Interstitial lung disease - collins Flashcards

1
Q

what are other names for interstitial lung disease

A

diffuse parenchymal disease
pulmonary fibrosis

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2
Q

what is interstitial lung disease

A

group of lung disorders that impair the interstitium - damage also extends to the parenchyma (capillaries, alveoli, bronchioles)
does not extend proximal to bronchioles
leads to impaired gas exchange

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3
Q

what is the pathophysiology of interstitial lung disease

A

inflammation -> fibrosis (deposits of connective tissue) -> impaired gas exchange

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4
Q

what are the most common types of interstitial lung disease

A

exposure related (drug and occupation/environmental)
autoimmune related
idiopathic (Most common)

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5
Q

how many types of interstitial lung disease are there

A

over 200 - we only talk about 3 major types

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6
Q

what are drug induced/exposure related interstitial lung disease

A

chemotherapy, methotrexate, amiodarone, macrobid

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7
Q

what are occupation/environmental related interstitial lung disease

A

inorganic or organic dust
gas/fumes
radiation

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8
Q

what are the autoimmune related interstitial lung disease

A

rheumatoid arthritis
SLE
Scleroderma
polymyositis
dermatomyositis
sjogrens syndrome

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9
Q

what are the different types of idiopathic interstitial lung disease

A

Idiopathic pulmonary fibrosis (IPF)
Sarcoid

acute interstitial pneumonia (AIP)
non-specific interstitial pneumonia (NSIP)
cryptogenic organizing pneumonia (COP)

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10
Q

what is the presentation of interstitial lung disease

A

often chronic symptoms (Months- years)
progressive exertional dyspnea m/c
may present as simple dry cough, SSX of autoimmune disease, asympathomatic (screening)

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11
Q

what are possible exam findings for interstitial lung disease

A

bibasilar crackles
clubbing of digits
PE consistent with pulmonary HTN
tachypnea
reduced chest expansion

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12
Q

what is the workup for interstitial lung disease

A

starts with thorough history
occupational history
thorough PMH with treatments: CT dz, malignancy, Rheumatologic condition
Family hx
lab tests to rule out other causes and check for known causes

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13
Q

what lab tests are typically run for interstistial lung disease

A

CBC, CMP, rheumatologic testing, HIV, HCV, etc

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14
Q

What are supportive CXR findings for interstitial lung disease

A

bronchiectasis 9Tram-track appearance)
miliary pattern
honeycombing
kerley lines
reticular or rediculonodual opacities

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15
Q

what are PFTs used for with interstitial lung disease

A

assess severity, prognosis and monitoring

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16
Q

what is a diagnostic test for ILD

A

biopsy

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17
Q

What are supportive CT findings for ILD

A

bronchiectasis
miliary pattern
honeycombing (coarse reticular)
kerley lines
reticular or rediculonobular opacities

18
Q

what is the treatment for ILD

A

fibrosis is irreversible - goal is to prevent/limit further damage
no BEST treatment
coricosteroids (pred)
antifibrotic agent (pirfenidone and nintedanib)
treat GERD (iansoprazole, omeprazole, pantroprazole)

19
Q

what are other supportive treatment options for ILD

A

supplemental O2
Pulmonary rehabilitation
Tobacco cessation
Flu and pneumococcal vaccines

lung transplant for refractory disease

20
Q

what is the only definitive treatment for ILD

A

lung transplant for refractory

21
Q

what is another name for idiopathic pulmonary fibrosis

A

usual interstitial pneumonia (histologic description)

22
Q

what is the presentation of Idiopathic pulmonary fibrosis

A

DOE and dry cough, often chronic (1 year)
classiually with bibasilar end inspiratory crackles on exam
‘usual interntitail pneumonia pattern’ on imaging

23
Q

what are usual interstitial pneumonia imaging findings

A

basilar reticular opacities
honeycombing
bronchiectasis

24
Q

what are the easy diagnostic criteria

A

Age > 65
no known cause of SSx (idiopathic)
inspiratory crackles on PE
Restrictive PFT
Classic UIP imaging findings

25
Q

what is sarcoidosis

A

systemic inflammatory disorder - lungs and lymph most commonly affected
Black pts, F>M, age 20-40s, second peak around age 60
increased incidence in winter/early spring

26
Q

what is sarcoidosis characterized by

A

non-caseating granuloma formation - no necrotic center

27
Q

what are the common symptoms of sarcoidosis

A

DOE, cough, chest pain, crackles
systemic: fatigue, malaise, weakness, anorexia, weight loss, fever

28
Q

what are extra-pulmonary manifestations of sarcoidosis

A

dacryocystosis
erythema nodosum
myopathy/muscle weakness
hepatic involvement
arthritis
lupus pernio
other skin rashes

29
Q

what is dacryocystitis

A

inflammation of lacrimal sac

30
Q

what is erythema nodosum

A

palpable, tender, red/violet subQ nodules

31
Q

what is lupus pernio

A

violacenous plaques on the face

32
Q

what is the primary means for diagnosis of sarcoidosis

A

CXR
hilar adenopathy
central central nodular opacities
predilection for the upper lobes

33
Q

what is CXR also used for with sarcoidosis

A

diagnosis and staging

34
Q

what is the definitive diagnostic test for sarcoidosis

A

biopsy

35
Q

What is the general approach to treatment of sarcoidosis

A

if acute or no to mild symptoms - no tx
if one organ involved - localized/topical therapy
if multi-organ involvement - systemic therapy

36
Q

what are the pharmacotherapeutic agents used for sarcoidosis

A

glucocorticoids (pred) - first line
methotrexate - second line

37
Q

how many stages are with Sarcoidosis

A

1 to 4

38
Q

what is seen on CXR stage 1 sarcoidosis

A

hilar adenopathy only

39
Q

what is seen on CXR in stage 2 sarcoidosis

A

hilar adenopathy + infiltrates

40
Q

what is seen on CXR in stage 3 sarcoidosis

A

infiltrates alone, adenopathy resolves

41
Q

what is seen on CXR in stage 4 sarcoidosis

A

pulmonary fibrosis

42
Q

what is pathognomonic of sarcoidosis

A

non-caseating granulomas