many diseases does ILD have

A

extremely diverse group of acute and chronic disorders

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

How is ILD categorized

A

By varying degrees of inflammation and fibrosis of the lung parenchyma

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

How many types of ILD are there

A

200 different subtypes

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

How is ILD diagnosed

A

By clinical, pathophysiological, immunological, and imaging

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD

A

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

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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)

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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])

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD #1

A

Fever, chills, and night sweats

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD #2

A

Prior medications used and irradiation

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD #3

A

Patient/ Family history- most critical step in evaluation

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD #4

A

Detail work history- occupational exposures

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD #5

A

Travel history

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD#6

A

Enviromental exposures- due to hobbies

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD #7

A

Smoking history

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clinical history of ILD #8

A

Past systemic conditions

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Dyspnea/ all ILD

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Nonproductive cough/ most ILD, productive cough is unusual

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

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

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Arthralgias, arthritis- CTD’s sarcoidosis

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Hemoptysis- diffuse alveolar hemorrhage syndrome, LAM

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Eye symptoms- sarcoidosis, CTD, polyangiitis, and granulomatosis
Salivary gland enlargement- sarcoidosis, Sjogren’s disease

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Abnormal gastroesophageal reflux, dysphagia- CTD (especially SCL), IPF

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Peripheral lymph adenopathy- Sarcoidosis, lymphoid interstitial pneumonitis, ILD with connective tissue disorders.

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Hepatosplenomegaly- Sarcoidosis, eosinophilic granuloma, chronic cor pulmonale

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Neurologic manifestations- Tuberous sclerosis, systemic vasculitis, sarcoidosis, eosinophilic granuloma

How well did you know this?

1

Not at all

2

3

4

5

Perfectly

Q

Clues from initial eval to suggest ILD

A

Rapid onset or worsening- Acute interstitial pneumonia, acute hypersensitivity pneumonitis, cryptogenic organizing pneumonia, CTD, diffuse alveolar hemorrhage

Q

Etiology (cause) of ILD

A

Drugs: ACE inhibitors, amiodarone, and nonsteroidal inflammatory drugs

Q

Etiology (cause) of ILD

A

Illicit drugs- cocaine heroin

Q

Etiology (cause) of ILD

A

Inorganic material - asbestos, coffee, farming material hot tub, bird droppings

Q

Etiology (cause) of ILD

A

Infections- aspergillosis, histoplasmosis, parasite infection, mycobacterial and viral infection

Q

Etiology (cause) of ILD

A

Connective tissue disorders- system sclerosis, autoimmune myosis, Goodpasture syndrome, and systemic lupus erythematosus

Q

Etiology (cause) of ILD

A

Vasculitis- eosinophilic granulomatosis with polyangiitis,

Q

Etiology (cause) of ILD

A

Miscellaneous causes- amyloidosis, chronic aspiration, sarcoidosis, tuberous, sclerosis, lipoid pneumonia

Q

Etiology (cause) of ILD

A

0% of persons with ILD have no clear-cut cause that is easily identifiable. This affects the diagnosis a precise etiology for ILD,

Q

Etiology (cause) of ILD

A

30% of persons with ILD have no clear-cut cause that is easily identifiable. This affects the diagnosis a precise etiology for ILD,

Q

Epidemiology

A

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.

Q

Epidemiology

A

The prevalence is 10 times greater in African Americans 34 cases per 100,000

Q

Epidemiology

A

Occurs in middle age toolder adults

Q

Epidemiology

A

Occurs in middle age to older adults

Q

Pathogenesis (manner of development)

A

ILD dominated by inflammation:
Organizing pneumonia
Nonspecific interstitial pneumonitis
ILD dominated by fibrosis:
Characterized by fibroblastic foci

Q

Pathogenesis (manner of development)

A

Lung fibrotic disorders
Characterized by accumulation of fibroblasts, myofibroblasts, and extracellular matrix leading to chronic respiratory failure.
Origins and activation of fibroblasts unknown.

Q

Etiology (cause) of ILD

A

Major subgroups of ILD are now broadly defined as:
Idiopathic interstitial pneumonias (IIPs)

Q

Etiology (cause) of ILD

A

Granulomatous ILD (e.g., sarcoidosis, hypersensitivity pneumonitis)

Q

Etiology (cause) of ILD

A

ILD with known associations (e.g., occupational and drug exposures, connective tissue diseases)

Q

Etiology (cause) of ILD

A

Miscellaneous ILD (e.g., pulmonary Langerhans cell histiocytosis [PLCH])

Q

Epidemiology

A

31.5 new cases diagnosed per 100,000 men/yr and 26.1 new cases diagnosed per 100,000 women/yr

Q

Pathogenesis

A

ILD dominated by inflammation:
Organizing pneumonia
Nonspecific interstitial pneumonitis

Q

Pathogenesis

A

ILD dominated by fibrosis:
Characterized by fibroblastic foci

Q

Pathogenesis

A

Lung fibrotic disorders
Characterized by accumulation of fibroblasts, myofibroblasts, and extracellular matrix leading to chronic respiratory failure.
Origins and activation of fibroblasts unknown

Q

What are some risk factors to ILD?

A

Smoking
Bronchiolitis associated ILD
Desquamative interstitial pneumonia
Pulmonary Langerhans cell histiocytosis

Q

What are some risk factors to ILD?

A

COPD
Higher risk for development of ILD

Q

What are some risk factors to ILD?

A

Rheumatoid arthritis
Older age and male gender increases risk

Q

What are some risk factors to ILD?

A

Exposure to radiation therapy

Q

What type of laboratory test are done for ILD?

A

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.

Q

Lung function tests

A

usually show restrictive disease, useful but not diagnostic

Q

What results would LFT show for ILD

A

Reduced values, Normal or elevated FEV1/FVC, Reduced diffusing capacity (DLCO)
Due to V/Q mismatch

Q

What will ABG result show?

A

Early ILD
Normal or mild hypoxemia/respiratory alkalosis at rest

Q

What will ABG result show?

A

Late ILD
Hypercapnea

Q

What will ABG result show?

A

Exercise test
Six-minute walk (6MWT) with pulse oximetry
Cardiopulmonary exercise test
Overnight pulse oximetry

Q

Why is cardiac assessment done?

A

Need to rule out heart failure as differential diagnosis.
If HF or PH is suspected, BNP is measured

Q

What will x-ray reveal?

A

CXR will show changes when symptoms are present.
Can be normal with some cases when lung involvement is mild.

Q

Which is more accurate CT or x-ray?

A

High-resolution CT
Provides greater accuracy.
Most appropriate protocol for ILD

Q

What typical patterns will be seen?

A

Reticulation
Honeycombing
Ground glass appearance
Consolidation
Micronodules

Q

Is tissue sampling (biopsy) effective?

A

Most likely method of definitive diagnosis is surgical lung biopsy.

Q

What treatment should provide a plan for each of the following?

A

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?

Q

What treatment or management is needed?

A

Dependent on underlying disease and primary histologic classification
May consist of immunosuppressive drugs

Q

What treatment or management is needed?

A

Corticosteroids
Nintedanib
Intracellular inhibitor
Pirfenidone
Antifibrotic, anti-inflammatory, and antioxidant

Q

Does treatment include O2 therapy?

A

Oxygen
Hypoxemia is a common finding with ILD

Q

Pulmonary rehab

A

Not well studied
Can improve quality of life

Q

How can it be prevented?

A

Vaccinations/avoidance of infection

Q

For long term survival what is a permanent treatment?

A

Lung transplant
May be required for ongoing survival
Potentially curative option
Timely referral needed
Rate of decline in FVC and DLCO predictor of requirement

Q

What is the prognosis?

A

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