ILD Flashcards
1
Q
ILD aka
A
Diffuse Parenchymal Lung Disease (DPLD)
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
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
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
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
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
7
Q
Erythema nodosum
A
sarcoidosis
8
Q
Gottrons papules
A
dermatomyositis
9
Q
Crackles
A
pneumo and ILD
10
Q
clubbing
A
ILD
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
12
Q
CXR in ILD
A
some normal
ground-glass appearance (non-specific)
reticular “netlike” - most common
Honeycombing (poor prognosis)
13
Q
poor prognosis for ILD
A
honeycombing
14
Q
Where is ILD in sarcoidosis
A
upper/central lung fields
15
Q
Reticular opacities
A
IPF or asbestos
16
Q
Ground glass
A
drug toxicity, respiratory bronchiolitis ILD
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!
18
Q
Used to assess disease severity/progress in ILD
A
PFT
19
Q
PFT in ILD
A
restrictive
- decreased TLC
- decreased FEV1 and FVC, but proportional changes lead to normal/increased FEV1/FVC ratio
20
Q
PFT to dx ILD
A
PFT restrictive pattern + low DLCO
21
Q
Which ILD has obstructive pattern?
A
sarcoidosis
22
Q
Low DLCO
A
early finding
signifies alveolar damage- impaired gas exchange
23
Q
ABG in ILD
A
normal or hypoxemia or respiratory alkalosis
- may need to perform exercise testing w/ serial ABGs
24
Q
Bronchoalveolar Lavage (BAL)
A
sampling of distal airways and alveoli: cell counts, cultures, cytology
25
Cell count in ILD
eosinophilic pneumo
26
When to perform lavage
not typically performed w/ HRCT findings c/w IPF
27
Gold standard for dx of ILD
Lung Bx
28
Indications for lung bx
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
29
Contraindication to lung bx
honeycombing
30
Types of Lung bx
transbronchial
surgical: Video assisted thorascopic surgery (VATS), thoracotomy
Endobronchial u/s-guided transbronchial needle aspiration (EBUS-TBNA)
31
Transbronchial bx
during bronchoscopy, bx forceps passed through bronchoscop; good for CENTRAL locations
32
VATS
two small incisions into lateral chest wall
| less morbidity
33
Thoracotomy
5-6 cm incision required
34
EBUS-TBNA
special bronchoscope used to evaluate hilar and mediastinal lymph nodes
general anesthesia
done in conjunction w/ TBLB but need to pass different scope
35
When is EBUS-TBNA useful
if sarcoid suspected
36
Most common ILD
Idiopathic Pulmonary Fibrosis (IPF)
37
who gets IPF
>50 yo
M>F
familial cases at younger age
POOR PROGNOSIS (2-5 yr survival)
38
Histopathology of IPF and asbestosis
Usual Interstitial Pneumonia (UIP)
39
Presentation of IPF
Gradual onset of exertional dyspnea and non-productive cough (>6 months)
Velcro crackles *(inspiratory)
Digital clubbing *
40
Dx of IPF
```
6-minute walk test
CXR: reticular opacities in bases, honeycombing
HRCT
PFT: restrictive
Bx NOT REQUIRED
Echo: pulmonary HTN
```
41
New Dx criteria for IPF
HRCT: UIP pattern, probable, indeterminate or alt. UIP pattern
Lung Bx + BAL if: probable UIP, indetermintate UIP or alt. dx
42
Tx for IPF
```
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`
```
43
Meds for IPF
Nintedanib
| Pirfenidone
44
Who gets sarcoidosis
F>M
African Americans
ages 20-40
45
What is sarcoidosis
Non-caseating granulomas- secreted dihydroxyvitamin D, so serum levels elevated
46
Serum levels in sarcoidosis
elevated Ca and Vit D
| Secrete ACE which acts as cytokine
47
Hx of sarcoidosis
```
DOE, chest pain, cough, hemoptysis (rare)
systemic complains (fever, anorexia)
```
48
Extra pulm findings in sarcoidosis
erythema nodosum
Lupus pernio
granulomatous uveitis
arthralgia
49
Dx for sarcoidosis
Restrictive pattern
reduced DLCO
CXR: ADENOPATHY
Bx usually required: EBUS-TBLB
50
Labs for sarcoidosis
elevated serum ACE, Ca
hypercalciuria
elevated ALP
51
Staging of sarcoidosis
```
0- normal
1- hilar adenopathy
2- hilar adeno + diffus infiltrates
3- only diffuse parenchymal infiltrates
4- pulm fibrosis
```
52
Tx for sarcoidosis
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
Main drug for sarcoidosis
high dose corticosteroid (taper over 6 months)
54
What is pneumoconiosis?
general term for lung disease caused by inhalationa nd deposition of mineral dust
55
Types of pneumoconiosis
asbestosis
silicosis
coal worker's pneumoconiosis
56
Silicosis
fibronodular lung disease
| inhaling silica dust (alpha-quartz or silicone dioxide)
57
Occupations at risk for silicosis
MINING
construction
granite cutting
pottery making
58
CXR in silicosis
enlarging opacities even after exposure eliminated, can cavitate (r/o TB)
59
PFT in silicosis
restrictive
| massive fibrosis leading to severe restriction
60
Chronic simple silicosis
10-12 yrs exposure
may be asymp.
non-progressive once exposure eliminated
Hilar node calcification (EGGSHELL PATTERN)
small round opacities (silicotic nodules)
61
Chronic complicated silicosis
>20 yrs exposure
progressive even after exposure eliminated
tachypnea, prolonged expiration, rhonchi, wheezing, rales
clubbing uncommon
cyanosis
cor pulmonale
62
Eggshell pattern
chronic simple silicosis
63
Management of silicosis
```
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
Asbestosis epidemiology
presents 15-20 yrs of exposure; dose-dependent
40-75 YO
<>F
occupation: construction, mechanics, pipefitters, plumbers, welders, janitors, shipyard, insulation workers
65
Asbestosis is associated w/
bronchogenic CA and malignant mesothelioma
66
PE for asbestosis
```
no specific s/sx
insidious onset - dyspnea, reduced exercise tolerance, chest discomfort
dry cough
end-inspiratory rales
digital clubbing
```
67
Dx for asbestosis
CXR: opacity in lower lung, thickened pleura, PLEURAL PLAQUES
Open lung bx: usually not indicated, provides definitive dx (asbestos bodies)
PFT: restrictive
68
Egg shell
silicosis
69
Pleural plaques
asbestosis
70
Pleural plaque location
diaphragmatic or parietal pleura of 6th-9th rib
71
Management of Asbestosis
```
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
Associated w/ high risk f or malignancy
asbestosis- smoking increases risk
73
Mesothelioma
CA almost ALWAYS associated w/ asbestos exposure
74
Mesothelioma epidemiology
```
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
Vasculitic ILD
granulomatosis w/ polyangiitis (GPA)
76
GPA
immune-mediated systemic vasculitis of small-medium vessel
77
GPA aka
"ANCA-associated vasculitis"
78
GPA characterized by
necrotizing granulomas of upper and lower RT
79
Epidemiology of GPA
```
RARE
northern european descent
men = women
35-55 yo
relapse common
```
80
Most common in african americans
sarcoidosis
81
Hx of GPA
recurrent resp infections
| nonspecific constitutional sx: fever, weight loss, night sweats, loss of appetite, fatigue/lethargy
82
PE of GPA
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
Necrotic blistering purpura
GPA
84
Saddle nose deformity
GPA
85
egg shell
silicosis
86
Pleural plaques
asbestosis
87
Dx of GPA
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
Labs for GPA
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
C-ANCA
GPA
90
Management for GPA
consult rheum and pulm
cyclophosphamide (immunosuppressant) & corticosteroid
91
Cyclophosphamide toxicities
cardiac (CHF), heme (thrombocytopenia), renal (hemorrhagic cycstitis, tubular necrosis), GI (n/v/d)
92
Cyclophosphamide
used to treat GPA; improves prognosis
93
Hypersensitivity pneumonitis cause
repetitive inhalation of antigens in a susceptible host
94
Potential exposures for Hypersensitivity pneumonitis
Bacteria, fungi, mold
proteins, chemiicals
environmental (droppings, fur/feathers, hot tubs, hardwood, dust)
farmers, cattle, metalworkers, plastic manufactureres, grain/flour processor, vets
95
Reversible process
hypersensitivity pneumonitis (several years)
96
Hx for hypersensitivity pneumonitis
flu-like syndrom w/i hours of exposure if acute
insidious cough dyspnea, fatigue if subactue
progressive dyspnea, cough, fatigue, malaise in chronic
97
PE for hypersensitivity pneumonitis
diffuse, fine bibasilar crackles, fevers, tachypnea, muscle wasting, clubbing, weight loss
98
Imaging for hypersensitivity pneumonitis
interstitial inflammation, honeycombing, centrilobular fibrosis, peribronchiolar fibrosis
99
Management of hypersensitivity pneumonitis
consul pulm
antigen avoidance/prevention
acute disease: no tx
Corticosteroids in severe disease
Bronchodilators, antihistamines, ICS are adjunct tx
100
Complications of ILD
```
pulm HTN
cor pulmonale/CVD (hypertrophy or dilation of RV)
pneumothorax
PE
Pulmonary ifnection
elevated cancer risk
progressive resp. insufficiency
```
101
velco-like inspiratory crackles in bilateral bases
IPF
102
Bibasilar Reticulonodular opacities
IPF
103
hypercalcemia
sarcoidosis
104
hilar adenopathy
sarcoidosis
105
calcified pleural plaques
asbestosis
106
eggshell
silicosis
