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Clinical Med II > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

Define ILD/DPLD?

A

A large group of pulmonary disorders, most of which cause progressive scarring of the lung tissue surrounding the alveoli

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2
Q

What is the pathophysiology of ILD?

A

A process of fibrosis and aberrant healing response

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3
Q

What are common symptoms of ILD?

A
  • Progressive dyspnea on exertion
  • Persistent non-productive cough
  • Wheezing and chest pain are UNCOMMON
  • Extra-pulmonary symptoms
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4
Q

What is the gold standard for diagnosing ILD?

A

Lung tissue biopsy

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5
Q

What diagnostic study offers the highest yield as a non-invasive test for ILD?

A

High-resolution chest CT (HRCT)

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6
Q

What is typically found on chest x-ray in those with ILD?

A
  • Ground-glass appearance (early finding)
  • Reticular “netlike” opacities (can also be nodular or mixed)
  • Honeycombing (poor prognosis)
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7
Q

Are most ILDs associated with obstructive or restrictive defect?

A

Restrictive

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8
Q

In restrictive disease, will the following values will be increased or decreased?

TLC
FVC
FEV1
RV
FEV1/FVC ratio
A 
TLC: decreased 
FVC: decreased
FEV1: decreased
RV: decreased 
FEV1/FVC ratio: normal
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9
Q

If the Diffusing Capacity of Lungs for Carbon Monoxide (DLCO) is reduced, what is this consistent with?

A

ILD

Indicates alveolar damage, impaired gas exchange and therefore decreased delivery of O2 into bloodstream

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10
Q

What might an ABG show in ILD?

A

May be normal or show hypoemia (low PaO2) or respiratory alkalosis (low PaCO2)

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11
Q

What is a contraindication to obtaining a lung biopsy?

A

Honeycombing on imaging; prognosis is already poor and will not change management at this point

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12
Q

What is transbronchial biopsy helpful in biopsying?

A

Helpful to biopsy central location, NOT periphery

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13
Q

What is Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) helpful in biopsying?

When is it especially useful?

A

Evaluating hilar and mediastinal lymph nodes.

Especially useful if sarcoid is suspected.

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14
Q

What is the most common interstitial lung disease?

A

Idiopathic pulmonary fibrosis

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15
Q

The following presentation is most common in which ILD?

  • Gradual onset of exertional dyspnea and non-productive cough for more than 6 months
  • Velcro crackles (inspiratory)
  • Digital clubbing
A

Idiopathic Pulmonary Fibrosis

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16
Q

In what conditions will you likely observe a Usual Interstitial Pneumonia (UIP) on histopathology?

A
  • Idiopathic Pulmonary Fibrosis

- Asbestosis

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17
Q

What should you obtain in Idiopathic Pulmonary Fibrosis to obtain a good baseline for testing?

A

6-minute walk test and PFTs

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18
Q

What is the management for Idiopathic Pulmonary Fibrosis? (use options other than medications)

A
  • Consult pulmonologist
  • Treat for GERD even if no symptoms
  • Quit smoking, stay UTD on vaccines
  • Supplement oxygen as needed
  • Lung transplant (only curative treatment)
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19
Q

What medications can be used in Idiopathic Pulmonary Fibrosis to help decrease the rate of progression?

A
  • Nintedanib (TKI)

- Pirfenidone

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20
Q

What is the most common population to be affected by Idiopathic Pulmonary Fibrosis?

A

Male smoker around 60 y/o

21
Q

What is the most common population to be affected by Sarcoidosis?

A

Younger African American females

22
Q

What clinical finding is pathognomonic of Sarcoidosis?

A

Lupus Pernio

23
Q

The following clinical presentation is most common with what condition?

  • Dyspnea on exertion
  • Cough
  • Chest pain
  • Systemic complaints (fever, anorexia)
  • Erythema nodosum
  • Lupus pernio
  • Arthralgias
  • Granulomatous uveitis
A

Sarcoidosis

24
Q

What do the non-caseating granulomas in Sarcoidosis secrete and what can this cause?

A

Secrete vitamin D and ACE.

Serum vitamin D, calcium, and ACE may be elevated.

25
What will laboratory studies possibly show in a patient with Sarcoidosis?
- Elevated serum ACE - Elevated serum calcium - Hypercalciuria - Elevated alkaline phosphatase
26
What is the most common pattern of lymphadenopathy in Sarcoidosis and will be seen on CXR?
Hilar adenopathy (Lambda sign)
27
What testing is typically required to diagnosis Sarcoidosis?
Lung biopsy (EBUS-TBLB sufficient)
28
At what stage would a patient require treatment for Sarcoidosis?
Stage 2 and symptomatic
29
What condition is characterized by fibronodular lung disease and is due to inhalation of silica dust? What occupations are at risk for this disease?
Silicosis At risk: mining, construction, granite cutting
30
Which ILD results from inhalation of coal dust particles?
Coal Worker's Pneumoconiosis (CWP)
31
What diagnostic studies are typically obtained in Silicosis and CWP?
CXR, then HRCT:
32
What is the length of exposure in chronic simple Silicosis and CWP? What is typically seen on imaging?
10-12 years exposure Imaging: - Hilar node calcification (eggshell pattern) - Small, round, nodular opacities on CXR (upper lobe predominance)
33
What is the length of exposure in chronic complicated Silicosis and CWP? What is typically seen on imaging?
> 20 years exposure Imaging: - Confluence of small nodules to form large opacities and masses - Progressive massive fibrosis (angel-wing appearance on CXR and honeycombing on HRCT)
34
What are some typically physical exam findings in Silicosis and CWP?
- Tachypnea, prolonged expiration, rhonchi, wheezing, rales - Cyanosis (advanced disease) - Cor pulmonale (advanced disease) - Digital clubbing is UNCOMMON
35
What is the management for Silicosis and CWP?
- Pulmonologist - Smoking cessation - No specific therapy other than corticosteroids may benefit ACUTE SILICOSIS only - Vaccinate - Elevated TB risk, so test and treat latent infection - Increased risk for lung cancer - Evaluate for transplant candidacy
36
When does Asbestosis typically present?
After 15-20 years of exposure; dose dependent
37
What is typically seen on physical exam in Asbestosis?
- No specific sign/symptom - Insidious onset of dyspnea, reduced exercise intolerance, nonspecific chest discomfort - Dry cough - End-inspiratory rales - Digital clubbing
38
What can be found on CXR in a patient with possible Asbestosis?
Opacities in lower lungs, thickened pleura, pleural plaques
39
What can provide definitive diagnosis of Asbestosis, but typically not indicated?
Open-lung biopsy
40
What is the management for Asbestosis?
Goal is to eliminate progression of symptoms and reduce risk of other lung disorders No immunotherapy drugs or steroids will alter disese course and are not effective
41
What form of cancer is almost always associated with asbestos exposure?
Mesothelioma
42
What is a reversible inflammatory syndrome of the lung caused by repetitive inhalation of antigens in a susceptible host?
Hypersensitivity Pneumonitis
43
The following physical exam findings are most consistent with what ILD? - Diffuse, fine bibasilar crackles - Fevers - Tachypnea - Muscle wasting - Digital clubbing - Weight loss
Hypersensitivity Pneumonitis
44
What is the management for Hypersensitivity Pneumonitis?
- Consult pulmonary - Avoid antigen - Corticosteroids (chronic process) - Bronchodilators, antihistamines, inhaled steroids as adjuncts
45
What process is characterized by necrotizing granulomas of the upper and lower respiratory tracts?
Granulomatosis with Polyangiitis (GPA)
46
The following physical exam findings and manifestations are most consistent with what process? - Pulmonary infilitrates, cough, hemoptysis - Renal failure, RBC casts - Palpable purpura, skin ulcers - Saddle nose deformity
Granulomatosis with Polyangiitis (GPA)
47
What may be found on CXR in a patient with Granulomatosis with Polyangiitis (GPA)? CT Chest?
CXR: nodules which may cavitate CT chest: stellate shaped peripheral pulmonary arteries
48
When would bronchoscopy be indicated in Granulomatosis with Polyangiitis (GPA)?
Hemorrhage or needed for therapeutic intervention
49
What is the management for Granulomatosis with Polyangiitis (GPA)?
- Consult rheumatology and pulmonary | - Initial treatment with cyclophosphamide (immunosuppressant) and corticosteroids

Clinical Med II (17 decks)

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