interstitial lung disease Flashcards

1
Q

What are other names for interstitial lung disease

A

Pulmonary fibrosis
Parenchymal disease

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2
Q

What is occurring in the lungs with interstitial lung disease

A

inflammation causing fibrosis and leading to impaired gas exchange

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3
Q

What is the interstitium

A

Space that is between the capillary and the alveoli

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4
Q

What is the most common type of interstitial lung disease

A

idiopathic

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5
Q

What is the epidemiology of interstitial lung disease

A

diagnosis of exclusion

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6
Q

What is the most common presentations of interstitial lung disease

A

progressive, exertion dyspnea is most common

Often have chronic symptoms for months to years

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7
Q

What known causes is interstitial lung disease associated with

A

occupational exposures
autoimmune conditions

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8
Q

How do you workup interstitial lung disease

A

need thorough history
Lab tests to rule out other causes

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9
Q

What in a persons PMH would raise concern for interstitial lung disease

A

Connective tissue disorder
Malignancy
Rheumatologic conditions

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10
Q

What is first line imaging for interstitial lung disease

A

Chest xray (supportive NOT diagnostic)

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11
Q

What is the best imaging test for interstitial lung disease

A

CT
*Biopsy if CT is inconclusive

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12
Q

How can you assess the severity of interstitial lung disease

A

PFTs
- normal early on
- restrictive pattern later on in disease

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13
Q

What are supportive Xray findings for interstitial lung disease

A

Bronchiectasis (tram-track appearance)

Honeycombing
Kerley lines
reticular opacities
military pattern

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14
Q

What is the treatment for interstitial lung disease

A

No real treatment
-goal is to prevent/limit further damage

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15
Q

What are pharmacologic options for interstitial lung disease

A

-Corticosteroids

-antifibrotic agents (pirfenidone/nintedanib) -> idiopathic only

-treat GERD w/ H2 blockers

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16
Q

When is a lung transplant beneficial in interstitial lung disease

A

For refractory disease in patients under 65 who are otherwise healthy

17
Q

What is the survival rate for idiopathic pulmonary fibrosis

A

5 year survival around 50%

18
Q

Which patient population is most affected by idiopathic pulmonary fibrosis

A

Males > 50

19
Q

How do patients with IPF generally present

A

DOE and dry cough for the past year w/ bibasilar end inspiratory crackles on exam

20
Q

What is seen on imaging with IPF

A

“usual interstitial pneumonia” patter
-basilar reticular opacities
-honeycombing
-bronchiectasis

21
Q

What characteristics does a patient need on exam to make a diagnosis of IPF

A

age >65
Idiopathic
-inspiratory crackles
-restrictive PFT
-Classic UIP imaging findings

22
Q

What is sarcoidosis

A

Systemic inflammatory disorder
*Lungs and lymphatic systems are most commonly effected

23
Q

Which population is sarcoidosis most common in

A

black patients
females
20-40 years old (bi-modal w/ second peak around 60)

24
Q

What time of year is sarcoidosis generally present

A

Winter / early spring

25
Q

How is sarcoidosis characterized

A

non-caveating granuloma with no necrotic center

26
Q

What is present in most patients with sarcoidosis

A

pulmonary disease

27
Q

What are extra-pulmonary manifestations of sarcoidosis

A

Dacryocystitis
erythema nodosum
myopathy
arthritic symptoms
Lupus pernio

28
Q

What is lupus pernio

A

violations plaques on the face

29
Q

What is the primary means for diagnosis of sarcoidosis

A

CXR
-hilar adenopathy
-central nodular opacities
0 predilection for upper lobes

30
Q

What is CXR also used for in sarcoidosis besides diagnosis

A

Staging

31
Q

What is stage 1 sarcoidosis

A

hilar adenopathy only

32
Q

What is stage 2 sarcoidosis

A

hilar lymphadenopathy + infiltrates

33
Q

What is stage 3 sarcoidosis

A

infiltrates alone -> adenopathy resolves

34
Q

What is stage 4 sarcoidosis

A

pulmonary fibrosis

35
Q

How do you get a definitive diagnosis of sarcoidosis

A

Biopsy from most accessible lesion

36
Q

When do you treat sarcoidosis

A

If 1+ organs are involved

37
Q

What pharmacological agents can be used for sarcoidosis

A

Glucocorticoids (1st line)
Methotrexate (2nd line)
Azathiopine
hydroxychloroquine