Exam 2: Interstital Lung Disease Flashcards

1
Q

What is the pathophysiology of ILD?

A

A process of fibrosis and aberrant healing response, sometimes inflammatory but not infectious

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2
Q

A patient presents with a progressive DOE and a persistent non-productive cough. There is no wheezing or chest pain. Patient has crackles at the lung bases and digital clubbing. What are you suspicious of?

A

ILD

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3
Q

Erythema nodosum is common in what disease?

A

Sarcoidosis

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4
Q

Gottrons papules are common in what disease?

A

Dermatomyositis

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5
Q

What is the gold standard of diagnosis of ILD?

A

Tissue biopsy

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6
Q

What CXR finding indicates poor prognosis?

A

Honey combing

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7
Q

What are the common CXR findings of ILD?

A
  • Ground glass appearance

- reticular (most common), nodular, or mixed pattern of opacities

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8
Q

If the distribution of disease is in the upper lung on CT, what does that indicate?

A

Inhalation related diseases

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9
Q

If the distribution of disease is in the lower portion of the lungs on CT, what does that indicate?

A

IPF, connective tissue, disease, asbestosis

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10
Q

What may be the only finding in early ILD?

A

Reduced Diffusing capacity of lungs for carbon monoxide

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11
Q

What might ABGs show in ILD?

A

Hypoxemia and respiratory alkalosis

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12
Q

What is the one contraindication to lung biopsy?

A

Honey combing on imaging because prognosis is already poor and biopsy wont change management

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13
Q

What is a trans bronchial biopsy?

A

Done during bronchoscopy, biopsy forceps passed through bronchoscope. Helpful to biopsy central locations, not periphery

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14
Q

What are the two options for surgical biopsy?

A

Video assisted thoracoscopic surgery or thoracotomy

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15
Q

What is the most common interstitial lung disease?

A

Idiopathic pulmonary fibrosis

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16
Q

What is the histopathology pattern seen with idiopathic pulmonary fibrosis?

A

Usual interstitial pneumonia (UIP)

17
Q

What are the two pharmacologic treatment options for idiopathic pulmonary fibrosis?

A

Nintedanib: suppresses multiple signaling receptors involved in pathogenesis of fibrosis
Pirfenidone: reduces lung fibrosis though down regulation of production of growth factors and procollagens

18
Q

What is sarcoidosis?

A

A multisystem inflammatory disease that forms non-caseating granulomas

19
Q

What do the non- caseating granulomas in sarcoidosis secrete?

A
  • 1,25 dihydroxyvitamin D (elevated calcium)

- ACE

20
Q

A young female presents with DOE, chest pain, and systemic complaints. She has erythema nodosum, lupus pernio, and granulomatous uveitis. What are you suspicious of?

A

Sarcoidosis

21
Q

What labs will be elevated in sarcoidosis?

A
  • Elevated serum ACE
  • hypercalcemia
  • elevated alkaline phosphatase
22
Q

What is often seen on CXR in sarcoidosis?

A

Hilar adenopathy

23
Q

What is the staging based on CXR of sarcoidosis?

A

Stage 0: Normal
Stage 1: Hilar adenopathy
Stage 2: Hilar adenopathy and diffuse infiltrates
Stage 3: Only diffuse parenchymal infiltrates
Stage 4: Pulmonary fibrosis

24
Q

What is silicosis?

A

A fibronodular lung disease characterized by inhalation of silica dust

25
Q

What kind of pattern do PFTs show for silicosis and coal workers pneumonconiosis?

A

Restrictive

26
Q

What is seen on CXR in patients with chronic simple silicosis and coal workers pneumonconiosis?

A
  • Hilar node calcification (eggshell pattern)

- Small round nodular opacities

27
Q

What is seen on CXR is asbestosis?

A

Opacities in the lower lungs, thickened pleura, and pleural plaques

28
Q

What kind of pattern do PFTs show in asbestosis?

A

Restrictive pattern

29
Q

What is mesothelioma almost always associated with?

A

Asbestosis

30
Q

What is hypersensitivity pneumonitis?

A

An inflammatory syndrome of the lung caused by repetitive inhalation of antigens in a susceptible host

31
Q

Is hypersentivity pneumonitis chronic?

A

No, it is a reversible process that is temporally related to exposure

32
Q

What disease is characterized by necrotizing granulomas?

A

Granulomatosis with polyangiitis (GPA)

33
Q

What disease is an immune mediated systemic vasculitis of small-medium vessels

A

GPA

34
Q

What disease is saddle most deformity associated with?

A

GPA

35
Q

What is the initial treatment for GPA?

A

Cyclophosphamide and corticosteroids