Clin Med: Interstitial Lung Disease

Question 1

Interstitial Lung Disease is AKA

Answer 1

pulmonary fibrosis

Question 2

ILD pathophysiology

Answer 2

inflammation – fibrosis (deposits) – impaired gas exchange

Question 3

Types of ILD

Answer 3

exposure related
autoimmune related
idiopathic (m/c)

Question 4

ILD presentation

Answer 4

often chronic sx
progressive exertional dyspnea
dry cough
ssx of autoimmune disorder
asymptomatic
other: pleuritic CP, hemoptysis

Question 5

ILD associated with

Answer 5

occupational exposures
autoimmune conditions

Question 6

Imaging for ILD

Answer 6

usually CXR firstline
High resolution CT is diagnostic and is best imaging
If CT not diagnostic –> bx

PFTs added to assess severity

Question 7

PE for ILD

Answer 7

non-specific:
bibasilar crackles
clubbing of fingers
PE consistent with Pulm HTN
tachypnea
reduced chest expansion

Question 8

ILD findings on CXR

Answer 8

bronchiectasis (tram-track appearance)
miliary pattern (1-4mm nodules - looks fuzzy)
honeycombing (lacy reticular pattern)
kerley lines (linear opacities)
reticular or reticulonodular opacities (finer pattern than honeycombing)

Question 9

Treatment goal

Answer 9

fibrosis is irreversible so goal is to prevent and limit further damage

Question 10

Treatment (pharm)

Answer 10

Corticosteroids (prednisone)
antifibrotic agents (pirfenidone and nintedanib) for idiopathic pulmonary fibrosis
treatment of GERD (omeprazole or pantoprazole)

Question 11

Treatment (pharm)

Answer 11

Corticosteroids (prednisone)
antifibrotic agents (pirfenidone and nintedanib) for idiopathic pulmonary fibrosis
treatment of GERD (omeprazole or pantoprazole)

Question 12

Treatment (supportive)

Answer 12

supplemental O2
pulm rehab
tobacco cessation
flu and pneumococcal vaccines
lung transplant

Question 13

For refractory ILD only definitive treatment is

Answer 13

lung transplant (for pts under 65 and otherwise healthy and have refractory disease)

Question 14

Idiopathic Pulmonary Fibrosis is when

Answer 14

there is recurrent injury to alveolar epithelium
no significant occupation hx, medication exposures, or PMH

Question 15

Presentation of Idiopathic Pulmonary fibrosis

Answer 15

present with DOE and dry cough, often chronic for 1 yr
classically with bibasilar end inspiratory crackles on exam

Question 16

Characteristic usual interstitial pneumonia pattern on imaging

Answer 16

basilar reticular opacities
honeycombing
bronchiectasis

Question 17

DX criteria for IPF

Answer 17

age less than 65
no known cause of ssx
inspiratory crackles on PE
restrictive PFT
classic UIP imaging findings

Question 18

Treatment of IPF

Answer 18

Corticosteroids (prednisone)
antifibrotic agents (pirfenidone and nintedanib) for idiopathic pulmonary fibrosis
treatment of GERD (omeprazole or pantoprazole)
O2, vaccines, tobacco cessation, lung transplant

Question 19

Sarcoidosis is a __________ disorder

Answer 19

autoimmune
systemic inflammatory disorder

Question 20

Sarcoidosis is less strongly correlated with

Answer 20

fHx

Question 21

Sarcoidosis is characterized by

Answer 21

non-caseating granuloma formation (no necrotic center)

Question 22

Difference between sarcoidosis and tb in granuloma formations

Answer 22

TB - caseating granulomas
Sarcoidosis - non-caseating granulomas

Question 23

Sarcoidosis presentation

Answer 23

DOE, cough, CP, crackles
other systemic sx - autoimmune sx
fatigue, malaise, fever, wt loss, weakness, anorexia

Question 24

CXR sarcoidosis findings

Answer 24

hilar adenopathy
central nodular opacities
predilection for the upper lobes

Question 25

What is used for definitive dx in sarcoidosis

Answer 25

bx - non-caseating granuloma is pathognomonic

Question 26

Sarcoidosis treatment (pharm)

Answer 26

glucocorticoids - prednisone is 1st line
methotrexate - second line
azathioprine
hydroxychloroquine
TNF alpha inhibitors (infliximab)

Question 27

Sarcoidosis: if acute and no to mild sx =

Answer 27

no treatment necessary

Question 28

Sarcoidosis: if one organ involved =

Answer 28

topical/ localized therapy

Question 29

Sarcoidosis: if multi-organ involvement

Answer 29

systemic therapy