Restrictive Disorder

Question 1

What is interstitial lung dz (Diffuse Parenchyma lung dz)?

Answer 1

-inflammation of the parenchyma that has IRREVERSIBLE fibrosis

Question 2

What is parenchyma?

Answer 2

lung tissues that includes the alveolar

Question 3

What is a common finding of interstitial lung dz?

Answer 3

LATE INSPIRATORY CRACKLES

Question 4

What pneumonia is a the most common in interstitial?

Answer 4

idiopathic interstitial pneumonia

Question 5

What population is affected by IIP?

Answer 5

• 65 yo

- there are CXRs (basline) that show fibrosis over years

Question 6

What PE for IIP?

Answer 6

inspiratory crackles

Question 7

What labs of IIP?

Answer 7

• Hi Resolution CT
• patchy fibrosis WITH HONEY COMBING
• broncioalveolar lavage* for suspicions of pathogen
• transbronchial bx*
• surgical lung bx (dangerous)

Question 8

What is broncialveolar lavage?

Answer 8

bronchoscope that is passed through the mouth or nose and sprays the lungs and collected to test for a pathogen

Question 9

What is transbronchial bx?

Answer 9

safer than surgical lung bx and used to differentiate focal and diffuse/cystic and cavity lung dz

Question 10

Tx IIP?

Answer 10

corticosteroids

Question 11

Tx UIP?

Answer 11

lung transplant but only 50% of 5 yr survival rate

Question 12

What is UIP?

Answer 12

Usual Intersitial Pneumonia under IIP

Question 13

What is IIP?

Answer 13

• Idiopathic Interstitial Pneumonia

- under interstitial lung dz which is under fibrotic/restrictive disorder

Question 14

What is sarcoidosis

Answer 14

• underlying systemic dz

- has granulomatous (collection of macrophages) d/t the inflammation in the lungs

Question 15

Who does sarcoidosis affect?

Answer 15

• African Americans
• Females
• 30-40yo

Question 16

PE of sarcoidosis?

Answer 16

• NO crackles
• it is a systemic dz cxing disoder: large parotid glands, hepatosplenomegaly, lymphadenopathy, fever
• INSIDEOUS ONSET
• CLASSIC: erythema around the ankles that are itchy and painful, peripheral neuropathy, arthritis, eye probs

Question 17

Labs for sarcoidosis?

Answer 17

• bilateral hilar adenopathy (enlarged lymph nodes in the hilar part of lung b/c its a systemic dz!)
• CBC: leukopenia (only if pt doesn’t have splenomegaly which is rare); incr ESR (d/t inflammation); hypercalcemia/calciuria (d/t renal dz and increase PTH from the inflammatino of the granulomatous)
• lung bx: non necrosis granulomas

Question 18

Tx for sarcoidosis?

Answer 18

oral corticosteroids (prednisone for months~yrs)

Question 19

Prognosis for sarcoidosis?

Answer 19

best if NO parenchymal involvement (not affecting the lungs and JUST the body)

Question 20

What is penumoconiosis?

Answer 20

having inorganic dusts in the lungs

Question 21

What are the types of penumonocniosis?

Answer 21

• coal worker
• silicosis
• asbestosis

Question 22

What is coal worker?

Answer 22

• the macrophages in the alveolar tries to engulf the coal particles cxing COAL MALCULES
• usually an asymptomatic finding

Question 23

Labs for coal worker?

Answer 23

Hi Res CT:

-diffuse small opacities in upper lung

Question 24

What is siliocosis?

Answer 24

• breathing in silica dust (construction workers)

- asymptomatic finding

Question 25

Labs for siliocosis?

Answer 25

Hi Res CT:

-silicotic nodules w/ eggshell calcifications

Question 26

What is absetosis?

Answer 26

hx of asbestosis fibers (shipyard construction)

Question 27

PE for absetosis?

Answer 27

• PROGRESSIVE dyspnea
• inspiratory crackles
• clubbing
• cyanosis

Question 28

Labs for absetosis?

Answer 28

Hi Res CT:

• linear streaking at the bases
• honeycombing

Question 29

What are the restrictive lung dz?

Answer 29

• sarcoidosis
• interstitial lung dz (IIP –> UIP)
• pneumoconiosis