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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
Q

What will laboratory studies possibly show in a patient with Sarcoidosis?

A
  • Elevated serum ACE
  • Elevated serum calcium
  • Hypercalciuria
  • Elevated alkaline phosphatase
26
Q

What is the most common pattern of lymphadenopathy in Sarcoidosis and will be seen on CXR?

A

Hilar adenopathy (Lambda sign)

27
Q

What testing is typically required to diagnosis Sarcoidosis?

A

Lung biopsy (EBUS-TBLB sufficient)

28
Q

At what stage would a patient require treatment for Sarcoidosis?

A

Stage 2 and symptomatic

29
Q

What condition is characterized by fibronodular lung disease and is due to inhalation of silica dust?

What occupations are at risk for this disease?

A

Silicosis

At risk: mining, construction, granite cutting

30
Q

Which ILD results from inhalation of coal dust particles?

A

Coal Worker’s Pneumoconiosis (CWP)

31
Q

What diagnostic studies are typically obtained in Silicosis and CWP?

A

CXR, then HRCT:

32
Q

What is the length of exposure in chronic simple Silicosis and CWP?

What is typically seen on imaging?

A

10-12 years exposure

Imaging:

  • Hilar node calcification (eggshell pattern)
  • Small, round, nodular opacities on CXR (upper lobe predominance)
33
Q

What is the length of exposure in chronic complicated Silicosis and CWP?

What is typically seen on imaging?

A

> 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
Q

What are some typically physical exam findings in Silicosis and CWP?

A
  • Tachypnea, prolonged expiration, rhonchi, wheezing, rales
  • Cyanosis (advanced disease)
  • Cor pulmonale (advanced disease)
  • Digital clubbing is UNCOMMON
35
Q

What is the management for Silicosis and CWP?

A
  • 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
Q

When does Asbestosis typically present?

A

After 15-20 years of exposure; dose dependent

37
Q

What is typically seen on physical exam in Asbestosis?

A
  • No specific sign/symptom
  • Insidious onset of dyspnea, reduced exercise intolerance, nonspecific chest discomfort
  • Dry cough
  • End-inspiratory rales
  • Digital clubbing
38
Q

What can be found on CXR in a patient with possible Asbestosis?

A

Opacities in lower lungs, thickened pleura, pleural plaques

39
Q

What can provide definitive diagnosis of Asbestosis, but typically not indicated?

A

Open-lung biopsy

40
Q

What is the management for Asbestosis?

A

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
Q

What form of cancer is almost always associated with asbestos exposure?

A

Mesothelioma

42
Q

What is a reversible inflammatory syndrome of the lung caused by repetitive inhalation of antigens in a susceptible host?

A

Hypersensitivity Pneumonitis

43
Q

The following physical exam findings are most consistent with what ILD?

  • Diffuse, fine bibasilar crackles
  • Fevers
  • Tachypnea
  • Muscle wasting
  • Digital clubbing
  • Weight loss
A

Hypersensitivity Pneumonitis

44
Q

What is the management for Hypersensitivity Pneumonitis?

A
  • Consult pulmonary
  • Avoid antigen
  • Corticosteroids (chronic process)
  • Bronchodilators, antihistamines, inhaled steroids as adjuncts
45
Q

What process is characterized by necrotizing granulomas of the upper and lower respiratory tracts?

A

Granulomatosis with Polyangiitis (GPA)

46
Q

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
A

Granulomatosis with Polyangiitis (GPA)

47
Q

What may be found on CXR in a patient with Granulomatosis with Polyangiitis (GPA)?

CT Chest?

A

CXR: nodules which may cavitate

CT chest: stellate shaped peripheral pulmonary arteries

48
Q

When would bronchoscopy be indicated in Granulomatosis with Polyangiitis (GPA)?

A

Hemorrhage or needed for therapeutic intervention

49
Q

What is the management for Granulomatosis with Polyangiitis (GPA)?

A
  • Consult rheumatology and pulmonary

- Initial treatment with cyclophosphamide (immunosuppressant) and corticosteroids

Clinical Med II (17 decks)

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