Pulmonology #2 (Restrictive Lung Diseases)

Question 1

What is typical on a PFT for restrictive lung disease?

Answer 1

-TLC, FVC, VC all decreased
-FEV1/FVC increased (easier to breathe out quickly)
–the lungs cannot expand fully because they are RESTRICTED from doing so

Question 2

Idiopathic pulmonary fibrosis, which is ________, is MC in men > 40 and smokers. What are symptoms of this condition?

Answer 2

Scar tissue within the lungs due to unknown cause

-Dyspnea, dry/nonproductive cough, dry crackles at the base of the lungs

Question 3

Diagnostics for idiopathic pulmonary fibrosis and what do they show?

PFT:
CXR:
Chest CT:
Biopsy:

Answer 3

PFT: Restrictive pattern (FVC, VC, TLC, DLCO decreased)

CXR: reticular opacities (honeycombing)

CT: reticular honeycombing, focal ground-glass opacification

Biopsy: honeycombing

Question 4

Treatment for idiopathic pulmonary fibrosis

Answer 4

-Prednisone may help scarring
-Oxygen improves symptoms
-Smoking cessation
-Lung transplant is the only cure

Question 5

What are pneumoconioses?

What is the general pathology of these conditions?

Answer 5

Group of lung diseases caused by inhalation of specific types of dust

Inhale something –> scarring of the lung tissue –> unable to expand completely

Question 6

Coal Worker’s Lung (Black Lung Disease) is caused by inhalation of coal dust particles. Although general symptoms occur such as dyspnea, fine crackles/rales, there is a syndrome that can occur with this.

What part of the lungs does this condition affect?

Name and describe the syndrome that can occur with Coal Worker’s Lung.

Answer 6

Upper lobes of the lungs (coal is low, so think opposite)

Caplan Syndrome: RA + Coal Worker’s Lung

Question 7

What is seen on chest X-ray for Coal Worker’s Lung?

Answer 7

Small nodules in the upper lung with hyperinflation of lower lobes in obstructive pattern (resembles emphysema)

Question 8

A lung biopsy for coal worker’s lung shows what?

Answer 8

Dark/black lungs

Question 9

Asbestosis affects what part of the lungs? Where do you get this condition from?

Answer 9

Lower lobes

Inhalation of asbestos (siding and insulation on old houses)
-Destruction, repair, renovation of old buildings. Ship building.

Question 10

What is seen on CXR for asbestosis?

Answer 10

Pleural plaques (thickening) involving the lower lobes
-Shaggy heart sign: indistinct heart border, ground glass appearance

Question 11

How about a biopsy for asbestosis? What is seen?

Answer 11

Linear asbestos bodies in the lung tissue (ferruginous bodies)

Question 12

There are two common complications with asbestosis. What is the:

Most common:
Most specific:

Answer 12

Common: bronchogenic carcinoma

Specific: Malignant mesothelioma

Question 13

Silicosis is due to inhalation of silicon dioxide. What careers/hobbies are at risk for this? What part of the lung does this affect?

Answer 13

Upper lobes

-Coal mining, quarry work with granite/slate/quartz
-Pottery makers
-Sandblasting, Masonry

Question 14

What is seen on CXR for silicosis?

What is the definitive diagnostic?

Answer 14

-Multiple, small round opacities (miliary pattern) in the upper lobes
-Eggshell calcifications of hilar and mediastinal nodes

Lung biopsy is definitive

Question 15

What careers/people are at risk for berylliosis?

Answer 15

Inhalation of beryllium

-Aerospace, electronics, tool manufacturing, jewelry making, fluorescent light bulbs

Question 16

What is seen on CXR for berylliosis?

Lung biopsy?

Answer 16

CXR: may be normal, hilar LAD, increased interstitial lung markings

Biopsy: noncaseating granulomas

Question 17

Treatment for berylliosis

Answer 17

Corticosteroids, oxygen

Question 18

What is byssinosis from?

What is one unique symptom of this condition?

Answer 18

Cotton exposure, textile industry, hemp dust exposure

Monday Fever: symptoms get worse at the beginning of the week and improve later in week/weekend

Question 19

In sarcoidosis, granulomas form throughout the body, but the lungs are the MC site for these. What is a granuloma?

Who is at risk for sarcoidosis?

Answer 19

Granuloma: WBC’s and macrophages that try to wall off foreign body or substance that immune system can’t get rid of

-Females, AA, Northern-Europeans

Question 20

Symptoms of sarcoidosis (include all the extra pulmonary symptoms as well)

Answer 20

-50% asymptomatic
-Fever, weight loss, fatigue
-LAD: Hilar
-Erythema Nodosum: on shins
-Lupus Pernio (face and extremities)
-Anterior Uveitis
-Restrictive Cardiomyopathy
-Cranial nerve palsies: CNVII

Question 21

In regards to sarcoidosis, what is Lofgren Syndrome?

Answer 21

Erythema Nodosum + Bilateral Hilar LAD + Polyarthralgias with fever

Question 22

What is classic on CXR for sarcoidosis?

Answer 22

Bilateral hilar LAD (bat wing appearance), reticular opacities, ground glass appearance, eggshell calcifications, fibrosis

Question 23

PFT for sarcoidosis is a restrictive pattern as well. What is the definitive/gold standard diagnosis for sarcoidosis?

Answer 23

-Tissue biopsy: noncaseating granulomas

Question 24

What are the two specific lab findings in sarcoidosis?

Answer 24

Increased ACE levels from T-cells

Hypercalcemia (from macrophages)

Question 25

Treatment for sarcoidosis

Answer 25

-Asymptomatic: observation
-Symptomatic: oral corticosteroids

Question 26

What is hypersensitivity pneumonitis?

Name some types of this condition

Answer 26

-Generalized lung inflammation of alveoli and respiratory bronchioles due to organic dusts, molds, foreign proteins, and chemicals

Farmer’s/Cattle Worker’s Lung, Parrot Fever, Grain Worker’s Lung, Silo Filler Disease

Question 27

What are the etiologies of the following hypersensitivity pneumonitis?

-Farmer’s/Cattle Worker’s Lung:
-Parrot Fever:
-Grain Worker’s Lung:
-Silo Filler Disease:

Answer 27

-Farmer’s/Cattle Worker’s Lung: moldy hay
-Parrot Fever (Psittacosis): infected birds
-Grain Worker’s: wheat infested with c weevils
-Silo Filler: nitrogen dioxide gas from plant matter

Question 28

Symptoms of general hypersensitivity pneumonitis

Answer 28

-Rapid onset fever, chills, dyspnea, 4-8 hours after exposure
-Inspiratory crackles
-Dyspnea
-Productive cough

Question 29

On chest xray, where are the diffuse, micro nodular interstitial patterns associated with hypersensitivity pneumonitis?

Answer 29

Lower lung fields MC

Question 30

Silo Filler Disease is hypersensitivity pneumonitis from….

What are some recommendations to avoid this condition?

Answer 30

From nitrogen dioxide gas exposure released from plant matter stored in silos as they ferment
-Gas converted into nitric oxide in lungs when inhaled
-Also seen with combustion exposure (fires, diesel fumes)

-Do not enter the silo for 2 weeks, enter at the top of the silo, and wear N95 masks

Question 31

Parrot Fever (Psittacosis) is from an infection with ______ due to infected bird exposure. This organism is inhaled in dried feces (cleaning cages, etc.)

Answer 31

Chlamydophila psittaci

Question 32

What is the first-line treatment for Parrot Fever?

Answer 32

Tetracyclines (Doxycycline)!