Restrictive Lung Disease

Question 1

how does restrictive lung disease impact lung volumes?

Answer 1

decrease in RV compared to normal

overall, total lung volume also decreases

Question 2

what is the overall impact of restrictive lung diseases on the lungs?

Answer 2

1. reduced total lung volume
2. decrease compliance
3. decrease inspiratory reserve capacity
4. hypoxemia
5. associated with conditions that reduce cell wall movement

Question 3

how are restrictive lung diseases treated?

Answer 3

1. symptom management
2. corticosteroids
3. ensure adequate oxygenation, maintain an airway, and obtain max physical function
4. sometime surgical modification
5. heart-lung transplants

Question 4

Restrictive lung diseases can be put into what 3 categories?

Answer 4

1. Chronic Interstital diseases
2. Chest wall disorders
3. Pulmonary involvement in autoimmune diseases

Question 5

List restrictive lung diseases that are chronic interstitial diseases

Answer 5

1. Pneumoconiosis
2. Honeycomb lung
3. Pulmonary fibrosis

Question 6

what is Pneumoconiosis?

Answer 6

a disease caused by breathing in certain kinds of dust particles that damage the lungs

often called occupational lung disease

results in interstitial fibrosis

Question 7

describe the pathology of Pneumoconiosis

Answer 7

usually takes years to develop

inflammation from particulate matter leads to pulmonary scarring

blood vessels and alveolar damage-become thicker and stiffer

Question 8

What are the s/s of Pneumoconiosis?

Answer 8

1. cough
2. phlegm
3. SOB contributing to reduced physical functional capacity
4. progressive respiratory failure
5. lung cancer
6. TB
7. heart failure (cor pulmonale)

Question 9

Give some examples of conditions that are a type of Pneumoconiosis

Answer 9

1. Black lung disease (coal dust)
2. Brown lung disease (agricultural dust)
3. Asbestoses
4. Silicosis (silica)

Question 10

how is Pneumoconiosis treated?

Answer 10

nonreversible

treat complications

some meds

chest physiology → mobilize and remove secretions

exercise

Question 11

what is honeycomb lung?

Answer 11

widespread lung fibrous

dialted and thickened terminal and respiratory bronchioles

Question 12

what is pulmonary fibrosis?

Answer 12

refers to a variety of disorders in which ongoing epithelial damage or chronic inflammation of lung tissue leads to progressive scarring of the lung resulting in respiratory failure

Question 13

what are some causes of pulmonary fibrosis?

Answer 13

1. Idiopathic (66% of the time)
2. scar tissue resulting from recovery from active diseases like:
   
   • TB
   • ARDs
   • RA, SLE (CT diseases)
   • XRT and chemotherapy

Question 14

describe the clinical course for idiopathic pulmonary fibrosis

Answer 14

• begins insidiously w/gradual increasing exertional dyspnea and dry cough
• unpredictable progression
• hypoxemia, cyanosis and clubbing
• decreased diffusing capacity of the alveolocapillary membrane leading to hypoxemia
• median survival ~3 years after dx

Question 15

what is the only definitive therapy for idiopathic pulmonary fibrosis?

Answer 15

lung transplantation

Question 16

list categories/types of chest wall disorders that can be considered restrictive lung diseases

Answer 16

1. Neuromuscular
   
   • ALS, polio, MD, Guillian-Barre
2. Skeletal deformities
   
   • kyphosis, scoliosis, chest wall injury or deformity
3. Postsurgical status
   
   • abdominal and thoracic surgeries
4. Obesity (impact diaphragm descending)
5. Collagen vascular diseases
   
   • scleroderma, SLE, RA

Question 17

List some autoimmune diseases that impact pulmonary function and result in restrictive lung diseases

Answer 17

1. Systemic sclerosis (scleroderma)
2. RA
3. SLE

Question 18

what is scleroderma?

Answer 18

an autoimmune disease of CT characterized by excessive collagen deposition in the skin and internal organs, particularly the kidneys and lungs

Question 19

how does scleroderma impact/damage organs?

Answer 19

inflammation

severe thickening and obstruction of vessels

cutaneous fibrosis occurs

Question 20

how does RA impact the lungs?

Answer 20

30-40% of RA pts have pulmonary involvement

can increase infection risk and it impacts CT leading to fibrosis → leads to various lung related pathologies like chronic pleuritis, pneumonitis and fibrosis and pulmonary HTN

Question 21

what is a characterstic sign of SLE?

Answer 21

a facial rash that resembles the wings of a butterfly unfolding across both cheeks

Question 22

how many SLE patients experience pulmonary related issues and what are the 2 types of pulmonary issues?

Answer 22

~50%

acute lupus pneumonitis

chronic lupus pneumonitis

Question 23

what is acute lupus pneumonitis?

Answer 23

an acute infection of the lungs in a patient with lupus

this is a serious condition requiring immediate attention

can cause lung scarring

Question 24

what is chronic lupus pneumonitis?

Answer 24

long term pneumonitis

slowly developing infection that results in scarring and pulmonary HTN

Question 25

T/F: SLE is a multi-system disease

Answer 25

TRUE

impacts kidneys, lungs, CNS, blood vessles, and heart

Question 26

chest wall trauma that impact lungs function can be broadly described as _______________________

Answer 26

anything that disrupts normal thoracic wall integrity and thus movement

blunt force trauma, rib/sternal injuries, surgical injury (thoracotomy)