Interstitial Lung Dz/fibrosis

Question 0

3 ways in which lung is affected in ILD

Answer 0

1 parenchyma (tissue) is damaged

2. Walls of alveoli become inflamed (bronchioles or capillaries may be involved)
3. Scarring (FIBROSIS) begins in the interstitium (tissue bwt alveoli) and LUNGS BECOME STIFF

Question 1

Is ILD malignant or infectious

Answer 1

no

Question 2

Which other dzs is ILD associated with

Answer 2

sarcoidosis
exposure to asbestos, coal dust, aluminum
drugs: macrobid, minocycline, amiodarone (JG), STATINS

Question 3

2 main sx of ILD

and PE findings

Answer 3

NONPRODUCTIVE dry cough and SOB
Wheezing and CP UNCOMMON
PE:
crackles (lower lung bases)
COR PULMONALE (late)
Cyanosis (late)
Digital Clubbing (late)
ERYTHEMA NODUSUM in sarcoidosis

Question 4

Abnormal CXR shows which 3 characteristic findings

Answer 4

GROUND GLASS APPEARANCE (Brds!) early: Hazy opacity (ass with inflam)
RETICULAR (netlike) MOST COMMON, nodular or mixed pattern
HONEYCOMBING (small cyst spaces) is POOR PROGNOSIS

Question 5

What do you expect to see on PFT?

Answer 5

RESTRICTIVE = interstitial:
decreased TLC
decreased FEV1 and FVC but both changes so ratio is NORMAL (or increased)

Question 6

TLC

Spirometry

Answer 6

total amt of air in lung after full inspiration
normal is 80%
Spirometry:
FEV1 - forced expiratory volume (FEV) in 1 second )80% normal)
FVC - forced vital capacity - amnt forcefully exhaled after max inhale: all air you can exhale
ratio - normal 70-80%

Question 7

what do you expect in restrictive PFT

Answer 7

TLC decreased
FVC decreased because can’t breath in
ratio normal

Question 8

what do you expect in obstructive PFT

Answer 8

TLC increased (bc can't breath out?)
FVC normal

Ratio is DECREASED

Question 9

DLCO in ILD is

Answer 9

decreased (maybe the only finding in early stage)
Diffusing capacity = ability of gas to cross from air to interstitium to blood
Measure body’s ability to absorb CO from signle inhalation
Diffusing capacity of Lung for CO.

Question 10

ABG

Answer 10

normal resting but severe exercise or sleep induced hypoxemia (may need serial excercise testing with ABGs)
or repiratory alkalosis (Low Pco2 because hyperventilating and losing CO2, so losing Hs)

Question 11

GOld standard to Dx ILD

Answer 11

Lung biopsy
to make definite Dx or to stage
indicated 
1) r/o CA or inf
2) to provide spec dx when:
-atypical or progressive sx
-  <50 yo
-fever, wgt loss, hemoptysis to r/o?
-sx wtih normal or atyp CXR
-need to confirm dx before staring serious therapy
- unexplained extrapulm manifest (erythema NODOsus)
- unexpl. pulm HTN, or cardiomegaly
-rapid clin deterioration
-sudden change in CXR

Question 12

Types of lung biopsy

Answer 12

fiberoptic BRONCHOSCOPY with transbronchial lung biopsy:
less invasive but less tissu
often initial proceedure of CHOICE, esp if sarcoidosis, infec suspected
- Thoracoscopy
- open lung biopsy

Question 13

Complications of ILD

Answer 13

• pulm HTN - Cor Pulmonale (R ventricular hypertrophy) - R hrt failure:
  jvd, hepatomegaly, pedal edema (peripheral= R sided HF)
• pneumothorax (collapsed lung)
• high CA risk
  -progr resp insufficiency

Question 14

Cardiac blood flow to L V is from

Answer 14

LUNGS:
from lungs to LV - to Aorta - to tissues to RA/RV - to pulm arteries (r and l) - to lungs
If blood backs up in LV - back up to lung and get pulm edema
If blood backs up in RV - back up to tissues and get periph edema

Question 15

ILD overview

Answer 15

Inflammations -> fibrosis (scarred lung tissues)
Sx: DOE, dry cough
CXR: bilat opacities, reticular most common
PFT: can’t expand: restrictive - decreased TLC but ratio normal
DLCO low
Hypoxemia, Resp Alkalosis (low Pco2)

Question 16

ILD classes

Answer 16

Known cause:
occupational and envir exposures:
inorganic dust (asbestos, silica, hard metals)
organic dust (bacreria, animal proteins0
gases, fumes

Drugs and poisons

• chemo med
• ABx: macrobid (rare)
• radiation CA tx

Infections

Question 17

IDL classes

Answer 17

Dur to known dz:
Sarcordosis
CT or autoimm dz:
- scleroderma 
-SLE, RA
-Polymyositis/Derma....
-Syst Vasculitis

ILD due to unknown/idiopathic cause:
Idio Interstitial pneumonias (PNA)
Idio Pulm Fibrosis (IPF)

Question 18

PneumoCONIOSIS

Answer 18

any dz of resp tract due to inhalation of dust particles:

ASBESTOSIS, SILICOSIS

Question 19

ASBESTOSIS

Answer 19

M>F
occupation hx: construction, plumbers, welders, janitor, shipyard, auto mechanics
linked to cancer and mal mesothelioma: smoking greatly increasis risk

Question 20

Mesothelioma

Answer 20

Ca ass’d with ASBESTOS exposure:
may be short term 1-2 yrs
CA in mesothelium (protective lingin that covers most organs)
MOST COMMON IN PLEURA (lining of lungs and chest)
can occur in peritonium or pericardium
NOT CAUSED BY SMOKING
poor prognosis and rare

Question 21

PE of Asbestosis

Answer 21

No spec sx
insidious sudden onset - SOB, reduced exc tolerance
usu DRY cough
insp crackler
DIGITAL CLUBBING (40%) - no severity correlation

Question 22

Asbestosis dx studies

Answer 22

CXR - opacities in LOWER lungs, thickened pleura and plaques
(HX, PE, SX AND CXR -> suggest Dx)

Open lung biopsy - definite dx but not indicated
histology will show fibrosis and asbestos through microscope

Question 23

ASbestosis PFTs

Answer 23

Restrictive: can’t inhale :
decreased TLC
decrease FEV1
normal ratio

Question 24

Asbestosis mngt

Answer 24

SMOKING CESSATION consult pulm - long term O2 no drugs vaccinate : influenza, pneumovax

Question 25

Al Asbestos is

Answer 25

50 yo, male, smoker, pipefitter with PLEURAL PLAQUES and irreversible lung damage

Question 26

Silicosis

Answer 26

type of pneumoconiosis FibroNodular lung dz inhalation of alpha quartz (granite, slate, sandstone) or silicon dioxide mining, construction, granite cutting, pottery making

Question 27

Silicosis

Answer 27

``` no race or age M>f smoking increases riks Acute or subacute Chronic: simple or complicated ```

Question 28

Chronic SIMPLE slicicosis

Answer 28

10-12 yr exposure may be asx NON PROGRESSIVE ONCE EXPOSURE ELIMINATED HILARE node calcificaiton (eggshell pattern) small round opacities (silicotic nodules) on CXR

Question 29

Chronic COMPLICATED silicosis

Answer 29

``` >20 yr exp progresses even after stop exp PE Tachypnea, prolonged expiration (like in chronic bronchitis, COPD) rhonci, wheezing, rales no clubbing (uncommon) cyanosis in advanced COR pulmonale - advanced, increased pulm HTN bc can't get blood into restrictive fibrous tissue ```

Question 30

what are on CXR for chronic complicated silicosis

Answer 30

CXR : enlarging opacities even after exposure is eliminated, can cavitate (R/O TB0 PFT: restrictive: cant inhale: decreased TLC, normal ratio

Question 31

Sam silicosis is

Answer 31

male, miner, indeterminate age, SMOKER with cxr eggshel calcification

Question 32

Sarcoidosis

Answer 32

unknow etiology NonCaseating GRANULOMAS (vs TB) - predom in lungs - can affect: hrt, liver, spleen, joints, skin bones - can have spont resolution of sx AA> whites 20-40 yo Sx: varied, asx to multisystem dz; 45% have systemic fever, anorexia, arthralgias DOE, cough, chest pain arthritis, cranial nerve palsies, visual dist, ERYTHEMA NODUSUM

Question 33

erythema nodosum is part of

Answer 33

sarcoidosis

Question 34

NonCaseating Granulomas are part of

Answer 34

sarcoidosis

Question 35

CXR stages 4 is | PFTs

Answer 35

pulm fibrosis | restrictive in late: can't inhale = decreased TLC, but ratio normal

Question 36

most common pattern of LAD in sarcoidosis

Answer 36

bilat symm hilar adn R paratracheal mediastinal adenopathy

Question 37

sarcoidosis labs

Answer 37

HyperCa (increased Vit D activity) high ESR (inflamm dz) ghigh serium protein (excess immunoglobulins) HIGH SERUM ACE in 60% most cases must biopsy to Dx fiberoptic bronchoscoty with transbronchial biopsy

Question 38

what is the most common lab finding in sarcoidosis

Answer 38

HIGH SERUM ACE IN 60%

Question 39

SALLY Sarcoidosis

Answer 39

AA female, 30ish, non-smoker wtih HILAR ADEnopathy, she's an ACE