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Clin Med II- Exam 2 > ILD > Flashcards

ILD Flashcards

1
Q

ILD aka

A

Diffuse Parenchymal Lung Disease (DPLD)

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2
Q

ILD lung damage

A
  • irreversibly enlarged, damaged bronchioles and distorted alveoli
  • honey combing (clustered cycstic air spaces)
  • fibrosis between alveoli decreases gas exchange, reducing oxygen transfer
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3
Q

Causes of ILD

A

idiopathic
autoimmune (RA, scleroderma, sarcoidosis, sjogren)
hazardous material (asbestos, silica, droppings, radiation, hot tubs)
drugs (amiodarone, propanolol, nitrofurantoin, MTX, rituximab)

*generally irreversible

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4
Q

Pathophys of ILD

A

alveolar epithelium damage –> type 2 epithelial cells proliferate (to repair damage) –> repair leads to fibrosis and scarring –> lung stiffens, ability to transport O2 diminished –> hypoxemia

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5
Q

Sx of ILD

A

progressive DOE
persistent non-productive cough
wheezing and chest pain UNCOMMON
Extra-pulm sx: suggestive of CT disease; musculoskeletal pain, weakness, joint pain or swelling, fevers, dry eyes/mouth

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6
Q

PE for ILD

A

Crackles: “velcro”
inspiratory squeaks (high pitched rhonchi)
cor pulmonale - middle/late stages
cyanosis
digital clubbing** - advanced disease
extram pul: ereythema nodosum, gottrons papules

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7
Q

Erythema nodosum

A

sarcoidosis

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8
Q

Gottrons papules

A

dermatomyositis

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9
Q

Crackles

A

pneumo and ILD

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10
Q

clubbing

A

ILD

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11
Q

Dx of ILD

A 
MDD: pulmonologist, radiologist, pathologist
HRCT - best noninvasive
Tissue bx = gold standard (rarely done)
Sero studies - r/o autoimmune
PFT
ABG
BAL
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12
Q

CXR in ILD

A

some normal
ground-glass appearance (non-specific)
reticular “netlike” - most common
Honeycombing (poor prognosis)

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13
Q

poor prognosis for ILD

A

honeycombing

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14
Q

Where is ILD in sarcoidosis

A

upper/central lung fields

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15
Q

Reticular opacities

A

IPF or asbestos

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16
Q

Ground glass

A

drug toxicity, respiratory bronchiolitis ILD

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17
Q

Serologic studies for ILD

A

ANA and RF: done 1st
if (+): Anti-ds-DNA for SLE

If pulm hemorrhage or suspect systemic sx, evaluate for vasculitis: ANCA!

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18
Q

Used to assess disease severity/progress in ILD

A

PFT

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19
Q

PFT in ILD

A

restrictive

  • decreased TLC
  • decreased FEV1 and FVC, but proportional changes lead to normal/increased FEV1/FVC ratio
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20
Q

PFT to dx ILD

A

PFT restrictive pattern + low DLCO

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21
Q

Which ILD has obstructive pattern?

A

sarcoidosis

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22
Q

Low DLCO

A

early finding

signifies alveolar damage- impaired gas exchange

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23
Q

ABG in ILD

A

normal or hypoxemia or respiratory alkalosis

  • may need to perform exercise testing w/ serial ABGs
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24
Q

Bronchoalveolar Lavage (BAL)

A

sampling of distal airways and alveoli: cell counts, cultures, cytology

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25
Q

Cell count in ILD

A

eosinophilic pneumo

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26
Q

When to perform lavage

A

not typically performed w/ HRCT findings c/w IPF

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27
Q

Gold standard for dx of ILD

A

Lung Bx

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28
Q

Indications for lung bx

A

specifying dx (<50 yo, fever,, weight loss, hemoptysis, vasculitis)
atypical/rapidly progressing
unexplain extrapulm manifestations
exclude neoplasm or infection
identify a more treatable process
predict likelihood of response to therapy

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29
Q

Contraindication to lung bx

A

honeycombing

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30
Q

Types of Lung bx

A

transbronchial
surgical: Video assisted thorascopic surgery (VATS), thoracotomy
Endobronchial u/s-guided transbronchial needle aspiration (EBUS-TBNA)

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31
Q

Transbronchial bx

A

during bronchoscopy, bx forceps passed through bronchoscop; good for CENTRAL locations

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32
Q

VATS

A

two small incisions into lateral chest wall

less morbidity

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33
Q

Thoracotomy

A

5-6 cm incision required

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34
Q

EBUS-TBNA

A

special bronchoscope used to evaluate hilar and mediastinal lymph nodes
general anesthesia
done in conjunction w/ TBLB but need to pass different scope

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35
Q

When is EBUS-TBNA useful

A

if sarcoid suspected

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36
Q

Most common ILD

A

Idiopathic Pulmonary Fibrosis (IPF)

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37
Q

who gets IPF

A

> 50 yo
M>F
familial cases at younger age
POOR PROGNOSIS (2-5 yr survival)

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38
Q

Histopathology of IPF and asbestosis

A

Usual Interstitial Pneumonia (UIP)

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39
Q

Presentation of IPF

A

Gradual onset of exertional dyspnea and non-productive cough (>6 months)
Velcro crackles *(inspiratory)
Digital clubbing *

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40
Q

Dx of IPF

A 
6-minute walk test
CXR: reticular opacities in bases, honeycombing
HRCT
PFT: restrictive
Bx NOT REQUIRED
Echo: pulmonary HTN
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41
Q

New Dx criteria for IPF

A

HRCT: UIP pattern, probable, indeterminate or alt. UIP pattern

Lung Bx + BAL if: probable UIP, indetermintate UIP or alt. dx

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42
Q

Tx for IPF

A 
consult: pulmonologist, MMD
Poor tx options, not much work
**Tx for GERD (even if not sx
Nintedanib (TKI)
Pirfenidone: reduced fibrosis by downregulation GF and procollagens
Smoking cessation, UTD vaccines
Supplemental O2 if needed`
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43
Q

Meds for IPF

A

Nintedanib

Pirfenidone

44
Q

Who gets sarcoidosis

A

F>M
African Americans
ages 20-40

45
Q

What is sarcoidosis

A

Non-caseating granulomas- secreted dihydroxyvitamin D, so serum levels elevated

46
Q

Serum levels in sarcoidosis

A

elevated Ca and Vit D

Secrete ACE which acts as cytokine

47
Q

Hx of sarcoidosis

A 
DOE, chest pain, cough, hemoptysis (rare)
systemic complains (fever, anorexia)
48
Q

Extra pulm findings in sarcoidosis

A

erythema nodosum
Lupus pernio
granulomatous uveitis
arthralgia

49
Q

Dx for sarcoidosis

A

Restrictive pattern
reduced DLCO
CXR: ADENOPATHY
Bx usually required: EBUS-TBLB

50
Q

Labs for sarcoidosis

A

elevated serum ACE, Ca
hypercalciuria
elevated ALP

51
Q

Staging of sarcoidosis

A 
0- normal
1- hilar adenopathy
2- hilar adeno + diffus infiltrates
3- only diffuse parenchymal infiltrates
4- pulm fibrosis
52
Q

Tx for sarcoidosis

A

consult
tx if >stage 2 and symptomatic
High dose corticosteroid (PJP prophylaxis)
MTX alternative
Chlroquine/hydroxychloroquine for cutaneous lesions, neuro manifestations, hypecalcemia
Topical steroids for ocular disease
Lung transplant in stage IV

53
Q

Main drug for sarcoidosis

A

high dose corticosteroid (taper over 6 months)

54
Q

What is pneumoconiosis?

A

general term for lung disease caused by inhalationa nd deposition of mineral dust

55
Q

Types of pneumoconiosis

A

asbestosis
silicosis
coal worker’s pneumoconiosis

56
Q

Silicosis

A

fibronodular lung disease

inhaling silica dust (alpha-quartz or silicone dioxide)

57
Q

Occupations at risk for silicosis

A

MINING
construction
granite cutting
pottery making

58
Q

CXR in silicosis

A

enlarging opacities even after exposure eliminated, can cavitate (r/o TB)

59
Q

PFT in silicosis

A

restrictive

massive fibrosis leading to severe restriction

60
Q

Chronic simple silicosis

A

10-12 yrs exposure
may be asymp.
non-progressive once exposure eliminated
Hilar node calcification (EGGSHELL PATTERN)
small round opacities (silicotic nodules)

61
Q

Chronic complicated silicosis

A

> 20 yrs exposure
progressive even after exposure eliminated
tachypnea, prolonged expiration, rhonchi, wheezing, rales
clubbing uncommon
cyanosis
cor pulmonale

62
Q

Eggshell pattern

A

chronic simple silicosis

63
Q

Management of silicosis

A 
consult
no tx alters disease cource
corticosteroids may benefit ACUTE phase
eliminate exposure
vaccinate
elevated TB risk, treat latent TB
eval for lung transplant candidacy
64
Q

Asbestosis epidemiology

A

presents 15-20 yrs of exposure; dose-dependent
40-75 YO
<>F
occupation: construction, mechanics, pipefitters, plumbers, welders, janitors, shipyard, insulation workers

65
Q

Asbestosis is associated w/

A

bronchogenic CA and malignant mesothelioma

66
Q

PE for asbestosis

A 
no specific s/sx
insidious onset - dyspnea, reduced exercise tolerance, chest discomfort
dry cough
end-inspiratory rales
digital clubbing
67
Q

Dx for asbestosis

A

CXR: opacity in lower lung, thickened pleura, PLEURAL PLAQUES
Open lung bx: usually not indicated, provides definitive dx (asbestos bodies)
PFT: restrictive

68
Q

Egg shell

A

silicosis

69
Q

Pleural plaques

A

asbestosis

70
Q

Pleural plaque location

A

diaphragmatic or parietal pleura of 6th-9th rib

71
Q

Management of Asbestosis

A 
consult- may need long term O2
No drugs alter disease
Goal: eliminate progression, reduce risk of other disorders
Vaccinate: influenza, pneumovax
Promptly treat resp. infection
evaluate lung lesions
Prevention: avoid exposure, mask
72
Q

Associated w/ high risk f or malignancy

A

asbestosis- smoking increases risk

73
Q

Mesothelioma

A

CA almost ALWAYS associated w/ asbestos exposure

74
Q

Mesothelioma epidemiology

A 
short term exposure to asbestos (1-2 yrs)
most common in pleura
can occur in peritoneum or pericardium
not caused by smoking
poor prognosis (6-12 mo)
75
Q

Vasculitic ILD

A

granulomatosis w/ polyangiitis (GPA)

76
Q

GPA

A

immune-mediated systemic vasculitis of small-medium vessel

77
Q

GPA aka

A

“ANCA-associated vasculitis”

78
Q

GPA characterized by

A

necrotizing granulomas of upper and lower RT

79
Q

Epidemiology of GPA

A 
RARE
northern european descent
men = women
35-55 yo
relapse common
80
Q

Most common in african americans

A

sarcoidosis

81
Q

Hx of GPA

A

recurrent resp infections

nonspecific constitutional sx: fever, weight loss, night sweats, loss of appetite, fatigue/lethargy

82
Q

PE of GPA

A

ocular involvement: conjunctivitis, episcleritis, uveitis, RAO
ENT: chronic sinusitis, rhinitis, epistaxis, SADDLE NOSE
Pulm: infiltrates, cough, hemoptysis, dysnpea, stridor
MSK: arthralgia
Renal failure, erythrocyte casts
CN palsies, sensorimotor polyneuropathy
Skin: purpura, ulcers
Cardiac: pericarditis, coronary vasculitis

83
Q

Necrotic blistering purpura

A

GPA

84
Q

Saddle nose deformity

A

GPA

85
Q

egg shell

A

silicosis

86
Q

Pleural plaques

A

asbestosis

87
Q

Dx of GPA

A

CXR: variable, nodules which may cavitate
HRCT: stellate shaped peripheral pulmonary arteris (“vasculitis”, feeding vessels leading to nodules/cavities, diffuse alveolar hemorrhage
Bronchoscopy: only indicated if hemorrhage or intervention (stenting)

Tissue bx: vasculitis, granulomatous inflammation

88
Q

Labs for GPA

A

ESR/CRP elevated
CBC/CMP: normocytic anemia, thrombocytosis, leukocytosis, renal involvement (BUN/Cr)

UA: proteinuria, RBC casts (GN)
C-ANCA!
consider RF and ANA

89
Q

C-ANCA

A

GPA

90
Q

Management for GPA

A

consult rheum and pulm

cyclophosphamide (immunosuppressant) & corticosteroid

91
Q

Cyclophosphamide toxicities

A

cardiac (CHF), heme (thrombocytopenia), renal (hemorrhagic cycstitis, tubular necrosis), GI (n/v/d)

92
Q

Cyclophosphamide

A

used to treat GPA; improves prognosis

93
Q

Hypersensitivity pneumonitis cause

A

repetitive inhalation of antigens in a susceptible host

94
Q

Potential exposures for Hypersensitivity pneumonitis

A

Bacteria, fungi, mold
proteins, chemiicals
environmental (droppings, fur/feathers, hot tubs, hardwood, dust)

farmers, cattle, metalworkers, plastic manufactureres, grain/flour processor, vets

95
Q

Reversible process

A

hypersensitivity pneumonitis (several years)

96
Q

Hx for hypersensitivity pneumonitis

A

flu-like syndrom w/i hours of exposure if acute
insidious cough dyspnea, fatigue if subactue
progressive dyspnea, cough, fatigue, malaise in chronic

97
Q

PE for hypersensitivity pneumonitis

A

diffuse, fine bibasilar crackles, fevers, tachypnea, muscle wasting, clubbing, weight loss

98
Q

Imaging for hypersensitivity pneumonitis

A

interstitial inflammation, honeycombing, centrilobular fibrosis, peribronchiolar fibrosis

99
Q

Management of hypersensitivity pneumonitis

A

consul pulm
antigen avoidance/prevention
acute disease: no tx

Corticosteroids in severe disease

Bronchodilators, antihistamines, ICS are adjunct tx

100
Q

Complications of ILD

A 
pulm HTN
cor pulmonale/CVD (hypertrophy or dilation of RV)
pneumothorax
PE
Pulmonary ifnection
elevated cancer risk
progressive resp. insufficiency
101
Q

velco-like inspiratory crackles in bilateral bases

A

IPF

102
Q

Bibasilar Reticulonodular opacities

A

IPF

103
Q

hypercalcemia

A

sarcoidosis

104
Q

hilar adenopathy

A

sarcoidosis

105
Q

calcified pleural plaques

A

asbestosis

106
Q

eggshell

A

silicosis

Clin Med II- Exam 2 (7 decks)

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